Chronic lung & Systemic diseases Flashcards by Waleed Eldahshan (2024)

1

Q

  1. The CT chest of a 35-year-old-patient reveals parenchymal nodules, emphysema and multiple thin-walled cysts. Some of these have a bizarre configuration and are predominantly located in the upper and middle zones, sparing the costophrenic recesses and tips of the middle lobe and lingual. What is the most likely diagnosis?

A. Lymphangioleiomyomatosis

B. Birt–Hogg–Dubé syndrome

C. Lymphocytic interstitial pneumonia (LIP)

D. Sarcoidosis

E. Langerhans’ cell histiocytosis (LCH)

A

E. Langerhans’ cell histiocytosis (LCH)

Early findings include interstitial infiltrate centred on the small airways, leading to the development of small parenchymal nodules. These are typically irregular, of soft-tissue density, and measure 1-5mm in diameter, although some may be over 1cm in size.

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2

Q

  1. The findings of a High Resolution Computed Tomography (HRCT) scan include irregular bronchovascular, interlobular septal and pleural thickening. There is beading along the bronchovascular bundles, traction bronchiectasis and areas of ground-glass opacification. There is also lymph node enlargement at both hilar nodes and some air trapping. Which is the most likely diagnosis?

A. Tuberculosis

B. Sarcoid

C. Lymphoma

D. Usual Interstitial pneumonitis (UIP)

E. Non-specific Interstitial Pneumonitis (NSIP)

A

B. Sarcoid

These are characteristic features of sarcoid on HRCT. Perilymphatic nodules (beading along the fissures) and architectural distortion are commonly found.

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3

Q

  1. Which of the following features detected on HRCT would most favour NSIP rather than USIP?

A. Predominant ground-glass opacification

B. Honeycombing

C. Predominant lower zone disease

D. Traction bronchiectasis

E. Subpleural predominance

A

A. Predominant ground-glass opacification

Although Ground glass opacification (GGO) can be commonly seen in USIP, it is found in 75-100% of cases of NSIP, where it is usually bilateral, symmetrical and subpleural in two thirds of patients and has a lower zone predominance on over 50%. Honeycombing is not a major feature, although it can occur in up to 50%. Typical HRCT features of USIP include reticulation, honeycombing and traction bronchiectasis with basal and subpleural predominance.

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4

Q

1) A 40-year-old male presents with shortness of breath. He also has lower back pain and stiffness of the spine. A chest radiograph shows bilateral upper-zone fibrosis with elevation of the hila. Spinal ligamentous ossification is also noted. High-resolution CT shows peripheral interstitial changes with traction bronchiectasis and paraseptal emphysematous changes in the upper zones. What is the most likely diagnosis?

a. ankylosing spondylitis

b. Reiter’s syndrome

c. tuberculosis

d. sarcoidosis

e. chronic extrinsic allergic alveolitis

A

a. ankylosing spondylitis

Pulmonary changes occur in ankylosing spondylitis in 1% of patients, with reticulonodular abnormality in the upper zones progressing to confluent opacification and fibrosis. There may be apical bullous formation and cavitation mimicking tuberculosis. No granulomatous disease is seen. Ossification of spinal ligaments is typical in ankylosing spondylitis. Upper-zone fibrosis is seen with tuberculosis, sarcoidosis and chronic extrinsic allergic alveolitis, but spinal ossification is not a feature of these conditions. The reciprocal is true of Reiter’s syndrome.

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5

Q

8) In systemic sclerosis, what is the most common pulmonary finding on CT?

a. consolidation secondary to aspiration pneumonia

b. pulmonary hypertension

c. pleural effusions

d. interstitial lung disease

e. pulmonary emboli

A

d. interstitial lung disease

All of these features can occur, but 65% of patients have fibrosis on CT, although only 30% of patients with systemic sclerosis have clinically significant lung disease. The commonest interstitial lung disease is nonspecific interstitial pneumonitis, seen in two-thirds of cases. This is less coarse and extensive than usual interstitial pneumonitis, the other type seen, and is almost always associated with ground-glass opacification. Other respiratory features seen in systemic sclerosis are organizing pneumonia, pulmonary hemorrhage, pleural thickening and malignancy.

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6

Q

(Ped) 10) A 12-year-old boy with known Langerhans’ cell histiocytosis presents with tachypnoea, cough and dyspnoea. A chest radiograph and then high-resolution CT are performed. What are the most likely findings?

a. bilateral, symmetrical consolidation

b. bronchiectasis

c. reticulonodular changes in mid-zones

d. bronchial wall thickening

e. bilateral hilar lymphadenopathy

A

c. reticulonodular changes in mid-zones

Langerhans’ cell histiocytosis presents with features similar to adults in children over 10 years. Typically, there are reticular/reticulonodular changes that progress to cysts and honeycombing. These predominate in the upper zones and mid-zones with sparing of the costophrenic angles. The diagnosis is made by the characteristic radiographic and clinical findings. Bronchoalveolar lavage or biopsy may be necessary if the diagnosis is in doubt.

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7

Q

36) A 68-year-old patient presents with cough and dyspnea. A chest radiograph is performed. Which feature would suggest sarcoidosis as a more likely diagnosis than tuberculosis?

a. pleural effusion

b. cavitating upper lobe lesion

c. calcified lung lesion

d. symmetrical hilar lymphadenopathy

e. consolidation

A

d. symmetrical hilar lymphadenopathy

Differentiation of tuberculosis and sarcoidosis can be difficult clinically and radiologically. The presence of symmetrical hilar lymphadenopathy is seen more often with sarcoidosis than with tuberculosis. Pleural effusions, and cavitating and calcified lesions are much more common with tuberculosis. Consolidation can be seen in both conditions, though it is again more commonly seen in tuberculosis.

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8

Q

44) A 38-year-old pregnant female presents with hemoptysis and shortness of breath. A chest radiograph shows a large effusion, which is drained and found to be chylous. What is the most likely diagnosis?

a. histiocytosis

b. tuberous sclerosis

c. neurofibromatosis

d. lymphangiomyomatosis

e. idiopathic pulmonary fibrosis

A

d. lymphangiomyomatosis

Lymphangiomyomatosis is exclusively seen in women of child-bearing age and is worsened in pregnancy. Classically, it causes coarse reticular/ reticulonodular interstitial changes, and patients develop recurrent chylous effusions and pneumothoraces. Lung volumes are preserved or may be increased (the only interstitial lung disease with increased lung volumes). It may progress to honeycombing. Histiocytosis usually affects men, and causes cystic change in the upper lobes, small nodules and septal thickening. Tuberous sclerosis has similar features to histiocytosis but is associated with skin changes, reduced IQ and epilepsy. Neurofibromatosis tends to cause progressive fibrosis, but effusions and pneumothoraces are uncommon. Idiopathic pulmonary fibrosis tends to affect the older age group with a reticulonodular pattern.

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9

Q

51) A 52-year-old female presents with cough. She is on dialysis, but, apart from abnormal urea and creatinine, her bloods are normal. A chest radiograph is abnormal and high-resolution CT is performed. This demonstrates fluffy, nodular, 5–10 mm opacities of airspace-type appearance with foci of calcification, in an upper lobe distribution with subpleural sparing. Calcification of chest wall vessels is noted. What is the most likely cause of the appearances?

a. varicella

b. chronic renal failure

c. tuberculosis

d. histoplasmosis

e. talcosis

A

b. chronic renal failure

The appearances are suggestive of ‘metastatic’ pulmonary nodular calcification secondary to chronic renal failure. The upper lobe distribution is due to the relative alkalinity of the upper lobes, caused by the higher ventilationto-perfusion ratio. It is often associated with calcification of chest wall vessels. Varicella produces multiple calcified nodules of 1–3mm after the acute episode has resolved. Patients with tuberculosis or histoplasmosis, where calcified nodules are seen, have 2–5mm, well-defined nodules, and most have calcified lymphadenopathy. Talcosis characteristically produces 1mm, very-high density nodules.

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10

Q

52) A 75-year-old man presents with worsening shortness of breath. He was a mine worker. A chest radiograph shows multiple nodules in the upper zones with a large upper-zone mass on the left. CT confirms multiple small nodules up to 5 mm with a sausage-shaped mass paralleling the mediastinum. What is the most likely diagnosis?

a. coal worker’s pneumoconiosis with bronchogenic carcinoma

b. coal worker’s pneumoconiosis with progressive massive fibrosis

c. tuberculosis

d. primary lung carcinoma with metastases

e. chronic extrinsic allergic alveolitis

A

b. coal worker’s pneumoconiosis with progressive massive fibrosis

The history of mining (dust exposure) with small nodules in the upper zones is typical of pneumoconiosis. The sausage-shaped mass is characteristic of progressive massive fibrosis (PMF), although malignancy cannot be excluded. PMF is often seen to have reduced nodularity surrounding it, as it incorporates the surrounding nodules and migrates towards the hilum. Tuberculosis usually produces a generalized distribution of 2–3mm nodules in its miliary form, often with lymphadenopathy. Metastases from primary lung cancer are not uniformly small (though they can be in thyroid cancer and melanoma). Chronic extrinsic allergic alveolitis produces fibrotic changes in the mid and lower zones.

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11

Q

56) In patients with rheumatoid arthritis, what is the commonest pulmonary finding seen on the chest radiograph?

a. pleural effusion

b. fibrosis

c. pulmonary nodules

d. bronchiectasis

e. heart failure

A

a. pleural effusion

Between 2% and 54% of patients with rheumatoid arthritis have pulmonary abnormalities. Pleural abnormalities are most frequent, being either an effusion (unilateral in 92% of cases) or pleural thickening (usually bilateral). Fibrosis occurs in 30% of patients with pulmonary involvement. Nodules are unusual and seen in advanced disease. They are usually peripheral and may cavitate. Bronchial abnormalities are seen in 30% of patients with rheumatoid lung, and include bronchiectasis and bronchiolitis obliterans. Other findings include pulmonary arterial hypertension and heart failure secondary to carditis/pericarditis.

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12

Q

70) A 38-year-old male presents with a cough, not responding to antibiotics. A chest radiograph shows reticular change in both lungs with normal volumes. High-resolution CT confirms multiple thin-walled cysts with centrilobular nodules of 3–10 mm throughout the lungs. The intervening lung is normal. No pleural effusion is present. What is the most likely diagnosis?

a. neurofibromatosis

b. tuberous sclerosis

c. lymphangiomyomatosis

d. histocytosis

e. usual interstitial pneumonitis

A

d. histocytosis

Honeycombing is seen with all of these conditions, but the combination of normal volumes, cysts and peribronchial nodules is very suggestive of histiocytosis. Often the lung bases are preserved early in the disease but the whole lung is eventually involved. Lung volumes in usual interstitial pneumonitis are reduced. Neurofibromatosis causes interstitial fibrosis with little nodularity. Tuberous sclerosis causes lower-zone fibrotic change with miliary nodules that progresses to honeycombing. Lymphangiomyomatosis produces coarse reticular change due to cyst formation, but nodularity is not seen. Pneumothoraces and pleural effusions are seen with neurofibromatosis, tuberous sclerosis and lymphangiomyomatosis.

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13

Q

91) A 60-year-old female presents with increasing shortness of breath. She is known to have rheumatoid arthritis. A chest radiograph shows reticulonodular changes, and high-resolution CT is performed. Which feature would suggest underlying sarcoidosis as a more likely diagnosis than rheumatoid lung?

a. lower-zone predominance of reticulation

b. mid-zone predominance of reticulation

c. pleural effusion

d. multiple nodules larger than 20 mm

e. cardiomegaly

A

b. mid-zone predominance of reticulation

Sarcoidosis is seen more commonly in females and affects the thorax in 90% of cases. It usually produces adenopathy with or without parenchymal disease. There is a mid-zone predominance with irregular septal thickening, peri lymphatic nodules, ground-glass opacification, traction bronchiectasis and honeycombing. Rheumatoid lung is more common in males and mostly presents with pleural abnormalities, usually an effusion. It may present with an interstitial fibrotic picture with a lower-zone predominance. Multiple large nodules of up to 7cm in diameter are seen with rheumatoid lung. Cardiomegaly may be seen with both sarcoidosis and rheumatoid, as a result of congestive cardiac failure.

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14

Q

99) A 67-year-old presents with shortness of breath. A chest radiograph shows a reticulonodular pattern with hilar adenopathy. Which feature on high-resolution CT would make silicosis a more likely diagnosis than sarcoidosis?

a. nodules .10 mm

b. calcified hilar lymph nodes

c. traction bronchiectasis

d. honeycombing

e. progressive massive fibrosis

A

e. progressive massive fibrosis

Silicosis is due to inhalation of silicon dioxide particles, and radiographic changes of chronic silicosis are seen 10–20 years after exposure. The nodules are typically less than 10mm, compared with sarcoid nodules, which can be more variable in size (acinar type 6–7mm, alveolar type .10mm). Calcified hilar lymph nodes (eggshell pattern), traction bronchiectasis and honeycombing occur in both conditions and are not discriminatory. Progressive massive fibrosis is a complication of the pneumoconiosis and is not seen in sarcoidosis.

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15

Q

100) A 25-year-old male presents with hemoptysis. A chest radiograph shows symmetrical, bilateral, perihilar consolidation, extending to the bases but with sparing of the apices. There is mild hilar enlargement. The patient is also found to be in renal failure. Appearances on the chest radiograph subsequently progress to an interstitial pattern. What is the most likely diagnosis?

a. primary pulmonary haemosiderosis

b. secondary pulmonary haemosiderosis

c. Goodpasture’s syndrome

d. hereditary hemorrhagic telangiectasia

e. histoplasmosis

A

c. Goodpasture’s syndrome

Goodpasture’s syndrome is an autoimmune disease characterized by glomerulonephritis and pulmonary hemorrhage. Respiratory features are usually preceded by respiratory infection. Mild hemoptysis occurs associated with cough and dyspnea. Bilateral consolidative changes and sometimes enlarged hilar nodes may occur. Pulmonary haemosiderosis presents in a similar manner, but usually occurs in patients under 10 years of age in the primary form, with adults affected in the secondary form, which is rare. Patchy airspace change occurs in the first 2 days and then resolves. Progression to fibrosis may occur. Patients with hereditary hemorrhagic telangiectasia usually have multiple arteriovenous malformations.

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16

Q

3- A CXR is requested in a 52-year-old man with chronic dyspnea. The CXR shows bi-apical volume loss and reticulonodular changes within both upper zones. Which of the following is the most likely underlying etiology?

(a) α-anti-trypsin deficiency

(b) Bleomycin toxicity

(c) Silicosis

(d) Systemic sclerosis

(e) Usual interstitial pneumonitis

A

(c) Silicosis

Causes of interstitial lung disease which predominantly affects the upper lobes include T B, silicosis, sarcoid, prior radiation therapy, ankylosing spondylitis, chronic EAA, histoplasmosis, and PCP in patients who have received aerosol pentamidine prophylaxis. The typical distribution of α1-AT is peripheral and basal, the other conditions predominantly affect the lower zones.

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17

Q

18- Which of the is the following is not feature of idiopathic Pulmonary fibrosis at CT?

(a) ground glass opacification

(b) Tractional dilatation of the airways

(c) honeycombing.

(d) Basal predominance

(e) Sub pleural distribution

A

(a) ground glass opacification

Answers (b) — (e) are the classical features of I PF on CT. Ground glass opacification is not a significant feature of IPF- IPF is more common in smokers and, as such, may co-exist with emphysema which may preserve total lung volumes. Irrespective of smoking history, the incidence of bronchogenic carcinoma is increased 7 x in IPF.

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18

Q

(Ped) 22 A 15 year old boy under investigation for polyuria presents to A&E with an acute episode of dyspnoea. CXR reveals a small right-sided pneumothorax and lung cysts are noted: What is the most likely underlying diagnosis?

(a) a1-anti-trypsin deficiency

(b) Langerhans Cell Histiocystosis

(c) Lymphangiomyomatosis

(d) Neurofibromatosis

(e) Tuberous sclerosis

A

(b) Langerhans Cell Histiocystosis

All are causes of cystic lung disease which may present in young patients. LAM is seen in female patients. In chronic disseminated LCH (Hand-Schuller-Christian disease) there is a classic triad of exophthalmos, diabetes insipidus (due to pituitary stalk lesions) and lytic skull lesions. In 25% of these patients there are lung cysts associated, with bleb formation and spontaneous pneumothorax. The history of polyuria makes LCH the likely diagnosis.

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19

Q

24- A 20-year-old male smoker presents with a history of recurrent spontaneous pneumothoraxes. CXR and subsequent CT show bilateral upper zone fine reticulonodular change with some larger discrete upper thin-walled cysts and increased lung volumes. What is the likeliest diagnosis?

(a) Sarcoidosis

(b) Non-specific interstitial pneumonitis

(c) Usual interstitial pneumonitis

(d) Langerhans cell histiocytosis

(e) Lymphangiomyomatosis

A

(d) Langerhans cell histiocytosis

Pulmonary L CH is a rare condition affecting young male smokers, characterized by bilateral upper and mid zone changes starting ‘With nodules (which may cavitate) and progressing through the spectrum of reticular change finishing with true fibrosis. It results in increased lung volumes in up to a third of patients

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20

Q

27- A 46-year-old man with a dry cough is referred for a CT of the thorax. This demonstrates multiple pulmonary nodules, each less than 4 mm in diameter, in a peri lymphatic and sub pleural distribution. What is the most likely diagnosis?

(a) Sarcoidosis

(b) Metastases

(c) Tuberculosis

(d) Varicella pneumonia

(e) Extrinsic allergic alveolitis

A

(a) Sarcoidosis

This is the typical distribution of pulmonary sarcoid nodules. TB, varicella pneumonia, and metastatic disease are randomly distributed. EAA nodules are typically centrilobular.

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21

Q

29- The Intonational Labor Office (ILO) 1980 Intonational Classification of Radiographs pertains to which of the following conditions?

(a) Pneumoconiosis

(b) Sarcoidosis

(c) Extrinsic allergic alveolitis

(d) Hypersensitivity pneumonitis

(e) Asbestos-related lung disease

A

(a) Pneumoconiosis

This is the system most widely used to code pneumoconiosis by comparing against a standardized set of chest radiographs for nodule size, shape and extent. This semi-quantitive measure enables intonational epidemiological studies to be carried out.

22

Q

32- With regard to emphysema, which of the following statements is not true?

(a) It is clinically evident in 15% of smokers

(b) Panacinar emphysema has a predilection for the lower lobes

(c) The chest radiograph has a high sensitivity for diagnosing emphysema

(d) Heterogeneous involvement of the lungs improves outcome following volume reduction surgery

(e) Panacinar emphysema is associated with bronchiectasis

A

(c) The chest radiograph has a high sensitivity for diagnosing emphysema

The chest radiograph has a low sensitivity, but high specificity, for diagnosing emphysema. Centrilobular and panacinar emphysema predominantly affect the upper and lower lobes respectively, the latter being associated with bronchiectasis in 40% of cases. Centrilobular emphysema is the result of pollutants, notably smoking, whilst α1-antitrypsin deficiency is the commonest cause of the panacinar type.

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23

Q

45- A previously healthy 32-year-old man presents to A&E with progressive breathlessness. He has a 3-day history of a flu- like illness- Initial chest radiography demonstrates patchy bilateral consolidation. Over the next few days he develops fulminant respiratory failure. A CT performed 1 week after the onset of symptoms demonstrates areas of consolidation and ground glass opacification; in addition, tractional dilatation of the airways is evident on a CT performed at 2 weeks. What is the most likely diagnosis?

(a) Desquamative interstitial pneumonia

(b) Pneumocystis infection

(c) Lymphoid interstitial pneumonia

(d) Accelerated-phase usual interstitial pneumonia

(e) Acute interstitial pneumonia

A

(e) Acute interstitial pneumonia

The clinical history and CT appearances are characteristic of AIP, formerly known as Hamam-Rich syndrome, which pathologically resembles ARDS.

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24

Q

12 A 32 year old man presents to his GP with increasing shortness of breath. He has never smoked. The initial bloods show a normal full blood count, but abnormal liver function. A CXR is requested and shows emphysematous change within both lower zones only. A subsequent CT is requested. What sub-type of emphysema is likely to be seen?

(a) Centriacinar emphysema

(b) Centrilobular emphysema

(c) Panacinar emphysema

(d) Paracicatricial emphysema

(e) Paraseptal emphysema

A

(c) Panacinar emphysema

α1-anti-trypsin deficiency is a genetically inherited disorder. In individuals with the PiZZ phenotype there is insufficient cx1 AT to protect the lungs from neutrophil elastase. Even non-smokers develop emphysema in the lower zones which involves the pulmonary acinus diffusely (another cause is i. v. methylphenidate abuse). Liver fibrosis/ cirrhosis can also occur in this condition. Centriacinar emphysema (centrilobular and focal subtypes) occurs in smokers. Paracicatricial emphysema is often seen in scar tissue (e.g. in pulmonary fibrosis).

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25

Q

29 A 62 year old otherwise well woman presents with chronic and progressive dysponea. CXR reveals fine reticular opacification in the upper zones bilaterally with volume loss.

Which of the following is the likeliest diagnosis?

(a) Tuberous sclerosis

(b) Extrinsic allegic alveolitis

(c) Sarcoidosis

(d) Usual interstitial pneumonitis

(e) Ankylosing spondylitis

A

(c) Sarcoidosis

Tuberous sclerosis and UIP typically affect the lower zones. Lung volumes are preserved in EAA. Ankylosing spondylitis is a possibility but is much less common than sarcoid.

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26

Q

41 Which of the following is not a risk factor for developing radiation pneumonitis?

(a) Withdrawal of steroids

(b) Increasing interval between radiotherapy fractions

(c) Concomitant chemotherapy

(d) Pulmonary fibrosis

(e) Smoking

A

(b) Increasing interval between radiotherapy fractions

Giving higher doses (>2Gy), more frequent fractions, or a total dose in excess of 10 Gy to the lung are also treatment-specific risk factors. Patient-specific risk factors are increasing age and decreasing performance status.

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27

Q

47 Which of the following is the earliest radiological feature of asbestos-related lung disease?

(a) Pleural thickening

(b) Asbestosis

(c) Pleural effusion

(d) Folded lung

(e) Pleural calcification

A

(c) Pleural effusion

A pleural effusion develops in up to 3% of workers, often occurring between 10 and 20 years after the initial exposure; effusions can be unilateral or bilateral. The effusion is usually small, with the fluid serous or mildly blood stained. The effusion persists up to 6 months, and recurs in up to of 30% cases. Some workers are left with blunting of the costo-phrenic angle, diffuse pleural thickening or round atelectasis. Mesothelioma appears to be unrelated to benign pleural effusions.

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28

Q

50 Which of the following is not a feature of amyloidosis?

(a) Tracheobronchial strictures

(b) Recurrent pneumothoraxes

(c) Calcified parenchymal nodules

(d) Septal thickening

(e) Lymphadenopathy

A

(b) Recurrent pneumothoraxes

Amyloidosis is an uncommon condition in which there is deposition of amyloid protein in the tissues. Its manifestations are manifold and may be localized or diffuse.

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29

Q

53 Which of the following causes of pneumoconiosis is considered a fibrogenic dust?

(a) Silver

(b) Coal dust

(c) Tin

(d) Silica

(e) Iron oxide

A

(d) Silica

Silica and Beryllium are considered fibrogenic dusts, i.e. they elicit a fibrogenic response in the lung parenchyma. The remaining dusts are inert within the lung parenchyma and cause very few clinical symptoms even though the radiographs may appear quite dramatic.

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30

Q

56 A 37 year old lady with Sjogren’s syndrome presents with a 2 year history of progressive breathlessness, lowgrade fever and malaise. A CT of the thorax demonstrates diffuse ground glass opacification and a number of discrete thin-walled cysts within the lungs. The CT is otherwise normal.

What is the most likely diagnosis?

(a) Pneumocystis infection

(b) Non-specific interstitial pneumonitis

(c) Cryptogenic organising pneumonia

(d) Desquamative interstitial pneumonitis

(e) Lymphocytic interstitial pneumonitis

A

(e) Lymphocytic interstitial pneumonitis

Histopathologically characterised by a lymphocytic infiltrate in the lung parenchyma, LIP has an insidious onset and is often diagnosed many months after onset of non-specific symptoms. LIP is also seen in HIV patients, presumably as a consequence of impaired immune function.

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31

Q

59 A 35 year old female is under investigation for known sarcoidosis. Her most recent CXR and HRCT show widespread mid and upper zone reticular opacification with multiple small nodules and no mediastinal or hilar lymphadenopathy.

What is the stage of her disease?

(a) 0

(b) I

(c) II

(d) III

(e) IV

A

(d) III

There is a normal CXR in stage 0. Stage I shows hilar and mediastinal lymphadenopathy only. Stage II shows lymphadenopathy and parenchymal disease. Stage Ill shows parenchymal disease only. Stage IV is characterized by pulmonary fibrosis.

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32

Q

70 At a multidisciplinary team meeting, the pathologist shows a surgical specimen with destroyed and fibrotic tissue. It contains numerous; cystic airspaces with thick fibrous walls and complete loss of acinar architecture. How might you best describe this at CT?

(a) Interlobular septal thickening

(b) Idiopathic pulmonary fibrosis

(c) Honeycombing

(d) Panacinar emphysema

(e) Non-specific interstitial pneumonitis

A

(c) Honeycombing

This is the pathological correlate of honeycombing, a descriptive term only, which is seen in end-stage fibrosis from a number of causes. It is an important criterion for IPF, but is not exclusive to it, and it is not a dominant feature of NSIP.

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33

Q

72 A 23 year old patient presents with acute dyspnoea . and pleuritic chest pain. The CXR shows a right-sided pneumothorax and multiple bilateral cystic changes. Following successful treatment, a CT chest is requested. Which feature favours a diagnosis of lymphangioleiomyomatosis over Langerhans cell histiocytosis?

(a) Chylous pleural effusion

(b) Pulmonary nodules

(c) Smoking history

(d) Thin-walled cysts > 1 cm

(e) Upper lobe predominance

A

(a) Chylous pleural effusion

LAM occurs in young women and is a form fruste of tuberous sclerosis. There is a proliferation of smooth muscle cells along the lymphatics (which may also result in chylothorax). The cysts are < 5-10 mm, thin-walled and surrounded by normal lung. Abdominal and pelvic LAM is associated and renal angiomyolipomas may be present. LCH occurs in young heavy-smoking men, there is an evolution from pulmonary nodules through to thin-walled small, then large cysts, often with a bizarre shape.

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34

Q

73 In the assessment of a thoracic CT, which of the following features is least supportive of a diagnosis of nonspecific interstitial pneumonitis?

(a) Traction bronchiectasis

(b) Volume loss

(c) Ground glass opacifrcation

(d) Thin-walled cysts

(e) Reticular abnormality

A

(d) Thin-walled cysts

Thin-walled cysts are not a feature of NSIP but should raise the possibility of LIP. The remaining features are typical. Consolidation, honeycombing and nodules are variably reported in the literature as being present or absent.

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35

Q

98) A 59-year-old with a history of ventricular arrhythmia presents with dyspnea on exertion. A chest radiograph shows alveolar and interstitial opacification with frank consolidation at the right lung base, which is of high density. There is no peripheral edema or cyanosis, and the heart size is normal. What is the most likely diagnosis?

a. congestive heart failure

b. sarcoidosis

c. extrinsic allergic alveolitis

d. allergic bronchopulmonary aspergillosis

e. amiodarone pulmonary disease

A

e. amiodarone pulmonary disease

Amiodarone lung disease occurs after 1–12 months of treatment. Features are of alveolar and interstitial infiltrates and high-density areas of consolidation (iodine attenuation). Heart failure usually gives a ‘bat-wing’ distribution of consolidation with increased heart size and vascular congestion, and often cyanosis. Sarcoidosis may produce reticulonodular changes, which coalesce to form areas of consolidation. Acute extrinsic allergic alveolitis may produce a consolidative pattern, but reticular changes are seen in the chronic stage. Allergic bronchopulmonary aspergillosis occurs in people with asthma and commonly produces fleeting areas of pneumonitis, with less common features being lobar consolidation, atelectasis and cavitation.

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36

Q

  1. A 50 year old male presents with a history of occasional haemoptysis and exertional shortness of breath which has been getting progressively worse. Plain chest radiograph demonstrates bibasal reticular shadowing with volume loss. HRCT demonstrates bibasal fibrosis and traction bronchiectasis. Incidental note is made of a patulous oesophagus. Which of the following is the most likely cause?

a. Tuberculosis

b. SLE

c. Rheumatoid arthritis

d. Wegener’s granulomatosis

e. Scleroderma

A

  1. e. Scleroderma

Whilst haemoptysis may be a presentation in tuberculosis and Wegener’s and bibasal fibrosis maybe seen in all of the above except tuberculosis (where apical fibrosis is the more likely feature), scleroderma is the only condition resulting in a patulous lower oesophageal sphincter, oesophageal shortening and stricture formation.

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37

Q

(Ped) 4. A 16 year old presents with recurrent pneumothoraces. Past history reveals the presence of a lytic lesion of the parietal bone with a tender soft-tissue mass. Which of the following features is most likely seen on HRCT?

a. Evenly distributed smooth thin-walled cysts

b. Centrilobular nodules

c. Extensive paraseptal emphysema with bulla formation

d. Sparing of the apices

e. Cystic lesions along the course of the bronchial tree

A

  1. b. Centrilobular nodules

The patient had an eosinophilic granuloma and presented with recurrent pneumothoraces due to Langerhans’ cell histiocytosis, which is characterised by centrilobular nodules which cavitate, initially forming thick-walled and then thin-walled cysts. The fibrosis that follows typically involves the upper zones. Cystic lesions along the bronchial tree are a feature of cystic bronchiectasis

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38

Q

  1. A 70 year old man, previously working in a ship-building yard, presents with progressive breathlessness. Chest radiograph demonstrates bilateral calcified pleural plaque disease with volume loss. Lung function shows a restrictive pattern. HRCT reveals pulmonary fibrosis. The most likely site of these changes would be:

a. Perihilar

b. Apical

c. Peribronchial

d. Subpleural

e. Fissural

A

  1. d. Subpleural

Pulmonary fibrosis associated with asbestos exposure is seen mainly in a subpleural distribution towards the lung bases

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39

Q

  1. A 25 year old woman with a longstanding history of non-erosive arthritis of the hands and a malar rash presents with progressive breathlessness and respiratory dysfunction. Blood serology demonstrates anti-DNA antibodies. Which of the following is the most common feature on the chest radiograph?

a. Pleural effusion

b. Consolidation

c. Cavitating nodules

d. Pulmonary oedema

e. Pulmonary fibrosis

A

  1. a. Pleural effusion

Whilst all the above are seen in SLE, pleural effusions are the commonest radiographic abnormality

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40

Q

  1. A 26 year old female patient with an optic nerve tumour and café-au-lait spots presents with exertional breathlessness. Imaging of the chest is most likely to reveal which of the following?

a. Multiple small lower lobe cysts

b. Emphysema

c. Lower zone fibrosis

d. Thick-walled cavities in the upper zone

e. Asymmetrical upper zone fibrosis

A

  1. c. Lower zone fibrosis

The case describes neurofibromatosis I, which is associated with lower zone fibrosis and thin-walled bullae, mainly in the upper zones. Apart from the pulmonary changes, skeletal abnormalities involving the ribs and spine and mediastinal masses may also be seen

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41

Q

  1. A 22 year old female patient with a known phakomatosis presents with anaemia and hypotension. CT angiogram reveals evidence of active bleeding in some of the multiple areas of low attenuation (approximately –20) seen scattered throughout both her kidneys. Which of the following features may be seen on chest CT?

a. Multiple pulmonary AVMs

b. Multiple bilateral small cysts

c. Mediastinal mass

d. Thin-walled upper zone bullae

e. Cardiac rhabdomyomas

A

  1. b. Multiple bilateral small cysts

Phakomatoses are a group of neurocutaneous syndromes, several of which have other multi-system abnormalities, including intrathoracic findings. These include neurofibromatosis I and II, Sturge–Weber syndrome, von Hippel Lindau syndrome, Osler–Rendu–Weber syndrome and tuberous sclerosis. The above features describe multiple renal angiomyolipomas, which are a feature of tuberous sclerosis. These patients may demonstrate multiple thinwalled cysts with lower zone fibrosis (forme fruste of LAM).

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42

Q

  1. A 33 year old female patient presents with a longstanding history of fever, dry cough and weight loss. The chest radiograph reveals mediastinal lymphadenopathy. Blood investigations reveal hypercalcemia and elevated angiotensin-converting enzyme (ACE). Which of the following appearances of lymphadenopathy on CT would be the least likely feature in favour of the clinical diagnosis?

a. Bilateral hilar lymphadenopathy

b. Egg-shell calcification

c. Predominant involvement of the right paratracheal lymph nodes

d. Lymphadenopathy without any parenchymal involvement

e. Posterior mediastinal lymph nodes

A

  1. e. Posterior mediastinal lymph nodes

All the above are features of mediastinal lymphadenopathy in sarcoidosis. Whilst sarcoidosis can involve different mediastinal and hilar groups, posterior mediastinal lymphadenopathy is a feature of NHL

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43

Q

  1. The HRCT of a 35 year old patient with shortness of breath and reticulonodular disease pattern on plain chest radiograph reveals cavitating nodules with interstitial septal thickening. Which of the following diagnoses is the least likely?

a. Lymphangioleiomyomatosis

b. Langerhans’ cell histiocytosis

c. Wegener’s granulomatosis

d. Sarcoidosis

e. Rheumatoid lung

A

  1. a. Lymphangioleiomyomatosis (LAM)

The cysts in LAM are thin-walled air-containing cysts, rather than cavitation in nodules

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44

Q

  1. A patient with a known collagen vascular disease has pulmonary fibrosis. HRCT reveals bilateral lower lobe bronchiectasis. Which collagen vascular disease is most likely?

a. Sjogren syndrome

b. Progressive systemic sclerosis

c. SLE

d. Rheumatoid arthritis

e. Dermatomyositis

A

  1. a. Sjogren syndrome

Whilst pulmonary fibrosis is a feature of all the above conditions, bronchiectasis is most likely seen in Sjogren syndrome

45

Q

QUESTION 1 A 30-year-old man presents to his GP with a cough and increasing shortness of breath. A chest radiograph (CXR) reveals the presence of symmetrical hilar masses. These masses have a lobulated and well-defined outline with some fine peripheral calcification. The lesions do not appear cystic or contain any fat. The only occupational exposure of note is that the patient keeps birds in his house. Which is the most likely diagnosis?

A Mature mediastinal teratoma

B Primary tuberculosis

C Sarcoidosis

D Silicosis

E Untreated lymphoma

A

C Sarcoidosis

Sarcoidosis is a disease of young adults commonly presenting with bilateral symmetrical lymphadenopathy; only occasionally appearing in an asymmetrical distribution

46

Q

QUESTION 7 A 30-year-old male nonsmoker presents to his GP with a three-month history of intermittent episodes of cough and wheeze. Initially diagnosed as having asthma, the patient was found to be α1-antitrypsin deficient after mentioning that several relatives have had similar problems in the past. As part of the subsequent investigations, an HRCT chest was performed. Which finding is most consistent with this clinical scenario?

A Low attenuation regions with a lower lobe predominance

B Low attenuation regions with an upper lobe predominance

C Pleural effusion

D Spontaneous pneumothorax

E Subpleural low attenuation areas

A

A Low attenuation regions with a lower lobe predominance

Panlobular emphysema is seen in αl-antitrypsin deficiency. The disease tends to occur in a lower lobe distribution (unless there is a smoking history, where an upper lobe predominance can be seen

47

Q

QUESTION 34 A previously fit and well 50-year-old man presents with progressive dyspnea for one year. On CXR, there are bilateral, peripheral reticular opacities seen at the lung bases. On HRCT chest, there is a subpleural basal reticular pattern with areas of honeycomb change seen. Which one of the following is the most likely diagnosis?

A Acute interstitial pneumonia (AIP)

B Cryptogenic organising pneumonia (COP)

C Desquamative interstitial pneumonia (DIP)

D Nonspecific interstitial pneumonia (NSIP)

E Usual interstitial pneumonia (UIP

A

E Usual interstitial pneumonia (UIP

The above HRCT findings are almost pathognomonic for UIP and when a confident diagnosis of UIP is made on HRCT it is usually correct

48

Q

QUESTION 35 A 50-year-old woman with rheumatoid arthritis undergoes an HRCT chest following a gradual increase in shortness of breath. Interstitial inflammation and fibrosis is noted. What additional finding is most likely to suggest a diagnosis of NSIP rather than UIP?

A Honeycombing

B Irregular changes over time

C Mediastinal lymphadenopathy

D Prominent ground glass attenuation

E Upper lobe predominance

A

D Prominent ground glass attenuation

NSIP is characterised by interstitial inflammation and fibrosis, but without any of the specific features that allow a diagnosis of UIP to be made. NSIP is usually of uniform temporality and has a more prominent ground glass component than the reticular pattern seen in UIP

49

Q

QUESTION 36 A 20-year-old woman presents with a dry cough and dyspnoea. A CXR has been performed and demonstrates bilateral hilar lymphadenopathy with bilateral well-defined 3 mm parenchymal nodules. The diagnosis is most likely to be?

A Stage 0 Sarcoidosis

B Stage 1 Sarcoidosis

C Stage 2 Sarcoidosis

D Stage 3 Sarcoidosis

E Stage 4 Sarcoidosis

A

C Stage 2 Sarcoidosis

Sarcoidosis has traditionally been staged according to chest radiograph appearances: Stage 0—normal chest radiograph, Stage I—bilateral hilar adenopathy, Stage II—bilateral hilar adenopathy and parenchymal involvement, Stage III—parenchymal involvement with shrinking adenopathy, Stage IV—parenchymal volume loss as a result of pulmonary fibrosis

50

Q

QUESTION 37 A 30-year-old woman has had an HRCT as a result of increasing shortness of breath. As well as mediastinal lymphadenopathy, it demonstrates welldefined nodular opacities (approximately 3 mm in diameter) found subpleurally and along the broncho vascular bundles. There is a predominantly mid to upper zone distribution with some air trapping demonstrated. Which one of the following is the most likely diagnosis?

A Acute extrinsic allergic alveolitis

B Langerhans cell histiocytosis

C Lymphangioleiomyomatosis

D Sarcoidosis

E Usual interstitial pneumonia

A

D Sarcoidosis

51

Q

QUESTION 38 A 30-year-old man has had an HRCT reviewed at your local multidisciplinary team meeting. The HRCT demonstrates ground glass opacification throughout both lungs with a mosaic pattern on expiratory images. Which additional finding would make a diagnosis of extrinsic allergic alveolitis (EAI) more likely than respiratory bronchiolitis—interstitial lung disease (RB-ILD)?

A A normal chest radiograph

B A positive smoking histoiy

C A stable clinical course

D Exposure to paint sprays

E Poorly defined centrilobular nodules

A

D Exposure to paint sprays

Extrinsic allergic alveolitis is an immunologically mediated lung disease as a result of exposure to lung antigens, including those found in paint sprays

52

Q

QUESTION 39 A 30-year-old patient has presented with a nonproductive cough, fatigue, weight loss and a fever. As a result of an abnormality seen on a CXR, an HRCT chest is performed and demonstrates multiple thin-walled cysts within the lung parenchyma. Which additional finding is most likely to suggest a diagnosis of Langerhans cell histiocytosis (LCH) rather than lymphangioleiomyomatosis?

A A smoking history

B Female sex

C Increased lung volumes

D No zonal predilection

E Pleural effusion

A

A A smoking histoiy

LCH is most commonly seen in men (4:1) and the vast majority of patients are cigarette smokers (the converse is true for lymphangioleiomyomatosis).

53

Q

QUESTION 40 A 30-year-old woman complains of shortness of breath and a CXR is requested. The request form states ‘connective tissue disease’ but no further medical history is provided. The CXR demonstrates mild pleural thickening with small, bilateral pleural effusions. Interstitial fibrosis, with some honeycomb formation, is seen within the lower lung zones. In addition there is a single, well-circ*mscribed 2 cm lesion in the right upper zone, with an area of cavitation seen centrally. Which one of the following connective tissue diseases is the most likely diagnosis?

A Ankylosing spondylitis

B Dermatomyositis

C Rheumatoid arthritis

D Scleroderma

E Systemic lupus erythematosus

A

C Rheumatoid arthritis

Rheumatoid arthritis is associated with pleural effusions/thickening, interstitial fibrosis, bronchiectasis and bronchiolitis. Necrobiotic nodules are uncommon and are usually associated with subcutaneous nodules.

54

Q

A 30-year-old woman presents with a history of a low grade fever, malaise, anorexia and weight loss. She also reports pleuritic type chest pain. A CXR shows bilateral small pleural effusions with linear band atelectasis at both bases. No other chest abnormality is seen. Which one of the following is the most likely diagnosis?

A Ankylosing spondylitis

B Dermatomyositis

C Rheumatoid arthritis

D Scleroderma

E Systemic lupus erythematosus

A

E Systemic lupus erythematosus

Pleuro-pulmonary disease is very common in SLE, occurring in over 50% of patients at some stage during the course of their disease. This often manifests as relatively small bilateral pleural effusions, associated with pleuritic chest pain

55

Q

QUESTION 80 A child with known Langerhans cell histiocytosis (LCH) presents with worsening respiratory symptoms. An HRCT chest is performed. Which one of the following statements is true regarding HRCT findings in LCH?

A Early stage involvement shows honeycombing.

B In children under the age of 10 it may spontaneously regress.

C Pulmonary involvement is unusual.

D Pulmonary involvement represents a poor prognosis.

E The costophrenic angles are commonly involved

A

B In children under the age of 10 it may spontaneously regress.

Pulmonary involvement is seen in 42% of patients but it doesn’t necessarily represent a poor prognosis. Reticular or reticulonodular shadowing is seen predominantly within the upper and mid zones, typically with sparing of the costophrenic angles

56

Q

  1. A 45-year-old male smoker has a 6-month history of gradually increasing shortness of breath and cough. CXR shows a mild increase in interstitial markings in the mid and upper zones. A high-resolution CT (HRCT) of chest is requested for clarification and this demonstrates ill-defined centrilobular ground-glass nodules, more pronounced in the mid and upper zones. There is no traction bronchiectasis or honeycombing. What is the most likely diagnosis?

A. Desquamative interstitial pneumonia (DIP).

B. Usual interstitial pneumonia (UIP).

C. Respiratory bronchiolitis interstitial lung disease (RBILD).

D. Non-specific interstitial pneumonia (NSIP).

E. Cryptogenic organizing pneumonia (COP).

A

  1. C. Respiratory bronchiolitis interstitial lung disease (RBILD).

RBILD is a disease of smokers and the centrilobular nodules reflect chronic inflammation in the respiratory bronchioles. GGO may occur and is typically multifocal and upper lobe predominant. Reticulation is uncommon and fibrosis is not a typical feature. The typical features of UIP are reticulation, honeycombing, and traction bronchiectasis, with a basal and subpleural predominance. NSIP has overlapping features with UIP, but GGO is more common and honeycombing less common. There is a similar basal and subpleural predominance on HRCT. DIP is a rare disease and, like RBILD, associated with smoking. The cardinal feature is GGO, with a basal and peripheral predominance. Sometimes small cystic areas can be seen within the areas of GGO. Progression to fibrosis/honeycombing is rare. COP was formerly known as bronchiolitis obliterans organizing pneumonia (BOOP). The main finding on HRCT is consolidation, which is usually multifocal and bilateral. It typically has a lower zone and peripheral predominance, but can affect any lobe. Dilated airways are often seen on HRCT with air bronchograms mimicking acute pneumonia. There may also be GGO and reticulation, but lung volumes are usually maintained

57

Q

  1. A 50-year-old woman presents with gradually increasing shortness of breath. A CXR and HRCT of chest show subpleural reticulation, more marked in the lower zones. Which of the following further findings on HRCT is most likely to support the diagnosis of NSIP?

A. Centrilobular nodules.

B. Air-trapping.

C. GGO.

D. Cystic changes.

E. Pleural effusions

A

  1. C. GGO.

This is a salient feature of NSIP and is seen in almost all cases. Diffuse nodules are very infrequent in NSIP. If centrilobular nodules are present, one should think of other forms of diffuse lung disease such as respiratory bronchiolitis interstitial lung disease (RBILD) or hypersensitivity pneumonitis. If multiple cysts are present, again other diffuse lung disease should be considered, such as lymphocytic interstitial pneumonia (LIP), DIP, lymphangioleiomyomatosis, and pulmonary Langerhans cell histiocytosis. Areas of air-trapping would be a more typical finding in hypersensitivity pneumonitis, rather than NSIP. Pleural effusions are not a typical finding in NSIP.

58

Q

  1. A 45-year-old male smoker has a history of fatigue and mild shortness ofbreath. He also keeps pigeons. A CXR shows mildly increased interstitial markings in the upper zones. An HRCT of chest demonstrates multiple small pulmonary nodules and reticulation, more marked in the upper lungs. What location of the nodularity is more likely to suggest a diagnosis of subacute extrinsic alveolitis or respiratory bronchiolitis interstitial lung disease as opposed to sarcoidosis?

A. Bronchovascular bundle.

B. Centrilobular region.

C. Fissural.

D. Subpleural region.

E. Interlobular septa

A

  1. B. Centrilobular region.

In sarcoid, the granulomatous nodules are typically distributed along the lymphatics and are therefore seen along the bronchovascular bundles, interlobular septa, major fissures, and subpleural regions. The centrilobular region of the secondary pulmonary lobule contains the bronchiole and therefore conditions that cause peribronchiolar inflammation, such as subacute extrinsic allergic alveolitis (EAA) and respiratory bronchiolitis interstitial lung disease (RBILD), more often cause centrilobular nodules. These are often ground glass in attenuation and illdefined, unlike the more solid appearing granulomatous nodules of sarcoid. Rarely sarcoid can cause centrilobular nodules because of the presence of peribronchiolar granulomas, but the other mentioned locations are much more typical

59

Q

  1. A 65-year-old man has a history of liver cirrhosis. He presents with increasing dyspnoea. A CXR shows some basal reticulo-nodular opacities. An HRCT of chest demonstrates distal vascular dilatation and subpleural telangiectasia. You suspect he may have hepato-pulmonary syndrome. Which of the following nuclear medicine techniques is most likely to prove the presence of right-to-left shunting?

A. 99mTc-labelled sulphur colloid scan.

B. 99mTc-labelled red blood cell scan.

C. 99mTc-labelled pertechnetate scan.

D. 99mTc-labelled HMPAO scan.

E. 99mTc-labelled macro-aggregated albumin scan

A

  1. E. 99mTc-labelled macro-aggregated albumin scan.

Whole-body imaging obtained after intravenous injection of 99mTc-labelled macro-aggregated albumin (MAA) shows activity in organs other than the lungs, for example the brain, liver, and spleen, findings that are consistent with an intrapulmonary right-to-left shunt. Normally only about 3% of activity is seen outside the lungs, as the MAA particles are usually efficiently trappedby the pulmonary capillaries, unless there is an intrapulmonary right-to-left shunt. Other imaging findings suggesting hepato-pulmonary syndrome may be seen on conventional and CT angiography. These findings include multiple slightly dilated subpleural vessels that do not taper normally and thus extend to the pleural surface (subpleural telangiectasia). Alternatively, there may be the presence ofindividual arteriovenous malformations on angiograms and nodular dilatation of peripheral pulmonary vessels on CT scans. 99mTc-labelled sulphur colloid is usually used in splenic imaging. 99mTc-labelled red blood cells are typically used to try and identify the location of GI bleeding. 99mTc-labelled pertechnetate is used in thyroid imaging and in the identification of a Meckel’s diverticulum (due to the presence within the diverticulum of ectopic gastric mucosa). 99mTc-labelled hexamethylpropylene amine oxime (HMPAO) is used in brain imaging in an attempt to differentiate different causal pathologies in dementia

60

Q

  1. A 56-year-old woman with a history of Sjogren’s syndrome complains of gradually increasing shortness of breath. A CXR has identified a mild generalized interstitial pattern, with maintained lung volumes. A subsequent HRCT of chest demonstrates a few scattered well-defined, regular lung cysts. Within the lung parenchyma there is also noted patchy groundglass change and mild centrilobular nodularity. Mild mediastinal and hilar lymphadenopathy is present. What is the most likely diagnosis?

A. Langerhans cell histiocytosis.

B. Desquamative interstitial pneumonia.

C. Lymphangioleiomyomatosis.

D. Lymphocytic interstitial pneumonia.

E. Birt–Hogg–Dube syndrome

A

  1. D. Lymphocytic interstitial pneumonia.

The key to this question, in addition to the described imaging features, is the history of Sjogren’s syndrome, which has an association with LIP.

LIP is a benign lymphoproliferative disorder that is also associated with AIDS, autoimmune thyroid disease, and Castleman’s syndrome. Lymphomas may arise in some cases. On HRCT, thin-walled cysts are seen in two-thirds and are randomly distributed, occupying less than 10% of the lung parenchyma. Other features include GGO, centrilobular/subpleural nodules, and septal thickening. Eventually larger nodules (>2 cm), consolidation, and architectural distortion may develop. The presence of mediastinal and hilar lymphadenopathy (two-thirds) and septal thickening help distinguish from Langerhans cell histiocytosis. The presence of centrilobular nodules assist in the differentiation from lymphangioleiomyomatosis.

Birt–Hogg–Dube syndrome is a rare autosomal dominant condition characterized by facial fibrofolliculomas, malignant renal tumours, and the development of thin-walled pulmonary cystsand spontaneous pneumothorax

61

Q

  1. A 67-year-old man who was previously a manual worker presents with chest pain, which subsequently turns out to be due to myocardial ischaemia. He has a CXR performed which shows numerous small nodular densities and you suspect he has an occupational lung disease, as these densities are unchanged from previous radiographs. A subsequent HRCT of chest shows no evidence of linear interstitial change or fibrosis. Pulmonary function tests are normal. Which of the following possible causes is least likely to result in functional lung impairment?

A. Coal workers’ pneumoconiosis.

B. Silicosis.

C. Berylliosis.

D. Siderosis.

E. Asbestosis

A

  1. D. Siderosis.

Siderosis is due to the inhalation of iron oxide particles and usually occurs in welders. It causes mutiple small centrilobular nodules, but is not usually associated with any symptoms or fibrosis. If combined with silica dust it can cause silicosiderosis, which can be associated with fibrosis. Silicosis and coal workers pneumoconiosis, secondary to inhalation of silica dust and washed coal dust, respectively, show similar features on CT. This is usually the presence of 2–5 mm nodules, mainly involving the upper and posterior lung zones. Large opacities (>1 cm) indicate progressive massive fibrosis. Calcification in lymph nodes can occur and egg shell calcification is more typical in silicosis. Berylliosis is a chronic granulomatous lung disease caused by exposure to beryllium dust or fumes. CT findings are similar to other granulomatous lung diseases, such as sarcoid. Fibrosis may therefore occur. Asbestosis is pulmonary fibrosis secondary to inhalation of asbestos fibres.

62

Q

  1. A 35-year-old male smoker presents with a history of progressive dyspnoea and rapidly deteriorating lung function. CXR shows hyperinflated lungs and decreased pulmonary vascular markings. Highresolution CT of chest shows well-defined foci of reduced lung attenuation without definable wall, decreased pulmonary vascular markings and bullae with basilar predominance. What is the likely diagnosis?

A. Centrilobular emphysema.

B. Paraseptal emphysema.

C. Alpha-1 antitrypsin deficiency.

D. Congenital lobar emphysema.

E. Chronic obstructive pulmonary disease (COPD

A

  1. C. Alpha-1 antitrypsin deficiency.

This is a rare autosomal recessive disorder. Alpha-1 antitrypsin is a glycoprotein synthesized in the hepatocytes, which acts as a proteolytic inhibitor. In the absence of alpha-1 antitrypsin, the enzyme elastase released by neutrophils and alveolar macrophages acts unopposed and digests the basem*nt membrane. There is rapid progressive deterioration of lung function due to severe pan-acinar emphysema, which shows basilar predominance (due to gravitational distribution of pulmonary blood flow). The alveolar destruction is accelerated in smokers. Cirrhosis of the liver is a complication

63

Q

  1. A 63-year-old male has a complex past medical history including testicular carcinoma, cardiac disease, rheumatoid arthritis (RA) and diabetes mellitus. He presents with shortness of breath and is referred for a CT chest. This reveals multiple areas of ground-glass attenuation, crazy-paving and consolidation in both lungs. You also notice that the spleen and liver are of increased attenuation. What is the most likely explanation for these findings?

A. RA-related lung disease.

B. Cardiac failure.

C. Amiodarone.

D. Bleomycin.

E. Methotrexate

A

  1. C. Amiodarone.

The pulmonary findings could equally be caused by NSIP and cardiac failure, among other causes. However, the high attenuation of the liver and spleen is due to deposition of amiodarone, which contains iodine. Amiodarone, methotrexate, and bleomycin all may cause pulmonary toxicity. Pulmonary toxicity occurs in 510% of patients on amiodarone, usually within months of starting therapy. The prognosis is good, with most patients improving after discontinuation of therapy. NSIP is the most common manifestation of amiodaroneinduced lung disease. Pleural inflammation is an accompanying feature and can manifest as pleural effusion. COP is less common and typically occurs in association with NSIP. A distinctive feature of amiodarone toxicity is the occurrence of focal, hom*ogenous pulmonary opacities. They are typically peripheral in location and of high attenuation at CT, due to the incorporation of amiodarone into the type II pneumocytes. The combination of high attenuation within the lung, liver, and spleen is characteristic of amiodarone toxicity.

RA can produce many pulmonary sequelae: pulmonary nodules, pleural effusion, fibrosis, obliterative bronciolitis, and COP. Methotrexate can be used to treat RA and psoriasis, and as chemotherapy for various cancers. NSIP is most common and COP is seen less frequently.

Bleomycin is a chemotherapy agent used in the treatment of testicular carcinoma, among others. Diffuse alveolar damage (DAD) is its most common manifestation, with NSIP and COP being less common. The prognosis is poor, with most patients dying of respiratory failure within 3 months of the onset of symptoms

64

Q

  1. A 30-year-old male smoker presents with a history of acute dyspnoea. CXR shows bilateral reticulonodular interstitial changes, predominantly in the upper and mid zones, with preservation of lung volume. There is a rightsided apical pneumothorax and a small right pleural effusion. HRCT of chest shows complex thin- and thick-walled cysts and irregular centrilobular nodules in a similar distribution with sparing of the bases. The intervening lung appears normal. What is the diagnosis?

A. Lymphangioleiomyomatosis.

B. Bronchiectasis.

C. Metastases.

D. Pulmonary Langerhans cell histiocytosis.

E. Idiopathic pulmonary fibrosis

A

  1. D. Pulmonary Langerhans cell histiocytosis.

This is a rare isolated form of Langerhans cell histiocytosis that primarily affects young adult smokers. Most patients are symptomatic and the most frequent symptoms are non-productive cough (50–70% of cases) and dyspnoea (35–87%). Less common symptoms include fatigue, weight loss, pleuritic chest pain, and fever. The most common finding on CXR is small irregular nodules, usually bilaterally symmetric, with upper lobe predominance and sparing of the costo-phrenic angles. Coarse reticular and reticulo-nodular pattern is seen in later stages. Pneumothorax occurs in up to 25% (may be recurrent). Pleural effusion is uncommon, but may occur with pneumothorax. Lung volumes are normal or increased in most patients. HRCT of chest demonstrates the cysts and nodules in a characteristic distribution with normal intervening lung. Interstitial fibrosis and honeycombing are seen in advanced stages. Treatment consists of smoking cessation; steroids may be useful in selected patients. Chemotherapeutic agents and lung transplantation may be offered in advanced disease. The prognosis is variable. Stable disease is seen in up to 50%. Spontaneous regression is reported in up to 25%. A variably progressive, deteriorating course is seen in up to 25%.

65

Q

  1. A 45-year-old male patient is referred by his GP for a CXR due to a history of dyspnoea and cough. The CXR shows a convex appearance to the hila with a right paratracheal stripe that measures 1.5 cm. You are arranging follow-up and the respiratory team ask you for your top differential. What do you say?

A. TB.

B. Lymphoma.

C. Sarcoid.

D. Castleman’s disease.

E. Silicosis.

A

  1. C. Sarcoid.

There are numerous causes of bilateral hilar lymphadenopathy (BHL) and in the absence of further clinical information, sarcoid would always feature high in the list of differentials. However, the finding of BHL in association with paratracheal adenopathy is classical for this disease

66

Q

  1. A 64-year-old woman presents with a 3-week history of dry cough. A CXR is performed and shows multifocal bilateral peripheral areas of consolidation. An HRCT of chest is recommended and this demonstrates bilateral peripheral areas of consolidation and GGO. There is no fibrosis. There is peripheral eosinophilia detected on routine blood tests. A careful drug history is obtained. Which of the following medications is the patient mostlikely to be on?

A. Nitrofurantoin.

B. Amiodarone.

C. Methotrexate.

D. Bleomycin.

E. Cyclophosphamide.

A

  1. A. Nitrofurantoin.

The findings described on the CXR and HRCT scan are those of pulmonary eosinophilia. Drugs known to cause pulmonary eosinophilia include nitrofurantoin, penicillamine, sulphasalazine, nonsteroidal anti-inflammatory drugs, and para-aminosalicylic acid.

Bleomycin and cyclophosphamide are more commonly associated with DAD. Other drugs that can cause this type of lung injury include busulphan, carmustine, gold salts, and mitomycin. In early DAD, HRCT typically shows scattered or diffuse areas of GGO. Fibrosis typically develops within 1 week and if progressive can cause marked architectural distortion and honeycombing. Amiodarone and methotrexate are associated with an NSIP pattern on HRCT. Carmustine and chlorambucil can also cause this appearance. With early disease, HRCT scans may show only scattered or diffuse areas of GGO. Later, findings of fibrosis (traction bronchiectasis, honeycombing) predominate in a basal distribution. Other recognized patterns of drug-induced lung injury include a COP pattern and diffuse pulmonary haemorrhage.

67

Q

36 A 38-year-old female of West African decent presented with a fever, malaise and large joint arthralgia. She was also noted to have erythema nodosum. Her calcium levels were elevated and her chest radiograph showed bilateral nodular pulmonary hila. What is the most likely diagnosis?

a Familial Mediterranean fever

b Malaria

c Acute sarcoidosis

d Severe acute respiratory syndrome (SARS)

e Lymphomatoid granulomatosis

A

36 Answer C: Acute sarcoidosis

Features of acute sarcoidosis (Lofgren syndrome) include fever, malaise, bilateral hilar lymphadenopathy, erythema nodosum, arthralgia and occasionally parotitis and uveitis. The Kveim-Siltzbach test is rarely used and involves intracutaneous injection of a suspension of human sarcoid spleen. ACE levels are more commonly used, which are elevated in 70% of sarcoid patients and are an indicator of the granuloma burden on the body

68

Q

42 A 45-year-old female presented with progressive dyspnoea. A chest radiograph demonstrated reticular densities with preserved lung volumes. An HRCT showed uniform cysts throughout the lung with normal intervening lung. What is the most likely diagnosis?

a Tuberous sclerosis

b Lymphangiomyomatosis

c Histiocytosis

d Tuberculosis

e Cystic fibrosis

A

42 Answer B: Lymphangiomyomatosis

LAM is seen exclusively in women, typically those of child-bearing age. The typical appearances are of thinwalled cysts with normal intervening lung. There may also be small pneumothoraces and chylous effusions

69

Q

47 A 50-year-old man who presented with progressive dyspnoea had a chest radiograph that demonstrated multiple opacities between 0.5 and 2 mm in size, which were noted to be more dense than soft tissue. Which of the following diagnoses is most likely?

a Fungal infection such as histoplasmosis

b Coal miners’ pneumoconiosis

C Sarcoidosis

d Acute extrinsic allergic alveolitis

e Silicosis

A

47 Answer E: Silicosis

All the other conditions would produce nodules of soft tissue density. In pure silicosis the nodules are very well defined and very dense. There is also relative sparing of the bases and apices with septal lines on HRCT

70

Q

55 A 46-year-old patient presented with a long history of dyspnoea, cough and fatigue. A chest radiograph demonstrated established fibrotic changes. What feature most suggests a diagnosis of idiopathic pulmonary fibrosis/usual interstitial pneumonia (UIP) rather than non-specific interstitial pneumonia (NSIP)?

a Traction bronchiectasis

b Septal thickening

c Ground-glass opacification

d Honeycombing

e Reduced lung volumes

A

55 Answer D: Honeycombing

Reduced lung volumes, septal thickening and traction bronchiectasis are features of both conditions. Honeycombing is more prevalent in UIP, but can be seen in NSIP. A consistent finding in NSIP is relatively symmetrical, basal ground glass opacification due to cellular infiltrate. The presence of ground-glass opacification on HRCT in UIP usually represents fine interstitial fibrosis, beyond the resolving power of HRCT rather than a potentially reversible cellular infiltrate. NSIP has been linked to connective tissue diseases such as scleroderma

71

Q

56 A 46-year-old woman presented to the Respiratory Clinic with a two-year history of cough, dyspnoea, fatigue and weight loss. She was under concurrent investigation by the rheumatologists for Sjogren’s disease. Examination was unremarkable apart from bilateral inspiratory crepitations. A chest radiograph revealed a reticular pattern of opacification and subsequent HRCT showed septal thickening, bilateral diffuse ground-glass shadowing and poorly defined centrilobular nodules. What is potential pathological finding on lung biopsy?

a Haemorrhagic alveolar infiltrate

b Aspergillus spores

c Neutrophilic alveolar infiltrate

d Lymphoid interstitial infiltrate

e Histeocytic alveolar infiltrate

A

56 Answer D: Lymphoid interstitial infiltrate

Lymphoid interstitial pneumonitis (LIP) is characterised by a widespread interstitial lymphoid infiltrate, resembling lymphoma but with a clinical course more in keeping with a chronic interstitial pneumonia. LIP is more common in women than in men. Frequently, patients with LIP have evidence of an abnormal immune response such as Sjogren’s disease or AIDS

72

Q

59 A 60-year-old man has chronic obstructive pulmonary disease (COPD). He has a long smoking history and is being considered for lung volume reduction surgery (LVRS). What pattern of disease would give the best chance of a successful outcome following LRVS?

a Mild, predominantly upper and mid zone paraseptal emphysema

b Severe lower zone bullous emphysema and mild upper zone paraseptal emphysema

C Severe upper zone bullous emphysema with relatively spared lower zones

d Severe upper zone bullous emphysema with moderate lower zone centrilobular emphysema

e Severe centrilobular emphysema affecting all zones

A

59 Answer C: Severe upper zone bullous emphysema with relatively spared lower zones.

Lung volume reduction surgery (LVRS) is a palliative procedure for patients with advanced disease. It has a number of clinical exclusion criteria because of relatively high operative risk. There are still some controversies around this form of surgery, but it is likely to continue to have a place in the treatment of emphysema. It comprises wedge resection of the areas of greatest disease, mainly the upper lobes, thus improving the performance of the remaining lung. Best candidates for surgery have upper lobe predominant emphysema, a good amount of normal or mildly emphysematous lung and significant regional heterogeneity on perfusion scintigraphy

73

Q

30 A 35-year-old female presented to the Emergency Department with acute shortness of breath on a background of progressive exertional dyspnoea and cough over the course of 20 months. She does not smoke. Her initial frontal chest radiograph shows a pneumothorax and a background of a coarse reticulonodular interstitial pattern with multiple cysts in all zones of both lungs. A chest drain tube was inserted and a subsequent CT scan showed numerous diffusely scattered thin-walled cysts surrounded by normal lung parenchyma. A left-sided effusion had a density of -18 HU and the precarinal lymph nodes measured 1.6 cm in short axis. What is the most likely diagnosis?

a Histiocytosis

b Lymphangiomyomatosis

c Emphysema

d Neurofibromatosis

e Bronchiectasis

A

30 Answer B: Lymphangiomyomatosis

Lymphangiomyomatosis is a rare condition seen exclusively in women of childbearing age. It is characterised by gradual progressive interstitial disease, recurrent pneumothoraces and chylous pleural effusions. The key feature is the presence of numerous thin-walled cysts of varying sizes diffusely scattered throughout the lungs with normal intervening lung parenchyma. In histiocytosis cysts are located in the upper two-thirds of the lungs, walls are of varying thickness and septal thickening is usually present. In neurofibromatosis, the cystic spaces are predominantly located in the lung apices.

74

Q

35 A 70-year-old man with long-standing severe rheumatoid arthritis developed progressive dyspnoea. Fine `Velcro-like’ crepitations were audible in his chest and a chest radiograph showed reticulonodular densities and well circ*mscribed nodules. Where in the lungs are the reticulonodular densities most likely to be located?

a Apices

b Perihilar

c Subpleural

d Bases

e Paratracheal

A

35 Answer D: Bases

Interstitial fibrosis is most common in the lower lobes. In early pulmonary disease, reticulonodular densities are seen and become progressively coarser in the later stages.

75

Q

46 In a patient with a long-standing history of rheumatoid arthritis, what is the most frequent respiratory manifestation of the disease?

a Pleural effusion

b Diffuse interstitial lung fibrosis

C Multiple well-circ*mscribed peripheral lung nodules

d Bronchiectasis

e Cardiomegaly

A

46 Answer A: Pleural effusion

All of these are found in rheumatoid lung disease but the commonest is a unilateral pleural effusion with no other pulmonary changes. On aspiration the fluid is an exudate with a low white cell count (high in lymphocytes) and a low glucose.

76

Q

50 A 26-year-old Afro-Caribbean lady presented with a painful rash on her shins, arthralgia, fever and malaise. A frontal CXR demonstrated bi-hilar lymphadenopathy, but no parenchymal abnormality. What is the most likely diagnosis?

a Primary pulmonary TB

b Lymphoma

C PCP pneumonia

d Post-primary TB

e Sarcoidosis

A

50 Answer E: Sarcoidosis

This constellation of symptoms and bi-hilar adenopathy, in the absence of parenchymal changes, is typical of acute sarcoid - Lofgren syndrome.

77

Q

51 A patient underwent an HRCT that demonstrated small lung volumes and coarsened septal thickening in a predominantly subpleural and basal distribution. There were areas of honeycombing and traction bronchiectasis and bronchiolectasis. What is the most likely diagnosis?

a Radiation fibrosis

b Silicosis

C Chronic extrinsic allergic alveolitis (hypersensitivity pneumonitis)

d Usual interstitial pneumonia (UIP)

e Beryliosis

A

51 Answer D: Usual interstitial pneumonia (UIP)

These are the classical HRCT findings in established pulmonary fibrosis. The lower-zone subpleural distribution is typical of UIP. The other diagnoses here have an upper-zone predilection.

78

Q

55 A 56-year-old airport baggage handler presented to the respiratory clinic with dyspnoea and cough in addition to weight loss and occasional chest pain. He had smoked 60 cigarettes a day for over 30 years. There was no evidence of finger clubbing on examination and pulmonary function tests demonstrated a mixed obstructiverestrictive pattern with reduced diffusion capacity. Groundglass opacification, air trapping, centrilobular nodules and mild septal thickening were seen on HRCT. What is the most likely diagnosis?

a Usual interstitial pneumonitis (UIP)

b Respiratory bronchiolitis-associated interstitial lung disease (RBILD)

C Lymphocytic interstitial pneumonitis (LIP)

d Non-specific interstitial pneumonitis (NSIP)

e Cryptogenic organising pneumonia (COP)

A

55 Answer B: Respiratory bronchiolitis-associated interstitial lung disease (RBILD)

RBILD is seen almost exclusively in heavy smokers. Centrilobular nodules are a key finding, and are not commonly seen in other interstitial pneumonitides, except LIP, where air trapping is not such a prominent feature. The histology and aetiology of RBILD and desquamative interstitial pneumonia (DIP) are very similar and they may represent the ends of a spectrum of a single disease.

79

Q

56 HRCT was performed on a 60-year-old patient who had a long history of dyspnoea. He was no longer able to climb the stairs at home due to breathlessness. His chest radiograph showed changes of fibrosis. What further finding on HRCT would most favour a diagnosis of sarcoid over chronic extrinsic allergic alveolitis (EAA)?

a Interstitial thickening

b Traction bronchiectasis

C Air trapping

d Pleural effusions

e Nodular thickening of the fissures

A

56 Answer E: Nodular thickening of the fissures

Pleural effusions are rare in both conditions and the remaining options are features of both sarcoid and EAA. The nodules in sarcoid, seen along bronchoarterial bundles and veins and within septal lymphatics, are caused by epitheliod cell granulomas.

80

Q

57 A 52-year-old miner presented with increasing exertional dyspnoea and cough, which were becoming more severe over many years. Fine mid- and upper-zone inspiratory crepitations were apparent on examination. His chest radiograph showed small (<10 mm), rounded mid- and upper zone opacities some of which displayed central calcification. There was also a reticular mid and upper-zone pattern of opacification. HRCT confirms the presence of thickened intra- and interlobular septal lines and nodules with some thicker parenchymal fibrotic bands and traction bronchiectasis. There is bilateral mediastinal adenopathy with peripheral eggshell calcification. What is the likely diagnosis?

a Silicosis

b Coal workers pneumoconiosis

C Siderosis

d Stannosis

e Caplan’s syndrome

A

57 Answer A: Silicosis

Pneumoconiosis is caused by the inhalation and deposition of fine particles of inorganic dust in the lungs. The inhaled dust may be classified as non-fibrogenic or fibrogenic, depending on how the body reacts to the inhaled particles. Coal, tin (stannosis) and iron oxide (siderosis) are non-fibrogenic, whereas silica and beryllium are fibrogenic. The clinical and radiological picture here is one of fibrosis caused by long-term silica dust inhalation. Hilar lymph node eggshell calcification is very typical of silicosis.

81

Q

35 An elderly male patient with long-standing severe rheumatoid arthritis developed progressive dyspnoea and a chest radiograph showed multiple nodules on a background of coarse reticulonodular densities. Where in the lungs are the pulmonary nodules most likely to be located?

a Lung periphery

b Perihilar

C Paratracheal

d Along the fissures

e No predilection for any lung zones

A

35 Answer A: Lung periphery

Rheumatoid nodules in the lungs are rare. They have the same composition as subcutaneous nodules and are most frequently located in the periphery. They do not calcify.

82

Q

41 A 30-year-old male smoker presented with a non-productive cough, weight loss and dyspnoea. His chest radiograph showed a diffuse, ill-defined reticulonodular pattern with preserved lung volumes. The cardiomediastinal contours and hilar were normal. On HRCT there are thin-walled cysts <5 mm in size and centrilobular nodules with normal intervening lung. What is the most likely diagnosis?

a Sarcoidosis

b Langerhans cell histiocytosis (LCH)

C Idiopathic pulmonary fibrosis

d Lymphangiomyomatosis (LAM)

e Emphysema

A

41 Answer B: Langerhans cell histiocytosis (LCH)

Although the individual features can be seen in several of these conditions, the combination of the clinical history and radiological findings are most in keeping with LCH.

83

Q

49 A 30-year-old male smoker presented to the Respiratory Clinic with dyspnoea and a productive cough. Spirometry demonstrated reduced lung function and the chest radiograph hyperinflation and bi-basal bullae formation with relative sparing of the upper zones. What is the most likely diagnosis?

a Early onset chronic obstructive pulmonary disease (COPD)

b Langerhans cell histiocytosis

c Congenital lobar emphysema

d a 1 antitrypsin deficiency

e Lymphangioleiomyomatosis (LAM)

A

49 Answer D: a 1 antitrypsin deficiency

COPD is an unlikely diagnosis in this setting. CXR changes in Langerhans cell histiocytosis include reticulonodular opacification with relative basal sparing. Congenital lobar emphysema presents in infancy and most often affects a single lobe. LAM is seen in female patients. The findings of lower zone emphysema in a young patient are typical of a 1 antitrypsin deficiency.

84

Q

55 A 50-year-old laboratory technician presented acutely with severe dyspnoea. She gave a history of recent upper respiratory tract infection and examination revealed cyanosis, tachypnoea and bilateral widespread crepitations. Pulmonary function tests showed a restrictive pattern with reduced diffusing capacity. Several days after admission her oxygen requirements increased and required non-invasive ventilatory support. A repeat chest radiograph showed bilateral patchy airspace opacification with air bronchograms sparing the costophrenic angles. HRCT showed consolidation and ground-glass change, bronchial dilatation and architectural distortion and subsequent lung biopsy demonstrated hyaline membrane formation. What is the most likely diagnosis?

a Acute interstitial pneumonitis (AIP)

b Cryptogenic organising pneumonitis (COP)

C Mycoplasma aviurn infection

d Hypersensitivity pneumonitis

e Respiratory bronchiolitis-associated interstitial lung disease (RBILD)

A

55 Answer A: Acute interstitial pneumonitis (AIP)

AIP can be regarded as an idiopathic form of adult respiratory distress syndrome (ARDS) and is histologically (and clinically) distinct from the other interstitial pneumonias. The condition is usually preceded by a history of upper respiratory tract infection and the clinical picture is that of ARDS. Pathologically the findings are of diffuse alveolar damage, of which hyaline membrane formation is the characteristic feature.

85

Q

56 A 72-year-old retired policeman, with a known history of cardiac arrhythmias and increasing dyspnoea over six months, presented acutely with worsening breathlessness. HRCT showed septal thickening and diffuse ground-glass changes in a largely symmetrical and predominantly basal distribution. The liver was also markedly more dense than the spleen. What is the most likely explanation for his dyspnoea?

a Right heart failure

b Drug-induced pulmonary damage

C Usual interstitial pneumonitis (UIP)

d Legionella pneumonia

e Primary pulmonary lymphoma

A

56 Answer B: Drug-induced pulmonary damage

The most plausible explanation is drug-induced pulmonary damage from amiodarone (a commonly prescribed anti-arrhythmic medication). The picture is that of non-specific interstitial pneumonitis (NSIP) indicated by septal thickening and bilateral ground-glass change. Amiodarone deposition within the liver causes increased attenuation due to its high iodine content.

86

Q

  1. A 70-year-old male presents to his GP with cough. The chest radiograph shows bilateral egg shell calcifications in the hilar regions. Which of the following is the least likely diagnosis?

(a) Silicosis

(b) Asbestosis

(c) Coal workers pneumoconiosis

(d) Sarcoidosis

(e) Histoplasmosis

A

  1. (b) Asbestosis

All the other given options are known to cause egg shell calcification of the hilar lymph nodes.

87

Q

  1. A chest radiograph shows diffuse lung disease with fibrotic changes predominantly affecting the upper lobes. What is the most unlikely diagnosis?

(a) Sarcoidosis

(b) Cystic fibrosis

(c) Allergic bronchopulmonary aspergillosis

(d) Langerhans cell granulomatosis

(e) Scleroderma

A

  1. (e) Scleroderma

Other conditions cause predominantly upper zone disease.

88

Q

  1. A 25-year-old man of African origin presents with dry cough. The chest radiograph shows bilateral lobulated hilar shadows. HRCT shows bilateral hilar and paratracheal lymphadenopathy with irregular and nodular septal thickening and traction bronchiectasis. Blood tests show elevated serum angiotensin-convertingenzyme. The most likely diagnosis is?

(a) Lymphoma

(b) Sarcoidosis

(c) Malignant lymphangitis

(d) Tuberculosis

(e) Sjögren’s syndrome

A

  1. (b) Sarcoidosis

Garland’s triad, seen in sarcoidosis, involves bilateral hilar nodes and paratracheal lymph nodes. Seventy per cent of cases of sarcoidosis have elevated serum levels of angiotensin-converting enzyme.

89

Q

  1. A 40-year-old female non-smoker presents with shortness of breath and reduced exercise tolerance. The chest radiograph shows marked lucency in both lower zones with superiorly displaced right horizontal fissure and flattened hemidiaphragm. The upper zones show normal vascularity and lung shadows.

The most likely diagnosis is?

(a) Centrilobular emphysema

(b) Alpha-1-antitrypsin deficiency

(c) Lymphoma

(d) Hypersensitivity pneumonitis

(e) Sarcoidosis

A

  1. (b) Alpha-1-antitrypsin deficiency

Patients with this condition develop severe panacinar emphysema with basilar predominance due to gravitational distribution of pulmonary blood flow. On CT, the margins of cysts are poorly visualised due to involvement of the entire secondary lobule. This pattern is significantly different from chronic smoker’s emphysema, which tends to develop centrilobular emphysema with upper zone predominance and cysts with well-defined margins.

90

Q

  1. A 38-year-old man presents with gradually progressive dyspnoea over 2 years. The chest radiograph shows reduced lung volumes with reticular interstitial changes in both lower zones. HRCT show peripheral and basilar reticular opacities with honeycombing and traction bronchiectasis. The most likely diagnosis is?

(a) Sarcoidosis

(b) Systemic lupus erythematosus

(c) Chronic hypersensitivity pneumonitis

(d) Idiopathic pulmonary fibrosis

(e) Rheumatoid arthritis

A

  1. (d) Idiopathic pulmonary fibrosis

These are typical radiographic and HRCT features of idiopathic pulmonary fibrosis.

91

Q

  1. A 48-year-old female non-smoker presents to the Accident & Emergency Department with acute dyspnoea and chest pain. The chest radiograph shows bilateral basal airspace shadowing. Chest CT shows disuse basal consolidation and air-bronchograms within a background of ground-glass opacity. There is septal thickening and bilateral pleural effusions. The most likely diagnosis is?

(a) Desquamative interstitial pneumonitis

(b) Lymphocytic interstitial pneumonitis

(c) Acute interstitial pneumonia

(d) Usual interstitial pneumonitis

(e) Cryptogenic organising pneumonia

A

  1. (c) Acute interstitial pneumonia

This clinically presents as adult respiratory distress syndrome and has high mortality. It has a fulminant course leading to respiratory failure and requiring mechanical ventilation with a mortality of > 50%. CT findings are non-specific but include bilateral, diffuse ground-glass opacity with consolidation and air bronchograms. Honeycombing and traction bronchiectasis may be seen in advanced cases after recovery.

92

Q

  1. A 70-year-old retired miner presents with shortness of breath for several months. There is no other significant medical history. The chest radiograph shows calcified pleural plaques at both lung bases and bi-basilar interstitial shadowing. CT shows extensive pleural thickening and calcified pleural plaques with bi-basal, peripheral, interstitial shadows and honeycombing. No lymphadenopathy seen. The most likely diagnosis is?

(a) Tuberculosis

(b) Asbestosis

(c) Silicosis

(d) Empyema

(e) Sarcoidosis

A

  1. (b) Asbestosis

This is defined as interstitial pulmonary fibrosis in association with asbestos exposure (pleural plaques and calcification). Disease progression is from bases to apices and honeycombing is seen later in the disease. Lymphadenopathy is usually absent, and its presence should suggest alternate diagnosis

93

Q

  1. A 60-year-old man presents with history of chronic cough. The chest radiograph shows a 5 cm subpleural mass in the right lower lobe. There is a curvilinear opacity from the lower pole of the mass and the mass courses towards the hilum. CT confirms the mass lesion and demonstrates the bronchovascular bundles converging into the mass in a curvilinear fashion. In addition, there are multiple pleural plaques but no lymphadenopathy. The most likely diagnosis is?

(a) Bronchogenic carcinoma

(b) Rounded atelectasis

(c) Large parenchymal metastasis

(d) Lymphoma

(e) Arteriovenous malformation

A

  1. (b) Round atelectasis

Also called ‘folded lung’ or ‘asbestos pseudotumour’. The lesion forms acute angles with the pleura indicating its parenchymal location. Pleural thickening is usually an associated finding. It usually affects the lower lobes and there is volume loss. The characteristic sign of round atelectasis is the ‘comet tail‘ sign. As the lung collapses, the bronchovascular bundle is pulled into the region. As they reach the mass they diverge and arch around the surface to merge with the inferior pole of the mass. This is typically well demonstrated on CT.

94

Q

  1. A 45-year-old woman presents with recurrent episodes of acute exacerbations of dyspnoea. The chest radiograph shows a left hydropneumothorax with large volume lungs and reticular pattern of interstitial opacities. HRCT shows extensive thin-walled cysts throughout the lung parenchyma. The most likely diagnosis is?

(a) Emphysema

(b) Langerhans cell histiocytosis

(c) Lymphangioleiomyomatosis

(d) Idiopathic pulmonary fibrosis

(e) Neurofibromatosis

A

  1. (c) Lymphangioleiomyomatosis

Exclusively seen in women of childbearing age, characterised by large lungs, coarse interstitial pattern, extensive lung cysts and recurrent pneumo- or chylopneumothorax.In histiocytosis, cysts are seen in the upper two-thirds of the lung with sparing of the costophrenic angle, cysts are variable in thickness, and there is septal thickening. Idiopathic pulmonary fibrosis show irregular thick-walled peripheral cysts and honeycombing. Neurofibromatosis has cysts in apical location. Emphysema shows imperceptible cyst walls; cysts show segmental distribution, lobular architecture preserved with the bronchovascular bundle in a central position.

95

Q

  1. A 20-year-old woman is brought to the Accident & Emergency Department by ambulance after being found unresponsive on the street. Examination shows pinpoint pupils and induration in the right groin. The chest radiograph shows bilateral patchy diffuse air space shadowing predominantly in the middle and upper zones with central peribronchial cuffing. No pleural effusion or pneumothorax seen. The most likely diagnosis is?

(a) Pulmonary oedema secondary to opiate overdose

(b) Acute respiratory distress syndrome

(c) Lung contusion

(d) Renal failure

(e) Fat embolism

A

  1. (a) Pulmonary oedema secondary to opiate overdose

Pin point pupils and right groin infection suggests intravenous drug abuser. Radiographic findings of noncardiogenic pulmonary oedema are non-central, extensive, patchy, bilateral airspace shadowing with indistinct vessels and peribronchial cuffing. Cardiogenic oedema is characterised by cardiac enlargement, pleural effusions, upper lobe venous diversion, Kerley-B lines and peribronchial cuffing.

96

Q

  1. A 65-year-old foundry worker presents with cough and progressive shortness of breath. The chest radiograph shows bilateral upper lobe opacities with multiple hilar lymph nodes showing ‘egg shell’ calcification. What is the most likely diagnosis?

(a) Silicosis

(b) Sarcoidosis

(c) Tuberculosis

(d) Lymphoma

(e) Metastases

A

  1. (a) Silicosis

This appearance can be seen in both silicosis and sarcoidosis; however, given the occupational history, silicosis is the favoured diagnosis. Treated lymphoma can also give egg shell calcification.

97

Q

  1. A 64-year-old woman known to have chronic rheumatoid arthritis presents with shortness of breath. The most common feature seen on the chest radiograph is?

(a) Pleural effusion

(b) Rheumatoid nodule

(c) Diffuse interstitial fibrosis

(d) Bronchiectasis

(e) Pericardial effusion

A

  1. (a) Pleural effusion

This is the most common finding on a chest radiograph in patients with chronic rheumatoid arthritis, seen in more than 90% cases.

98

Q

  1. A 68-year-old man presents with a history of chronic cough. The chest radiograph shows a diffuse reticular nodular pattern with mid-lower zone predominance. HRCT shows a symmetrical reticular pattern with sub-pleural honeycombing and traction bronchiectasis. What is the most likely diagnosis?

(a) Sarcoidosis

(b) Hypersensitivity pneumonitis

(c) Idiopathic pulmonary fibrosis

(d) Asbestosis

(e) Silicosis

A

  1. (c) Idiopathic pulmonary fibrosis

These radiographic and HRCT appearances are typically a feature of idiopathic pulmonary fibrosis. Sarcoidosis affects upper zones predominantly. Hypersensitivity pneumonitis usually spares extreme bases but sometimes may be difficult to separate from idiopathic pulmonary fibrosis. Asbestosis also may show peripheral honeycombing but is associated with pleural plaques.

99

Q

  1. Which of the following are correct about pulmonary sarcoidosis:

(a) Normal CXR excludes the diagnosis.

(b) The large airways are involved in 4-5% of cases.

(c) Subcarinal lymph nodes are characteristically spared.

(d) Fibrosis occurs predominantly in the lower zones.

(e) Pleural effusion is common CXR.

A

Answers:

(a) Not correct.

(b) Correct

(c) Not correct

(d) Not correct

(e) Not correct

Explanation:

Between 5% to 15% of patients have a normal CXR when first examined. Pleural effusion is a rare finding (2%). All mediastinal lymphnodes can be affected in sarcoidosis. On CT 50% of cases show enlarged subcarinal lymphnodes. Middle and upper zone fibrosis is characteristic

100

Q

  1. Which of the following are correct regarding Langerhans’ cell histiocytosis.

(a) Primarily affects cigarette smokers.

(b) Predominantly affects the lung bases.

(c) Most patients are asymptomatic.

(d) Nodular lesions frequently cavitate.

(e) Lung volumes are reduced.

A

Answers:

(a) Correct

(b) Not correct

(c) Not correct

(d) Correct

(e) Not correct

Explanation:

LCH mostly involves upper and mid zones with relative sparing of lung bases and characteristic appearance of bilateral nodular and reticulo-nodular areas. Most patients are symptomatic with non-productive cough and/or dyspnoea. Lung volumes are characteristically normal or increased.

101

Q

  1. When a mass-like lesion is seen on CT, which of the following findings support the diagnosis of rounded atelectasis.

(a) An anteromedial location of the mass.

(b) An acute angle with the pleural margins.

(c) Localized crowding of the pulmonary vasculature.

(d) Adjacent pleural thickening.

(e) Absence of enhancement following intravenous contrast.

A

Answers:

(a) Not correct

(b) Correct

(c) Correct

(d) Correct

(e) Not correct

Explanation:

Round atelectasis is usually seen in posterior or basal region of lower lobes and appears as a well-defined oval or round mass in subpleural location. On Ct the mass shows uniform post intravenous contrast enhancement.

102

Q

  1. Which of the following are true regarding silicosis?

(a) It predominantly affects the lower lobes in acute silicoproteinosis.

(b) Emphysema is associated with the development of progressive massive fibrosis.

(c) It mimics sarcoidosis on high resolution computed tomography (HRCT).

(d) It is a more frequent cause of nodal egg-shell calcification on radiograph than coal miner’s pneumoconiosis.

(e) Impairment of the lung function test correlates best with the profusion of nodules.

A

Answers:

(a) Not correct

(b) Correct

(c) Correct

(d) Correct

(e) Not correct

Explanation:

Acute silicoproteinosis has mid and upper zone predominance occurs from intense exposure to silica dust resulting in alveolar exudates. Impairment of the lung function test correlates best with the degree of emphysematous change. Nodular perfusion is a weaker independent correlate.

103

Q

  1. Which of the following are true regarding usual interstitial pneumonitis (UIP)?

(a) It is more common in females than males.

(b) It occurs most frequently in the sixth decade of life.

(c) It is the most common cause of cryptogenic fibrosing alveolitis.

(d) Areas of ground glass attenuation on HRCT in the absence of parenchymal distortion indicate reversibility.

(e) A confident diagnosis cannot be made on HRCT without lung biopsy.

A

Answers:

(a) Not correct

(b) Correct

(c) Correct

(d) Correct

(e) Not correct

Explanation:

UIP has no gender predilection. On HRCT temporal heterogeneity is characteristic of UIP and it refers to different areas of lung demonstrating different stages of inflammation and fibrosis at the same time. This helps to make confident diagnosis in majority of the cases

104

Q

  1. Which of the following are correct regarding Goodpasture’s syndrome:

(a) Hilar lymph nodes may be enlarged.

(b) Changes are commonly unilateral.

(c) Prognosis is good.

(d) Acute presentation is with air-space consolidation typically at the lung apices.

(e) Signs of renal failure precede pulmonary complaints.

A

Answers:

(a) Correct

(b) Not correct

(c) Not correct

(d) Not correct

(e) Not correct

Explanation:

Goodpasture’s syndrome is bilateral with poor prognosis and death usually within 3 yrs of diagnosis. Pulmonary involvement is before renal involvement with relative sparing of lung apices.

105

Q

  1. Which of the following are correct regarding lymphangioleiomyomatosis (LAM):

(a) Pulmonary abnormalities are similar to those seen in tuberous sclerosis.

(b) It is found exclusively n females.

(c) Cysts commonly have a bizarre outline.

(d) Cysts show sparing of the apices.

(e) There is an association with chylothorax.

A

Answers:

(a) Correct

(b) Correct

(c) Not correct

(d) Not correct

(e) Correct

Explanations:

In LAM cysts are usually uniform and round with uniform distribution. Cysts in LCH have bizarre irregular outlinesand show apical sparing.

106

Q

  1. Which of the following are correct regarding lymphangioleiomyomatosis (LAM):

(a) Pulmonary abnormalities are similar to those seen in tuberous sclerosis.

(b) It is found exclusively n females.

(c) Cysts commonly have a bizarre outline.

(d) Cysts show sparing of the apices.

(e) There is an association with chylothorax.

A

Answers:

(a) Correct

(b) Correct

(c) Not correct

(d) Not correct

(e) Correct

Explanations:

In LAM cysts are usually uniform and round with uniform distribution. Cysts in LCH have bizarre irregular outlinesand show apical sparing.

107

Q

  1. Causes of eggshell calcification of lymph nodes include:

(a) Rheumatoid arthritis.

(b) Silicosis.

(c) Scleroderma.

(d) Histoplasmosis.

(e) Amyloidosis.

A

Answers:

(a) Not correct

(b) Correct

(c) Correct

(d) Correct

(e) Correct

Explanation:

Rheumatoid arthritis rarely shows lymphnodal calcification.

108

Q

  1. Features of pulmonary asbestosis include:

(a) Increased severity in sub-pleural zones.

(b) Hilar adenopathy.

(c) Upper lobe massive fibrosis.

(d) Thickened interlobular septa on HRCT.

(e) Increased incidence of bronchio-alveolar cell carcinoma.

A

Answers:

(a) Correct

(b) Not correct

(c) Not correct

(d) Correct

(e) Correct

Explanation:

Asbestosis shows lower lobe fibrosis with no hilar lymphadenopathy.

Chronic lung & Systemic diseases Flashcards by Waleed Eldahshan (2024)

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