Chronic lung & Systemic diseases Flashcards

Question

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

Answer 1

(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.

Answer 2

(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.

Answer 3

(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.

Answer 4

(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.

Answer 5

(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.

Answer 6

(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.

Answer 7

(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.

Answer 8

(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.

Answer 9

(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.

Answer 10

(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.

Answer 11

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.

Answer 12

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.

Answer 13

4. 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

Answer 14

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

Answer 15

18. a. Pleural effusion Whilst all the above are seen in SLE, pleural effusions are the commonest radiographic abnormality

Answer 16

34. 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

Answer 17

42. 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).

Answer 18

43. 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

Answer 19

44. a. Lymphangioleiomyomatosis (LAM) The cysts in LAM are thin-walled air-containing cysts, rather than cavitation in nodules

Answer 20

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

Answer 21

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

Answer 22

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

Answer 23

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

Answer 24

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

Answer 25

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

Answer 26

D Sarcoidosis

Answer 27

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

Answer 28

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).

Answer 29

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.

Answer 30

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

Answer 31

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

Answer 32

3. 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

Answer 33

7. 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.

Answer 34

22. 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

Answer 35

27. 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 of individual 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

Answer 36

32. 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

Answer 37

37. 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.

Answer 38

38. 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 basement 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

Answer 39

40. 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, homogenous 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

Answer 40

48. 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%.

Answer 41

59. 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

Answer 42

65. 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.

Answer 43

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

Answer 44

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

Answer 45

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

Answer 46

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

Answer 47

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

Answer 48

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

Answer 49

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.

Answer 50

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.

Answer 51

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.

Answer 52

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.

Answer 53

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.

Answer 54

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.

Answer 55

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.

Answer 56

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.

Answer 57

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.

Answer 58

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.

Answer 59

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.

Answer 60

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.

Answer 61

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.

Answer 62

1. (b) Asbestosis All the other given options are known to cause egg shell calcification of the hilar lymph nodes.

Answer 63

3. (e) Scleroderma Other conditions cause predominantly upper zone disease.

Answer 64

4. (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.

Answer 65

6. (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.

Answer 66

7. (d) Idiopathic pulmonary fibrosis These are typical radiographic and HRCT features of idiopathic pulmonary fibrosis.

Answer 67

9. (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.

Answer 68

10. (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

Answer 69

11. (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.

Answer 70

12. (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.

Answer 71

15. (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.

Answer 72

18. (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.

Answer 73

45. (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.

Answer 74

48. (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.

Answer 75

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

Answer 76

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.

Answer 77

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.

Answer 78

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.

Answer 79

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

Answer 80

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.

Answer 81

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.

Answer 82

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.

Answer 83

Answers: (a) Not correct (b) Correct (c) Correct (d) Correct (e) Correct Explanation: Rheumatoid arthritis rarely shows lymphnodal calcification.

Answer 84

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

(108 cards)