Respiratory

Question 1

Which is false regarding hyaline membrane disease?

a. Surfactant is produced by Type I pneumocytes
b. Associated with maternal diabetes
c. Associated with Caesarian section
d. Steroids increase surfactant production

Answer 1

ANSWER: Surfactant is produced by type II pneumocytes (not type I pneumocytes)

All others are true.

Question 2

Regarding interstitial lung disease, which is false? (April 2013)

a. Connective tissue disorders are associated with UIP
b. Collagen vascular disorders are associated with NSIP
c. RB-ILD is associated with smoking
d. UIP has a better prognosis than NSIP
e. DIP has a better prognosis than UIP

Answer 2

ANSWER: UIP does not have a better prognosis than NSIP

Question 3

Regarding silicosis, which is false? (September 2013)

a. Lower lobe distribution
b. Calcified lymph nodes
c. PMF is in the lower zones
d. Asbestosis is in the lower lobes

Answer 3

Silicosis:
- Fibrotic pneumoconiosis caused by the inhalation of silica particles
- Acute silicosis: Alveolar silicoproteinosis
- Classic silicosis: Chronic interstitial reticulonodular disease
Divided into:
- Simple: pattern of small and round or irregular opacities
- Complicated: large conglomerate opacities which equate to progressive massive fibrosis

Imaging features:
Acute silicosis:
• CXR: bilateral consolidation or ground glass opacification in the perihilar regions
• HRCT: bilateral centrilobular ground glass opacities, multifocal patchy ground glass opacities, consolidation

Classic simple:
• CXR: well-defined and uniform nodular opacities (1-10mm), Upper lobe and posterior lung, Calcified nodules in 10-20%
• HRCT: Multiple small nodules, upper zone predominant with calcifications, Hilar and mediastinal lymphadenopathy, Eggshell pattern calcification

Classic complicated:
• CXR: large, symmetrical bilateral opacities, >1cm with an irregular margin, Middle lung zone or peripheral 1/3 of lung, Gradually migrate towards the hilum, leaving residual emphysematous lung tissue between the fibrotic region and pleural surface
• HRCT: Focal soft tissue masses, often with an irregular or ill-defined margins and calcifications, Surrounded by areas of emphysematous change, Typically upper lobes

ANSWER: Two false responses – silicosis is typically upper mid zone, and PMF is more common in the upper lobes (although can be seen in the apical lower lobes)

Question 4

Which is not a feature of sarcoidosis? (March 2017)

a. Hepatosplenomegaly
b. Miculikz syndrome

Answer 4

Miculikz syndrome:
o Inflammatory enlargement of 2 or more salivary or lacrimal glands with xerostomia
o Considered part of the IgG4 spectrum of disease
- Formerly classified as type 1 Sjogren syndrome
- Not a feature of sarcoidosis

ANSWER: Miculikz syndrome is not a feature of sarcoidosis. Hepatosplenomegaly can be seen in sarcoidosis.

Question 5

Regarding hypersensitivity pneumonitis, which is false? (September 2013)

a. Acute is a type III and type IV hypersensitivity reaction
b. Chronic HP affects the lower lobes

Answer 5

Hypersensitivity pneumonitis
Diagnosis relies on:
	Exposure to an antigen
	Characteristic signs and symptoms
	Abnormal chest findings on physical examination
	Abnormal pulmonary function tests
	Abnormal imaging findings

Pathology:
- Inhalation of triggering particles (1-5 micrometres)
- Chronic inflammation of the bronchi and peribronchial tissue
(Poorly defined granulomas and giant cells in the interstitium and alveoli, May lead to fibrosis and emphysema)

Four histologic features:
• Cellular bronchiolitis
• Diffuse chronic interstitial inflammatory infiltrates
• Poorly circumscribed interstitial non-necrotising granulomas
• Individual giant cells in the aveoli or interstitium

Subtypes:
Acute hypersensitivity pneumonitis:
• Features 4-12 hours after exposure
• Neutrophillic infiltration of the respiratory bronchioles and alveoli
• Pattern of diffuse alveolar damage
• Dominated by airspace abnormalities on imaging
(May be occult on CXR; Homogenous, ground glass alveolar opacities; May have a lower zone predominance)

Subacute hypersensitivity pneumonitis:
• Results from intermittent or continuous exposure to low antigen doses
Pathology:
o Cellular bronchiolitis
o Non-caseating granulomas
o Bronchiolocentric interstitial pneumonitis
o Lymphocyte predominance
Imaging:
• CXR may be normal
• Ground-glass and nodular centrilobular densities
(Mid-lower zone predominance; Air-trapping (reflecting associated bronchiolitis); Thin-walled cysts (uncommon))

Chronic hypersensitivity pneumonitis:
• Radiological and pathological evidence of fibrosis
• Represents the end stage of repeated or persistent hypersensitivity pneumonitis
• HRCT: Centrilobular and peribronchial nodular opacities of varying numbers; Ground glass opacities; Mosaic perfusion caused by bronchiole obstruction; Areas of pulmonary fibrosis (Honeycombing, Traction bronchiectasis, Reticulation)
o Mid-upper zone predominance, with sparing of the lung bases

ANSWER: Chronic HP spares the lower zones, although acute and subacute HP may have a mid-basal distribution of changes

Question 6

Regarding alveolar proteinosis, which is false? (March 2015)

a. Silicosis is associated with secondary alveolar proteinosis
b. Acquired PAP is associated with superimposed infection
c. Acquired PAP is associated with immunosuppression
d. The congenital form requires lung transplant
e. Acquired PAP is associated with smoking

Answer 6

Pulmonary alveolar proteinosis:

• Deposition of PAS+ lipoproteinaceous material in the alveoli as the result of impaired turnover of surfactant
• (GM-CSF) has also been implicated
• Associated with smoking (M>F)
• M=F in non smokers

Divided into three categories:
Idiopathic (90%) – ‘adult’ or ‘acquired’
• Autoimmune condition – IgG antibodies to GM-CSF

Secondary (5-10%)
• Haematological malignancy
• Inhalational lung disease - Silicon (silicoproteinosis), Titanium oxide
• Immunodeficiency/immunosuppression with co-existent infection - Nocardia, aspergillosis, PCP

Congenital (2%)
• Presents in the neonatal period in term babies
• Poor prognosis if left untreated (require lung transplantation)
• Due to mutation in several genes, including GM-CSF receptor

Complications - superinfection:
	Aspergillus
	Candida
	Cryptococcus
	CMV
	Histoplasma
	Myobacterium
	Nocardia
	Pneumocystis
	Pneumococcus

ANSWER: Acquired PAP is not associated with immunosuppression (secondary PAP is associated with immunosuppression)

Question 7

What is associated with allergic alveolar proteinosis? (August 2016)

a. Eosinophilia

Answer 7

Pulmonary eosinophilia
- Rare group of disorders characterized by an infiltration of eosinophils

Categories:
Acute eosinophilic pneumonia with respiratory failure:
•	Acute illness of unknown cause
•	Rapid onset: fever, dyspnea, hypoxic respiratory failure
•	Radiology: diffuse infiltrates
•	Pathology:
	- >25% eosinophils on bronchial lavage
	- Diffuse alveolar damage
•	Good response to steroids

Secondary eosinophilia:
• Causes:
	Infective: parasitic, fungal and bacterial infections
	Hypersensitivity pneumonitis
	Drug allergies
	Asthma
	ABPA
	Vasculitis (Churg-Strauss)

Idiopathic eosinophilia:
• Focal areas of cellular consolidation of the lung substance - Predominantly peripheral distribution
• Clinical: cough, fever, night sweats, dyspnea and weight loss
• Aggregates of eosinophils and lymphocytes within septal walls and alveolar spaces
• Usually occurs with interstitial fibrosis and organizing pneumonia

ANSWER: Alveolar proteinosis is not considered an eosinophilic lung disease

Question 8

What is the correct association? (August 2014)

a. Smoking and RB-ILD
b. Granulomas and immune complexes in Wegener granulomatosis

Answer 8

ANSWER: Smoking and RB-ILD

Question 9

Which is false regarding rheumatoid lung disease? (August 2016)

a. Upper lobe distribution

Answer 9

Pulmonary manifestations of rheumatoid arthritis:
- more common overall in women, however pulmonary manifestations are more common in men

• Interstitial, airway and pleural disease
  Interstitial:
  • Lower zone predominant pattern of fibrosis (10% of RA patients) - UIP pattern slightly more common than NSIP
  • COP
  • Bronchiectasis
  • Large rheumatoid nodules: (single or multiple, Peripherally distributed, May cavitate
  o Cavitating lesions can become complicated by pneumothorax or haemopneumothorax
  • Follicular bronchiolitis (rare)
  • Caplan syndrome – rheumatoid pneumoconiosis
• Pleural:
  • Pleural thickening
  • Pleural effusions can occur late in the disease

ANSWER: Rheumatoid lung disease does not have an upper lobe distribution

Question 10

What is not a cause of interstitial lung disease? (September 2013)

a. Aspirin
b. Bleomycin
c. Busulphan
d. Amiodarone
e. Nitrofurantoin

Answer 10

Causes of drug induced lung disease (ARDS to pulmonary fibrosis):

• Chemotherapy agents
  Patterns of disease:
  • NSIP, HP, ARDS/DAD, Bronchiolitis obliterans, Pulmonary haemorrhage
  Common agents:
  • Bleomycin, Bulsulfan, Cyclophosphamide, Docitaxel, Methotrexate
• Immunosuppressive agents: Sirolimus, Leflunomide
• Cardiovascular agents: Amiodarone (fibrosis and liver deposition)
• Antibiotic agents:
  Nitrofurantoin, Amphotericin B, Sulfonamide, Sulfasalazine
• Anti-inflammatory agents: Gold

- Recreational drugs:
	IV Ritalin (methylphenidate) / herion / cocaine

• Anti-convulsant agents: Phenytoin (eosinophilic pneumonia)

ANSWER: Aspirin is not associated with interstitial lung disease

Question 11

What is least associated with primary tuberculosis? (August 2016)

a. Apical calcified cavitating lesion
b. Pleural effusion
c. Hilar and mediastinal lymphadenopathy
d. Pulmonary miliary disease
e. Systemic miliary disease

Answer 11

Primary pulmonary tuberculosis manifests as four main entities:
o Parenchymal disease: Usually dense, homogenous parenchymal consolidation in any lobe. Predominance in the lower and middle lobes (subpleural), especially in adults
o Lymphadenopathy
o Miliary opacities
o Clustered parenchymal calcification (galaxy sign)
o Pleural effusion

Secondary tuberculosis:
o Lung apex lesion: ill defined patchy consolidation which develops cavitation, Course reticulonodular densities, Tree-in-bud spread
o Healing results in fibrosis, volume loss and traction bronchiectasis
o Lymphadenopathy in 5%, pleural effusions in 18%
o Chest wall involvement may occur from direct extension

ANSWER: Apical calcified cavitating lesion – this is seen in secondary tuberculosis

Question 12

Which is true regarding tuberculosis? (March 2016)

a. Ghon focus refers to both the lung focus and the lung node
b. Caseous necrosis does not commonly occur with active bacilli and therefore one should biopsy a necrotic lymph node
c. Pleural effusions are more common in primary TB
d. Progressive primary TB refers to primary TB then going onto secondary TB

Answer 12

ANSWER: Pleural effusions are more common in primary TB

Question 13

Which is the least likely cause of secondary activation of tuberculosis? (March 2016)

a. Asbestosis
b. Silicosis

Answer 13

At risk groups for tuberculosis:
o Immunocompromised
o Close contact with active tuberculosis
o Malnourishment (including alcoholic and IVDU patients)
o Endemic countries
o Workers and residents in long term residential facilities

High risk factors for reactivation:
o	HIV/AIDS
o	Close contacts
o	Organ transplant recipients
o	Chronic renal failure requiring dialysis
o	TNF-alpha blockers
o	Silicosis

Moderate risk factors for reactivation:
o	Fibronodular disease on CXR
o	Healthcare workers
o	Prisoners, homeless, IVDU
o	Immigrants from high prevalence countries

Low risk factors for reactivation:
o	Diabetes mellitus
o	Smoking
o	Use of corticosteroids
o	Underweight patients

ANSWER: Asbestosis is not a risk factor/cause of secondary activation of tuberculosis. Silicosis is a high risk factor for reactivation.

Question 14

Regarding PCP/PJP, which is true?

a. Apical distribution

Answer 14

Pneumocystis:
o Atypical yeast like fungus
o Histology: intra-alveolar eosinophilic masses with a foamy appearance due to the small cysts which harbor the organism

Epidemiology:

• Virtually never present in immunocompetent patients
• HIV, haematological malignancy or bone marrow transplant patients

Imaging findings:
- XR: small pneumatocoeles, subpleural blebs, fine reticulation, usually a perihilar distribution
• 90% of patients will have an abnormal CXR, however 30% will have non-specific or inconclusive findings
- CT: Ground glass pattern,
• Predominantly perihilar or midzones, May have an upper zone predominance if the patient is on aerosolized prophylaxis as this area is not well ventilated, Peripheral sparing in 40%
• Reticular opacities or septal thickening - May form a crazy paving pattern with the ground glass opacification
• Pneumatocoeles (30%)
• Pleural effusions uncommon (<5%)
• Lymphadenopathy is uncommon (10%)

ANSWER: PCP/PJP may demonstrate an apical predominant pattern in a patient who is on aerosolized prophylaxis due to the poor ventilation of the upper lobes

Question 15

Regarding aspergillosis, which is true? (March 2017)

a. ABPA is caused by superficial colonization of bronchial mucosa
b. The halo sign is caused by an expanding ring of gelatinous exudate

Answer 15

ANSWER: ABPA is caused by superficial colonization of the bronchial mucosa – it is a hypersensitivity reaction to the presence of the fungus

Question 16

Which is true regarding aspergillosis? (March 2017)

a. It can present similarly to mucormyocosis

Answer 16

ANSWER: Mucormycosis is an invasive fungal infection, aspergillosis can also have aggressive features

Question 17

Which malignancy is not associated with smoking? (September 2013, March 2015, March 2016)

a. Carcinoid (classic)
b. Adenocarcinoma with lepidic growth pattern
c. Invasive adenocarcinoma
d. Small cell lung carcinoma
e. Squamous cell lung carcinoma

Answer 17

ANSWER: Bronchial carcinoid is not associated with smoking

Question 18

Which is not a paraneoplastic syndrome associated with lung cancer? (March 2015)

a. Lambert-Eaton
b. Hypercalcaemia
c. Addison’s disease
d. Hyperparathyroidism
e. Gynaecomastia
f. ADH

Answer 18

• Paraneoplastic syndromes are encountered in 15% of cancer patients
• Small cell lung cancer most commonly associated

Paraneoplastic syndromes:
Endocrine syndromes:
	Carcinoid syndrome (GI with liver metastases or lung primary)
	Cushing syndrome
	Hypercalcaemia (most common)
	Hypoglycaemia
	SIADH

Neurological syndromes:
	Encephalitis
	Lambert-Eaton myasthenia syndrome
	Limbic encephalitis
	Optic neuropathy
	Cerebellar degeneration

Rheumatological/dermatological syndromes:
 Acanthosis nigracans
 Dermatomyositis
 HPOA
 Leukocytoclastic vasculitis
 Paraneoplastic pemphigus
 Sweet syndrome - Acute febrile neutrophilic dermatosis, Most commonly related to AML

Haematologic syndromes:
 Good syndrome

Miscellaneous:
 Stauffer syndrome - Nephrogenic hepatomegaly in the absence of metastases. Most commonly seen with RCC
 Gynaecomastia - Oestrogenic tumours, Adrenal carcinoma, hepatoma, lung cancer, pituitary adenoma, testicular cancer

ANSWER: Addison’s disease – more likely to be caused by metastases to the adrenal gland

Question 19

All of the following are known paraneoplastic syndromes except: (March 2014)

a. Diabetes insipidis

Answer 19

ANSWER: Diabetes insipidis is not a recognized paraneoplastic syndrome

Question 20

Which is true regarding small cell lung cancer? (August 2014)

a. Typical carcinoid is not a precursor
b. Associated with raised PTH and calcium

Answer 20

ANSWER: Associated with raised PTH and calcium – most common malignancy associated with paraneoplastic syndromes; typical carcinoid is not a precursor lesion to small cell lung cancer

Question 21

Which is true regarding lung cancer? (March 2015)

a. EGFR mutation confers sensitivity to chemotherapy
b. Malignant cells are rarely found in associated pleural fluid
c. Squamous cell cancer is more often peripheral than central
d. Calcifications commonly occur in SCC

Answer 21

• EGFR mutation positive lung cancers can be targeted with EGFR receptor inhibitor chemotherapy agents e.g. erlotinib (Tarceva)
• Pleural involvement of malignancy:
  o Direct extension e.g. from primary lung cancer
  o Primary tumour e.g. mesothelioma, primary pleural lymphoma
  o Pleural metastases
• Pleural effusions are not always directly caused by tumour:
  o Intra-parenchymal tumours may cause lymphatic or venous obstruction with a resultant pleural effusion
  o Reactive parapneumonic effusion

Squamous cell lung cancer:
o 30-35% of lung cancers, most commonly associated with smoking
o Location: Traditionally more common centrally (60-90%) but the incidence of peripheral cancers is increasing
- Central cancers: Symptoms from invasion or obstruction of central structures (vessels and bronchi). Patients may have a chronic cough and haemoptysis
- Peripheral cancers: Larger at detection, may invade the chest wall. Most common cause of superior sulcus tumours (Pancoast tumours)

Histological subtypes:
	Papillary
	Clear cell
	Small cell (distinct from small cell lung cancer)
	Basaloid

• Most common sites of metastatic disease: adrenal glands, brain, bone, liver
• The most common imaging feature is cavitation (82%), calcification is not typical

ANSWER: EGFR mutation confers sensitivity to chemotherapy

Question 22

What is false regarding lung carcinoid? (August 2016)

a. A few carcinoid cells in the lung scar tissue is not a worrisome finding?

Answer 22

Neoplasms of neuroendocrine cells in the lung:

• Tumorlets: small inconsequential, hyperplastic nests of neuroendocrine cells seen in areas of scarring or chronic inflammation
  
  • 5mm or less. Almost always benign
• Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: precursor to the development of multiple tumorlets or typical or atypical carcinoids
• Carcinoids
• Small cell carcinoma and large cell neuroendocrine tumours (highly aggressive)

ANSWER: A few carcinoid cells in lung scar tissue suggests that this is a tumorlet, which is almost always benign

Question 23

Regarding lung hamartomas which is false? (September 2013)

a. Central
b. Peripheral
c. Generally regarded as benign
d. Carcinoid arises from neurosecretory cells

Answer 23

Lung hamartomas
Pathology:
- Chondromatous (most common), leiomyomatous or mixed
- Mature components of normal lung tissue which are abnormally arranged
- Lipomatous hamartomas: when principally fat (uncommon)

Location:

• Majority peripheral (>90%)
• Endobronchial hamartomas (<5%)

Pulmonary mesenchymal cystic hamartomas:

• Rare subtype of pulmonary hamartomas
• Multiple bilateral cysts and nodules
  
  • Thin walled cysts lined by normal respiratory epithelium. Benign but rarely undergo sarcomatous transformation

ANSWER: Hamartomas do not typically arise centrally (<5%)

Question 24

Which of the following is false? (September 2013)

a. Small cell is peripheral
b. Adenocarcinoma has equal incidence in males and females
c. Squamous cell is central

Answer 24

• Small cell carcinoma and squamous cell carcinoma are more common centrally
• Adenocarcinoma is more common peripherally

Adenocarcinoma epidemiology:

• majority are associated with smoking
  
  • Increasing incidence of cancer in patients who have never smoked (or been exposed to second hand smoke) – more likely to be female and have adenocarcinoma
• Adenocarcinoma is more common in males overall, however is the most common lung cancer subtype in females

ANSWER: Small cell carcinoma is not peripheral

Question 25

Which is false regarding pleural effusions? (March 2014)

a. Haemorrhagic effusion is present in renal causes
b. Transudative effusion in liver causes
c. Empyema is defined as a collection of pus with spread of infection from the lung
d. Haemothorax is a collection of blood associated with trauma
e. Chylothorax is a collection of lymph fluid due to obstruction of the thoracic duct

Answer 25

Causes of a transudative pleural effusion
o CCF
o Cirrhosis (transdiaphragmatic movement of ascites)
o Atelectasis (decreased pleural pressure)
o Peritoneal dialysis (transdiaphragmatic movement of ascites)
o Hypoalbuminaemia (changed oncotic pressure)
o Nephrotic syndrome (hypoalbuminaemia, hypervolaemia, increased hydrostatic pressures)
o Urinothorax (transdiaphragmatic movement of urine leak)
o Post partum (hypervolaemia and increased hydrostatic pressure from valsalva)
o Central line placement in the pleural space
o Myxoedema (heart failure or pneumonia)

ANSWER: Pleural effusions from renal causes (e.g. nephrotic syndrome) are typically transudative

Question 26

Which condition is least likely to be complicated by pneumothorax? (August 2014)

a. Alpha 1 anti-trypsin
b. Ehlers-Danlos
c. Marfan syndrome
d. Kleinfelter syndrome
e. Homocystinuria

Answer 26

Conditions associated with spontaneous pneumothorax:
o	Alpha 1 anti-trypsin
o	Ehlers-Danlos
o	Marfan syndrome
o	Homocystinuria

Secondary causes of pneumothorax:
o Cystic lung diseases
	Blebs/bullae
	Emphysema/asthma
	PJP pneumonia 
	End stage ILD with honeycombing
	Lymphangiomyomatosis
	LCH
	Cystic fibrosis
	Ankylosing spondylitis
o Parenchymal necrosis
	Cavitating/necrotizing infection
	Cavitating neoplasm
	Cystic fibrosis
o Other
	Catamenial pneumothorax: pleural endometrioma
	Pleuroparenchymal fibroelastosis

ANSWER: Kleinfelter syndrome does not give increased risk of pneumothorax

Question 27

Which of these is least associated with mesothelioma? (March 2015)

a. Serpentine chrysotile
b. Ambiphole (crocidolite)
c. Erionite
d. SV40 virus
e. Vinyl chloride

Answer 27

Aetiology of mesothelioma – exposures:
o Asbestos fibres: Majority crocidolite. Serpentine crysotile much less commonly associated
o Erionite fibres:Building material (most commonly used in Turkey)
o Simian virus 40 (SV40)
o Radiation exposure

• Risk of mesothelioma is not increased with smoking
- Markedly increased risk of lung carcinoma in smokers who have been exposed to asbestos

• PVC implicated in angiosarcomas of the liver and spleen

ANSWER: Vinyl chloride is least associated with mesothelioma

Question 28

Regarding mesothelioma, which is true? (August 2016)

a. It is difficult to differentiate from metastatic adenocarcinoma, microscopically and macroscopically

Answer 28

Mesothelioma
o Epithelioid type can resemble metastatic adenocarcinoma
- Distinguished with specific immunohistochemical stains including calretin and cytokeratin S

o Types:
Epithelioid: more favourable prognosis
Sarcomatoid or mixed: worse prognosis

ANSWER: Mesothelioma is difficult to distinguish from adenocarcinoma microscopically and macroscopically – immunohistochemical stains can be used

Question 29

Which is true regarding pleural mesothelioma? (March 2014)

a. Smoking is associated
b. It can invade the mediastinum
c. It is commonly associated with asbestosis
d. Metastases are common at presentation

Answer 29

• Smoking is not associated with mesothelioma, however it does increase the risk of asbestos associated lung cancer
• Mesothelioma is locally invasive and can involve the mediastinum
• Asbestosis is present in 10% of patients with mesothelioma
- However > 90% have exposure to amphibole type asbestos
• Metastases to lymph nodes and distant organs occur late

ANSWER: Mesothelioma can invade the mediastinum

Question 30

Regarding CPAM, which is true? (September 2013)

a. Has a well defined bronchial tree
b. Supplied by systemic arteries
c. Usually contains cystic components
d. 5% of all congenital lung diseases
e. It most commonly appears as a homogenous lung mass

Answer 30

Congenital pulmonary airway malformations
o Multi-cystic masses of segmental lung tissue with abnormal bronchial proliferation
- Considered part of the spectrum of bronchopulmonary foregut malformations
o 25% of congenital lung lesions (1:1500-1:4000 live births)

Pathology:

• Failure of normal bronchoalveolar development
• Hamartomatous proliferation of terminal respiratory units in a gland-like pattern
  
  • Proliferation of bronchiole-like structures and macro/microcysts lined by columnar and cuboidal epithelium
  • Absence of cartilage and normal glands
• No formation of normal alveoli
• Have intracystic communications
• Can have a connection to the tracheobronchial tree
• Five subtypes 0 - IV

ANSWER: CPAMs usually contain cystic components

Question 31

20 week old, anomaly scan. In the left side of the chest there is a heterogenous mass with mediastinal shift to the right. No stomach seen in the LUQ. (September 2013)

a. Congenital diaphragmatic hernia
b. CPAM
c. Bronchopulmonary sequestration

Answer 31

ANSWER: Congenital diaphragmatic hernia

Question 32

What is the most common location for bronchopulmonary sequestration? (September 2013)

a. RUL
b. RML
c. RLL
d. LUL
e. LLL

Answer 32

Pulmonary sequestration:

• Abberant formation of segmental lung tissue that has no connection with the bronchial tree or pulmonary arteries (systemic arterial supply)
• Considered part of the spectrum of broncho-pulmonary foregut malformations
• No genetic predisposition

Types:
Intralobar sequestration (75-85%)
• Present later in childhood with recurrent infections
• Morphology: Closely connected to the adjacent normal lung and do not have a separate pleura. Venous drainage usually to the pulmonary veins but can be to the azygous system, portal system, right atrium or inferior vena cava

Extralobar sequestration (15-25%)
• Usually present in the neonatal period with respiratory distress, cyanosis or infection
• More common in males
• Morphology: Separated from the surrounding lung by a separate pleural covering. Venous drainage variable, but most commonly to the right atrium by systemic vessels

Location:
 Intralobar: 60% LLL, 40% RLL
 Extralobar: almost always LLL, 10% subdiaphragmatic

ANSWER: Bronchopulmonary sequestrations most commonly affect the LLL

Question 33

A patient has calcification in a centrilobular distribution. What is the most likely cause? (August 2016, March 2017)

a. Metastatic calcification
b. Alveolar microlithiasis
c. Haemosiderosis

Answer 33

Metastatic pulmonary calcification
o Calcium deposition in normal lung pulmonary parenchyma
- Centrilobular ground glass nodular opacities which may or may not appear calcified
- Most marked in the upper lobes
- Pulmonary opacities will show increased uptake on bone scan

Causes:
	Chronic renal failure (most common)
	Primary or secondary hyperparathyroidism
	Sarcoidosis
	Vitamin D intoxication
	Intravenous calcium therapy
	Multiple myeloma
	Massive osteolyosis (caused by metastases)

Alveolar microlithiasis
o Rare, idiopathic condition characterized by widespread intra-alveolar deposition of spherical phosphate microliths

Idiopathic pulmonary siderosis:
o Triad of haemoptysis, iron deficiency anaemia and diffuse pulmonary infiltrates (diffuse pulmonary haemorrhage)

ANSWER: Metastatic pulmonary calcification

Question 34

Regarding cystic fibrosis, which is most likely? (August 2016)

a. Disturbance of chloride channel movement
b. Disturbance of sodium transport
c. Disturbance of anion transport

Answer 34

Cystic fibrosis is due to a defect of the cystic fibrosis transmembrane regulator (CFTR) gene on the long arm of chromosome 7.

- Regulates passage of chloride across cell membranes
- Delta-F508 is the most common mutation of the CTFR gene (66-70%)

CTFR:
o Skin: responsible for influx of chloride and increases sodium channel activity
- increases the salt content of sweat (thus the sweat test)
o Other tissues: responsible for efflux of chloride and inhibition of sodium channel activity

CF: too much sodium is resorbed and too little chlorine is pumped out, leading to increased iso-osmotic water resorption from the lumen
• Dries out secretions or markedly increases their thickness

ANSWER: Disturbance of chloride channel movement

Question 35

Regarding cystic fibrosis and the union of two carriers, which of the following is true? (September 2013)

a. 2 heterozygotes without the disease have a 25% chance of an offspring with CF
b. 2 homozygotes without the disease have a 25% chance of an offspring with CF
c. 2 homozygotes with or without the disease have a 50% chance of an offspring with CF

Answer 35

ANSWER: 2 heterozygote parents have a 25% chance of an affected child (CF is autosomal recessive, this follows Mendelian inheritance patterns)

Question 36

Which is false of cystic fibrosis? (September 2013)

a. Defect of sodium transport channel
b. The most common mutation is ΔF508
c. Defect is on chromosome 7
d. Associated with digestive enzyme deficiencies
e. Associated with pseudomonas lung infection

Answer 36

ANSWER: CF is not associated with a defect of the sodium transport channel, it is associated with a defect of the chloride transport channel

Question 37

Which is not an area which is typically involved in Langerhans cell histiocytosis? (September 2013)

a. Hypothalamic pituitary axis
b. Large bronchi and airways
c. Orbit
d. Calvarium
e. Lung interstitim/parenchyma

Answer 37

ANSWER: Large bronchi and airways are not typically involved in LCH

Question 38

Chronic bronchitis may be confidently diagnosed in which of the following setting:

1. A fixed (nonreversible) restrictive lung defect with normal total lung volume
2. A cough productive of sputum for 3 months of 2 consecutive years
3. COPD in the absence of emphysema or asthma
4. Bronchial mucous gland to wall thickness of more than 0.4
5. Clinical evidence of inflammation without active infective or histological evidence of chronic inflammation (plasma cell/ lymphocytic infiltrate)

Answer 38

2. *A cough productive of sputum for 3 months of 2 consecutive years

Question 39

Which of the following is LEAST likely to associated with SLE

1. Pulmonary artery filling defect
2. Pericardial effusion
3. Patchy transient pulmonary infiltrate
4. Bronchopleural fistula
5. Pleural effusion

Answer 39

4. *Bronchopleural fistula

Question 40

Which of the following is LEAST likely to be associated of rheumatoid arthritis?

1. Pulmonary hypertension
2. Bilateral tracheal / hilar lymphadenopathy
3. Pleural effusion
4. Subpleural basal honeycombing
5. 1 – 2 cm lung parenchymal nodule

Answer 40

2. *Bilateral tracheal / hilar lymphadenopathy

Question 41

Which of the following is correct regarding panacinar emphysema?

1. There is diffuse uniform enlargement of acini from the respiratory bronchiol to the acini
2. It involves the whole lung uniformly.
3. It classically is most dominant in the mid and upper zones
4. It is most marked in the proximal bronchioles and proximal acini
5. It is the commonest cause of apical blebs

Answer 41

1. *There is diffuse uniform enlargement of acini from the respiratory bronchiole to the acini

Question 42

Which of the following is LEAST correct concerning asbestos exposure?

1. Pleural effusion without malignancy or infection is a recognised manifestation
2. While the incidence of lung carcinoma is increased in smokers it is also increased in nonsmokers
3. The incidence of lung carcinoma is increased by up to 55 fold in smokers
4. The extent of pleural plaques correlates well with the extent of asbestos exposure
5. Pleural plaques do NOT (or rarely) contain features diagnostic of asbestos exposure on biopsy.

Answer 42

4. *The extent of pleural plaques correlates well with the extent of asbestos exposure

Question 43

A clinical requests states “abundant eosinophils on bronchoscopy”, which of the following is most correct?

1. This can be seen with hypersensitivty pneumonitis
2. This can be seen with fungal lung infections
3. This can be seen with drug reactions
4. This can be seen with tuberculosis
5. This can be seen with Churg-Straus Syndrome

Answer 43

4. *This can be seen with tuberculosis

Question 44

Which of the following conditions is LEAST associated with pulmonary hypertension?

1. History of rheumatic fever
2. Rheumatoid arthritis without pulmonary fibrosis
3. Obstructive sleep apnoea
4. Emphysema
5. Untreated small cell lung carcinoma without emboli

Answer 44

5. *Untreated small cell lung carcinoma without emboli

Question 45

Concerning Histoplasmosis, which of the following is most correct?

1. It is an indolent saprophytic capsulated gram negative bacterium
2. It has many patterns of infection including a self-limited latent pulmonary infection to disseminated disease affecting adrenals liver and meninges
3. It is not seen in immunocompetent individuals
4. It is not a recognised cause of granulomas
5. The pattern of lung infection is similar to Strep. Pneumonia

Answer 45

2. *It has many patterns of infection including a self-limited latent pulmonary infection to disseminated disease affecting adrenals liver and meninges

Question 46

Which of the following statements is LEAST correct?

1. An apical lung carcinoma may present with ulnar nerve pain and Horner Syndrome
2. Lung carcinoma may cause Lambert-Eaton syndrome (muscle weakness similar to Myasthenia Gravis)
3. While varying with subtype, 5 Year survival is ~20% with regional lymphasdenopathy and less than 5% with distant metastasis
4. Hypercalcaemia indicates widespread bone metastasis
5. The T staging may be affected by encroachment on the mainstem closer than 2 cm, parietal pericardial involvement, diagram involvement and phrenic nerve involvement

Answer 46

4. *Hypercalcaemia indicates widespread bone metastasis

Question 47

Concerning Pseudomonas infections which of the following is most correct

1. It accounts for less than 1-5% of cases severe otitis externa (“malignant otitis externa”) in diabetics.
2. It is an uncommon pathogen in Cystic Fibrosis patients (<1-5%).
3. It causes a predominate interstital pneumonia due to type II pneumocyte toxicity with scant exudate
4. It is a common skin infection in burns patients, where severe infection frequently leads to DIC.
5. It is a common mass forming commensal in lung cavities.

Answer 47

4. *It is a common skin infection in burns patients, where severe infection frequently leads to DIC.

Question 48

A new AI programme has suggested “possible Aspergillus Infection” on 4 CT completely different scans – including a non-haemorrhagic nodule within an apical lung cavity / central bronchiectasis in a steroid dependant asthmatic / a haemorrhagic pneumonia chemotherapy patient/ and destructive rhinocerbral infection in leukemic patient. Which of the following is most correct?

1. The nodule within cavity is likely fungal rather than aspergillus: the AI programme is in error
2. The bronchiectasis is likely secondary to asthma alone and any aspergillus found is likely an oral contaminant
3. A haemorrhagic pneumonia in a chemotherapy patient strongly implies a platelet abnormality rather than Aspergillus Infection
4. Aspergillus is not an accepted cause of destructive rhinocerbral infection
5. The AI statements are potentially correct and appear reasonable

Answer 48

5. *The AI statements are potentially correct and appear reasonable

Question 49

Concerning embolic disease, which of the following is LEAST correct?

1. Over 90% of PE arises from leg veins
2. The incidence has more than tripled since 1970, (and comparing age/diagnosis matched patients)
3. Associated pulmonary haemorrhage almost always (>95%) indicates pulmonary infarction
4. Leg DVT cannot result in cerebral emboli in the absence of pulmonary AVM
5. 10 – 25% of PE is clinically silent.

Answer 49

3. *Associated pulmonary haemorrhage almost always (>95%) indicates pulmonary infarction