Interstitial Lung Disease

Question 1

What does interstitial lung disease present as early

Answer 1

Alveolitis, injury with inflammatory cell infiltration

Question 2

Late stage interstitial lung disease characterized by

Answer 2

Fibrosis

Question 3

What exemplifies interstitial lung disease

Answer 3

Adult respiratory distress syndrome

Question 4

Cause of interstitial lung disease

Answer 4

Environmental - Minerals, drugs, radiation
Hypersensitivity - Mouldy hay, avian protein
Idiopathic

Question 5

What biopsy is more reliable

Answer 5

Thoracoscopic biopsy > Transbronchial biopsy

Question 6

What is idiopathic pulmonary fibrosis

Answer 6

Cryptogenic fibrosing alveolitis (CFA) or usual interstitial pneumonia (UIP). Unknown cause of progressive interstitial fibrosis of unknown cause

Question 7

What is pulmonary interstitium

Answer 7

Alveolar lining cells - Type 1 and 2. Consists of elastin-rich thin connective tissue containing capillary blood-vessels

Question 8

Histological features of interstitial pulmonary fibrosis

Answer 8

Honeycombing which is dilated spaces surrounded by fibrous walls

Question 9

What is extrinsic allergic alveolitis

Answer 9

Also known as hypersensitivity pneumonitis

Causes chronic inflammation of small airways, interstitium and occasional granulomas

Question 10

Allergic origin in extrinsic allergic alveolitis

Answer 10

Hypersensitivity pneumonitis is of type 3 and 4

Question 11

Various types of extrinsic allergic alveolitis

Answer 11

Farmers lungs - Thermophillic bacteria
Bird fanciers lungs - Avian protein
Malt workers lungs - Fungi

Question 12

What is sarcoidosis

Answer 12

Multisystem granulomatous disorder of unknown cause

Question 13

Hypersensitivity involved in sarcoidosis

Answer 13

Type 4 hypersensitivity

Question 14

What type of granuloma histolically is positive for recovery

Answer 14

Granuloma without necrosis

Question 15

Nonspecific interstitial pneumonitis vs idiopathic pulmonary fibrosis

Answer 15

Nonspecific interstitial pneumonitis is often present with autoimmune conditions such as rheumatoid arthritis

Question 16

How can rheumatoid nodules be used to differentiate from TB

Answer 16

Rheumatoid nodules look histologically like TB however there are no pathogens present

Question 17

What is pneumoconiosis

Answer 17

Lung disease due to inhalation of mineral dust such as asbestos/coal/silicon. Characterized by inflammation, coughing and fibrosis

Question 18

Differentiate between types of asbestos

Answer 18

Serpentine (curved) asbestos fibres relatively safe

Straight (amphibole) asbestos fibres highly dangerous

Question 19

What can asbestos exposure lead to

Answer 19

Parietal pleura plaque on diaphragm or lungs, interstitial fibrosis (asbestosis), bronchial carcinoma, mesothelioma

Question 20

What are interstitial disease

Answer 20

Any disease process affecting the lung interstitium, Presents with restrictive lung pattern often with breathlessness and dry cough

Question 21

What type of hypersensitivity is sarcoidosis

Answer 21

Type 4 hypersensitivity

Question 22

What does sarcoidosis cause

Answer 22

Non-caseating granuloma of unknown aetiology in lungs, lymph nodes, joints, skins, eyes, liver

Question 23

What can acute sarcoidosis cause

Answer 23

Erythema nodosum, bilateral hilar lymphadenopathy, arthritis, uveitis, parotitis, fever

Question 24

What can chronic sarcoidosis cause

Answer 24

Lung infiltrates (alveoli), skin infiltrates, peripheral lymphadenopathy, hypercalcaemia, organomegaly

Question 25

Differential diagnosis of sarcoidosis

Answer 25

TB, (tuberculin test), lymphoma, carcinoma, fungal infection

Question 26

Diagnosis sarcoidosis

Answer 26

Chest x-ray, CT scan, tissue biopsy for non-caseating granuloma, blood tests (raised calcium, increased inflammatory markers), spirometry (restrictive pattern)

Question 27

Management of sarcoidosis

Answer 27

Acute - Self-limiting | Chronic - Oral steroids if vital organs affected, immunosuppression

Question 28

What type of hypersensitivity is extrinsic allergic alveolitis

Answer 28

Type 3 hypersensitivity

Question 29

Cause of extrinsic allergic alveolitis

Answer 29

Called hypersensitivity pneumonitis - | Idopathic, thermophillic actinomycetes, avian antigens, drugs

Question 30

Symptoms of acute extrinsic allergic alveolitis

Answer 30

Cough, dyspnoea, myalgia, pyrexia | Signs are crackles (no wheeze), hypoxia and widespread pulmonary infiltrates on chest x-ray

Question 31

Treatment of acute EAA

Answer 31

Oxygen, steroids and antigen avoidance

Question 32

Symptoms of chronic extrinsic allergic alveolitis

Answer 32

Due to repeated exposure to low dose antigen Progressive breathlessness, cough May have crackles, clubbing unusual X-ray has pulmonary fibrosis mainly in upper zone

Question 33

Treatment of chronic EAA

Answer 33

Remove antigen exposure, oral steroids if dyspnoea or low gas transfer

Question 34

Signs and symptoms of idiopathic pulmonary fibrosis

Answer 34

Progressive breathlessness, dry cough, finger clubbing, bilateral fine inspiratory crackles

Question 35

Spirometry of idiopathic pulmonary fibrosis

Answer 35

Restrictive defect, low FEV1 and FVC, normal or raised FEV1/FVC ratio with reduced lung volumes and low gas transfer

Question 36

CT scan observation of idiopathic pulmonary fibrosis

Answer 36

Reticulonodular fibrotic shadowing worse at lung base and periphery. Traction bronchiectasis with honey-combing cystic change.

Question 37

What are signet ring cells

Answer 37

Cells with a large vacuole often seen in carcinomas

Question 38

Treatment of idiopathic pulmonary fibrosis

Answer 38

Steroids and immunosuppressants do not change the course of the disease. Antifibrotic drugs such as Pirfenidone and Nintedanib slow down disease progression but expensive and many side effects. Also doesn't reverse fibrosis, only slows progression Lung transplant in younger patients, oxygen if hypoxic

Question 39

Prognosis of idiopathic pulmonary fibrosis

Answer 39

Most progress to respiratory failure, 4 years median survival from diagnosis

Question 40

Symptoms of simple coal workers pneumoconiosis

Answer 40

No symptoms generally are seen, chest x-ray abnormality present without impairment of lung function

Question 41

Symptoms of complicated coal workers pneumoconiosis

Answer 41

Progressive massive fibrosis with a restrictive pattern of breathlessness.

Question 42

What is Caplan's syndrome

Answer 42

Rheumatoid arthritis patient exposed to coal dust, leads to the formation of pulmonary nodules - Rheumatoid pneumoconiosis

Question 43

Simple vs complicated silicosis

Answer 43

Simple - Few symptoms, chest x-ray abnormality (eggshell calcification of hilar nodes) Chronic - Restrictive pattern, pulmonary fibrosis

Question 44

How does asbestos affect pleural lining

Answer 44

Benign pleural plaque - Asymptomatic Acute asbestos pleuritis - Fever, pain, bloody pleural effusion Pleural effusion and diffuse pleural thickening - Restrictive impairment Malignant mesothelioma - Incurable pleural cancer

Question 45

Asbestos bodies in sputum and lung biopsy

Answer 45

Asbestosis, due to prolonged exposure. Causes pulmonary fibrosis and restrictive defect