Interstitial Lung Disease Flashcards

1
Q

What does interstitial lung disease present as early

A

Alveolitis, injury with inflammatory cell infiltration

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2
Q

Late stage interstitial lung disease characterized by

A

Fibrosis

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3
Q

What exemplifies interstitial lung disease

A

Adult respiratory distress syndrome

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4
Q

Cause of interstitial lung disease

A

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

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5
Q

What biopsy is more reliable

A

Thoracoscopic biopsy > Transbronchial biopsy

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6
Q

What is idiopathic pulmonary fibrosis

A

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

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7
Q

What is pulmonary interstitium

A

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

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8
Q

Histological features of interstitial pulmonary fibrosis

A

Honeycombing which is dilated spaces surrounded by fibrous walls

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9
Q

What is extrinsic allergic alveolitis

A

Also known as hypersensitivity pneumonitis

Causes chronic inflammation of small airways, interstitium and occasional granulomas

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10
Q

Allergic origin in extrinsic allergic alveolitis

A

Hypersensitivity pneumonitis is of type 3 and 4

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11
Q

Various types of extrinsic allergic alveolitis

A

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

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12
Q

What is sarcoidosis

A

Multisystem granulomatous disorder of unknown cause

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13
Q

Hypersensitivity involved in sarcoidosis

A

Type 4 hypersensitivity

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14
Q

What type of granuloma histolically is positive for recovery

A

Granuloma without necrosis

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15
Q

Nonspecific interstitial pneumonitis vs idiopathic pulmonary fibrosis

A

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

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16
Q

How can rheumatoid nodules be used to differentiate from TB

A

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

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17
Q

What is pneumoconiosis

A

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

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18
Q

Differentiate between types of asbestos

A

Serpentine (curved) asbestos fibres relatively safe

Straight (amphibole) asbestos fibres highly dangerous

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19
Q

What can asbestos exposure lead to

A

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

20
Q

What are interstitial disease

A

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

21
Q

What type of hypersensitivity is sarcoidosis

A

Type 4 hypersensitivity

22
Q

What does sarcoidosis cause

A

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

23
Q

What can acute sarcoidosis cause

A

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

24
Q

What can chronic sarcoidosis cause

A

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

25
Q

Differential diagnosis of sarcoidosis

A

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

26
Q

Diagnosis sarcoidosis

A

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

27
Q

Management of sarcoidosis

A

Acute - Self-limiting

Chronic - Oral steroids if vital organs affected, immunosuppression

28
Q

What type of hypersensitivity is extrinsic allergic alveolitis

A

Type 3 hypersensitivity

29
Q

Cause of extrinsic allergic alveolitis

A

Called hypersensitivity pneumonitis -

Idopathic, thermophillic actinomycetes, avian antigens, drugs

30
Q

Symptoms of acute extrinsic allergic alveolitis

A

Cough, dyspnoea, myalgia, pyrexia

Signs are crackles (no wheeze), hypoxia and widespread pulmonary infiltrates on chest x-ray

31
Q

Treatment of acute EAA

A

Oxygen, steroids and antigen avoidance

32
Q

Symptoms of chronic extrinsic allergic alveolitis

A

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

33
Q

Treatment of chronic EAA

A

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

34
Q

Signs and symptoms of idiopathic pulmonary fibrosis

A

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

35
Q

Spirometry of idiopathic pulmonary fibrosis

A

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

36
Q

CT scan observation of idiopathic pulmonary fibrosis

A

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

37
Q

What are signet ring cells

A

Cells with a large vacuole often seen in carcinomas

38
Q

Treatment of idiopathic pulmonary fibrosis

A

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

39
Q

Prognosis of idiopathic pulmonary fibrosis

A

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

40
Q

Symptoms of simple coal workers pneumoconiosis

A

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

41
Q

Symptoms of complicated coal workers pneumoconiosis

A

Progressive massive fibrosis with a restrictive pattern of breathlessness.

42
Q

What is Caplan’s syndrome

A

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

43
Q

Simple vs complicated silicosis

A

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

44
Q

How does asbestos affect pleural lining

A

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

45
Q

Asbestos bodies in sputum and lung biopsy

A

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