T4 L13: Interstitial lung disease Flashcards

1
Q

What 4 things make up the interstitium?

A

Alveolar epithelium, capillary endothelium, basement membrane, and connective tissue

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2
Q

What is the mechanism for lung fibrosis?

A

It’s unknown

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3
Q

What are the functions of the extracellular matrix of the lungs?

A

Elasticity, low resistance for effective gas exchange, and tissue repair

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4
Q

What is the extracellular matrix?

A

A 3D fibre filled with macromolecules like collagen and elastin

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5
Q

What are the 3 types of occupational lung disease?

A

Silicosis, coal miners lung, and asbestosis

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6
Q

What are common causes of hypersensitivity pneumonitis?

A

Mould and bird proteins

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7
Q

What is sarcoidosis?

A

A condition where inflamed cells clump together to make non-necrotising granulomas. Cause is unknown but it’s a disease of the young (30-60 years)

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8
Q

What are come clinical presentations of idiopathic pulmonary fibrosis?

A

Slowly progressive exertional dyspnoea, non-productive cough, dry inspiratory bibasal crackles, clubbing of fingers, abnormal pulmonary function tests

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9
Q

What is idiopathic fibrosis?

A

Unknown cause but it causes progressive, irreversible fibrosis that is fatal. Its limited to the lungs and has minimal inflammation. Usually a diseases of the elderly but affects men twice as often

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10
Q

What is the prognosis for someone with idiopathic fibrisis?

A

50% survive past 3 years, 20% survive past 5 years

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11
Q

How is idiopathic pulmonary fibrosis treated?

A

No cure but Pirfenidone and Nintedanib slow progression. These drugs are often poorly tolerated and patients develop weight loss, GI upset, and photosensitivity

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12
Q

What part of the lung does hypersensitivity pneumonitis affect and why?

A

The upper lobes because it’s inhaled

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13
Q

How does hypersensitivity pneumonitis develop?

A

When a antigen in inhaled

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14
Q

What are the clinical presentations of acute hypersensitivity pneumonitis?

A

SOB, cough, fever, crackles within 4-6 hours of exposure

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15
Q

What is hypersensitivity pneumonitis often misdiagnosed as?

A

Infection

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16
Q

What are the subacute clinical presentations of hypersensitivity pneumonitis?

A

Gradual onset, weight loss is common

17
Q

What are the chronic clinical presentations of hypersensitivity pneumonitis?

A

Incomplete resolution when the antigen is removed, may lead to irreversible fibrosis

18
Q

How is hypersensitivity pneumonitis diagnosed?

A

Using serum precipitins (circulating igG antibody-antigen complexes)

19
Q

How is hypersensitivity pneumonitis managed?

A

Avoidance of the antigen or using steroids

20
Q

Which 3 drugs are known to be associated with drug-induced interstitial lung disease?

A

Nitrofurantoin (for UTI), Amiodarone (an anti-arrhythmic), and Methotrexate (an immunosuppressant)

21
Q

How is connective tissue disease-related interstitial lung disease managed?

A

Treat the underlying disease with biologics, steroids, or immunosuppressants

22
Q

Which diseases cause connective tissue disease-related interstitial lung disease?

A

Rheumatoid arthritis, scleroderma, sjogrens, and polymyositis

23
Q

What are some common clinical presentations of sarcoidosis?

A

Cough, SOB, wheeze, erythema nodosum, lupus pernio, nodules, anterior uveitis, bells palsy, mononeuropathies

24
Q

What is Lofgrens Syndrome?

A

A condition characterised by Erythema nodosum, bilateral hilar lymphadenopathy, and Arthralgia. It has an excellent prognosis and is usually self-limiting

25
Q

What is Erythema nodosum?

A

An inflammatory condition characterized by inflammation of the fat cells under the skin

26
Q

What is bilateral hilar lymphadenopathy?

A

Bilateral enlargement of lymph nodes

27
Q

What is Arthralgia?

A

Joint stiffness

28
Q

How is sarcoidosis treated?

A

corticosteroids for 6-24 months and additional immunosuppressants like methotrexate and azathioprine may be used

29
Q

What is obstructive spirometry and what is it used for?

A

Indicates a narrowing problem in the airways. Used for asthma and COPD

30
Q

What is restrictive spirometry and what is it used for?

A

Used for lung expansion or loss of lung function tests. Not useful diagnostically because it only indicates that there is a problem. Its useful for monitoring change and severity

31
Q

Why would lungs be small and stuff?

A

When they are fibrotic