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Respiratory > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

What are the 2 main symptoms of interstitial lung disease?

A

Breathlessness and dry cough.

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2
Q

What groups divide interstitial lung diseases based on time?

A

Acute, episodic or chronic.

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3
Q

What are the 4 groups of interstitial lung diseases?

A
  1. ILD of known cause.
  2. Idiopathic interstitial pneumonia.
  3. Granulomatous ILDs e.g. sacroidosis, EAA.
  4. Other forms e.g. LAM, HX.
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4
Q

What type of hypersensitivity reaction is sarcoidosis and what is its cause?

A

Type 4 (granulomatous), unknown cause.

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5
Q

What systems are involved in sarcoidosis?

A

Common: lungs, lymph nodes, joints, liver, skin, eyes. Less common: kidneys, brain, nerves, heart.

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6
Q

What type of granuloma is present in sarcoidosis?

A

Non-caseating granuloma.

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7
Q

What is a possible cause of sarcoidosis?

A

Probable infective agent in susceptible individual due to imbalance of immune system.

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8
Q

What group is sarcoidosis less common in?

A

Smokers.

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9
Q

What are the signs and symptoms of acute sarcoidosis?

A

Erythema nodosum, bilateral hilar lymphadenopathy, arthritis, uveitis, parotitis (inflammation of the parotid gland), fever.

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10
Q

What are the signs and symptoms of chronic sarcoidosis?

A

Lung infiltrates (alveolitis), skin infiltrations, peripheral lymphadenopathy, hypercalcaemia, other organs.

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11
Q

What are the differential diagnoses of sarcoidosis?

A

TB, lymphoma, carcinoma, fungal infections.

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12
Q

What are the tests and investigations for sarcoidosis and what are you looking for?

A

CXR (bilateral hilar lymphadenopathy), lung CT scan (peripheral nodular infiltrate), tissue biopsy (e.g. transbronchial, skin, lymph node, non-caseating granuloma), pulmonary function (restrictive), blood test (angiotensin converting enzyme levels as activity marker, raised calcium, increased inflammatory markers).

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13
Q

What is the treatment for acute sarcoidosis?

A

Self-limiting so usually no treatment, steroids if vital organ affected.

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14
Q

What is the treatment for chronic sarcoidosis?

A

Oral steroids usually needed if vital organ affected. Immunosuppression e.g. azathioprine, methotrexate, anti-TNF therapy.

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15
Q

In sarcoidosis why would you monitor CXR and pulmonary function for several years?

A

There are often relapses.

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16
Q

What type of hypersensitivity reaction is extrinsic allergic alveolitis (hypersensitivity pneumonitis)?

A

Type III (immune complex deposition), reaction to antigen (lymphocytic alveolitis).

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17
Q

What antigens cause EAA?

A

Thermophilic actinomyecytes (farmers, malt workers, mushroom workers), avian antigens (bird fanciers), drugs (gold, bleomycin, sulphasalazine),

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18
Q

What are the symptoms and signs of acute EAA?

A

Symptoms: cough, breathlessness, fever myalgia (all may occir several hours after exposure). Signs: pyrexia, crackles (no wheeze), hypoxia.

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19
Q

What would you see on CXR with acute EAA?

A

Widespread pulmonary infiltrates.

20
Q

What is the treatment for acute EAA?

A

Oxygen, steroids and antigen avoidance.

21
Q

What would cause chronic EAA?

A

Repeated low dose antigen exposure over time.

22
Q

What are the symptoms and signs of chronic EAA?

A

Symptoms: progressive breathlessness and cough. Signs: Crackles, clubbing is unusual.

23
Q

Where is pulmonary fibrosis most often seen in a CXR of chronic EAA?

A

The upper zones.

24
Q

What would pulmonary function tests of chronic EAA show?

A

Restrictive defect, low TLCO [transfer factor for the lung for carbon dioxide].

25
Q

How would you come to the diagnosis of chronic EAA?

A

History of exposure, precipitins (IgG antibodies to guilty antigen), lung biopsy if in doubt.

26
Q

What is the treatment for chronic EAA?

A

Remove antigen exposure, oral steroids if breathless or low gas transfer.

27
Q

What causes idiopathic pulmonary fibrosis?

A

Imbalance of fibrotic repair system (cause of this is unknown).

28
Q

Give some examples of secondary causes of pulmonary fibrosis.

A

Rheumatoid arthritis, SLE (systemic lupus), systemic sclerosis, asbestos.

29
Q

What drugs can cause pulmonary fibrosis?

A

Amiodarone, busulphan, bleomycin, penicillamine, nitrofurantoin, methotrexate.

30
Q

What are the signs and symptoms of IPF?

A

Symptoms: progressive breathlessness (several years), dry cough. Signs: finger clubbing, bilateral fine inspiratory crackles.

31
Q

What would show up in pulmonary function tests and CXR in IPF?

A

PFTs - restrictive defects, reduced lung volumes and low gas transfer. CXR - bilateral infiltrates.

32
Q

What would be seen in a CT scan of IPF?

A

Reticulonodular fibrotic shadowing, worse at the lung bases and periphery. Traction bronchiectasis (alvoeli pull airways apart). Honey combing cystic changes.

33
Q

When would a lung biopsy not be necessary in IPF?

A

If CT scan diagnostic.

34
Q

What is the differential diagnosis for IPF?

A

Occupational diseases, connective tissue disease (RhA, scleroderma, Sjogrens disease, SLE), LVF, sarcoidosis, EAA.

35
Q

What is the pathological structure of IPF?

A

Usual intersitial pneumonia patter (UIP). Heterogenous fibrosis in alveolar walls with fibroblastic foci and destruction of architecture causing honeycombing. Minimal inflammation.

36
Q

What is the treatment for IPF?

A

Antifibrotic drugs (pirfenidone and nintedanibe), slow down disease progression but very expensive and many side-effects.

37
Q

Describe the pulmonary interstitium.

A

Alveolar lining cells, thin elastin-rich connective component containing capillary blood vessels.

38
Q

What is the early stage of interstitial lung disease?

A

Alveolitis (injury with inflammatory cell infiltration).

39
Q

What is the disease that has acute ILD?

A

Adult respiratory distress syndrome (ARDS).

40
Q

What is the late stage of ILD characterised by?

A

Fibrosis.

41
Q

What two types of biopsy are used in ILDs?

A

Transbronchial (special forceps used at bronchoscopy) and thoracoscopic (more invasive but more reliable and generated far more tissue).

42
Q

What are the two commonest ILDs?

A

Granulomatous ILDs and idiopathic pulmonary fibrosis.

43
Q

What is a sign on someone’s hands that they have IPF?

A

Finger clubbing.

44
Q

What are the visible signs of IPF on the lungs?

A

Subpleural and basal fibrosis, inflammatory component variable, terminally lung structure replaced by dilated spaces surrounded by fibrous walls (honeycomb lung).

45
Q

What pulmonary effects may there be in connective tissue disease?

A

Interstitial fibrosis (milder than IPF), pleural effusions, rheumatoid nodules.

Respiratory (30 decks)

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