Fibrosing alveolitis

Published on 02/04/2015 by admin

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Last modified 02/04/2015

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106 Fibrosing alveolitis

Instruction

Examine this patient’s chest.

Examine the respiratory system from the back.

Salient features

History

• Progressive exertional dyspnoea (occurs in 90%)

• Chronic cough (occurs in 74%)

• Arthralgia/arthritis (occurs in 19%)

• Obtain a drug history (amiodarone, nitrofurantoin and busulfan).

Examination

• Clubbing (Fig. 106.1; see also Case 218)

• Central cyanosis

• Bilateral, basal, fine, end-inspiratory crackles that disappear or become quieter on leaning forwards. Furthermore, the crackles do not disappear on coughing (unlike those of pulmonary oedema). The crackles have been called ‘velcro’ or ‘cellophane’ crackles

• Tachypnoea (in advanced disease).

Fig. 106.1 Hands and index fingers of a normal person (A) and a person with clubbing of the digits (B) secondary to severe diffuse pulmonary interstitial fibrosis.

(With permission from Mason RJ et al. 2005.)

Proceed as follows:

• Examine the following:

• Hands (for rheumatoid arthritis, systemic sclerosis)

• Face (for typical rash of SLE, heliotropic rash of dermatomyositis, typical facies of systemic sclerosis, lupus pernio of sarcoid)

• Mouth (for aphthous ulcers of Crohn’s disease, dry mouth of Sjögren syndrome).

• Look for signs of pulmonary hypertension: ‘a’ wave in the JVP, left parasternal heave and P₂.

Diagnosis

This patient has bilateral, basal, fine end-inspiratory crackles (lesion) caused by fibrosing alveolitis (aetiology) and is tachypnoeic at rest (functional status).

Questions

In which other conditions is clubbing associated with crackles?

• Bronchogenic carcinoma (crackles are localized)

• Bronchiectasis (coarse crackles)

• Asbestosis (history of exposure to asbestos).

Advanced-level questions

Mention possible aetiological factors

Metal dust (steel, brass, lead), wood dust (pine), wood smoke and smoking.

Mention other conditions which have similar pulmonary changes

• Rheumatoid arthritis, SLE, dermatomyositis, chronic active hepatitis, ulcerative colitis, systemic sclerosis

• Pneumoconiosis

• Granulomatous disease: sarcoid, TB

• Chronic pulmonary oedema

• Radiotherapy

• Lymphangitis carcinomatosa

• Extrinsic allergic alveolitis: farmer’s lung, bird fancier’s lung.

What is the pathology in fibrosing alveolitis?

Fibrosing alveolitis is characterized by the presence of connective tissue matrix proteins within the acinar regions of the lung in association with a variable cellular infiltrate within the alveoli and in the interstitium.

What are the types of interstitial pneumonitis?

Liebow and Carrington in 1969 described five subgroups depending on histology.

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