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The lung in vasculitis 

The lung in vasculitis

The lung in vasculitis

A.U. Wells

and Roland M. du Bois


Relevant case histories from Oxford Case Histories in Respiratory Medicine has been added to this chapter.

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date: 24 April 2017

Lung involvement in vasculitic disease can manifest in two ways: (1) Diffuse alveolar haemorrhage—presenting features include fever, weight loss, and other systemic symptoms in association with cough, breathlessness, and clinical signs suggestive of pneumonia. Haemoptysis may be present but is not invariable. A fall in haemoglobin over a day or longer is diagnostically useful. Bronchoalveolar lavage is usually diagnostic. (2) Other pulmonary vasculopathies—present with breathlessness on exertion. Investigation reveals isolated reduction in gas transfer (carbon monoxide diffusing capacity, DLCO), with or without pulmonary hypertension.

Churg–Strauss syndrome—a prodromal phase of rhinitis with nasal polyps generally precedes the eventual development of treatment-resistant late-onset asthma that is followed, often years later, by vasculitic manifestations. Chest radiography shows patchy lung infiltration in up to 80% of patients: pulmonary nodules and pleural involvement can also occur. First-line treatment is with steroids, with cyclophosphamide added for severe disease.

Wegener’s granulomatosis—chronic rhinitis, sinusitis, or mastoiditis is typically followed by progression to generalized disease over months to years, with lower respiratory tract involvement in 65 to 85% often manifesting with cough, which may be purulent, and less frequently with haemoptysis. The main lung manifestations are with pulmonary nodules (one or more, which can cavitate), localized or diffuse infiltrates, alveolar haemorrhage that may be part of a pulmonary–renal syndrome, and large and small airway disease. Standard first-line treatment for the induction of remission is with prednisolone and cyclophosphamide, with the latter switched to azathioprine for maintenance treatment. Co-trimoxazole is effective for localized but not systemic disease. Rituximab therapy seems promising for disease that is refractory to standard treatments.

Other vasculitides—microscopic polyangiitis can present with diffuse alveolar haemorrhage, which can have a poor prognosis, and other primary systemic vasculitides occasionally present with respiratory features.

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