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The lung in vasculitis 

The lung in vasculitis
Chapter:
The lung in vasculitis
Author(s):

G.A. Margaritopoulos

, and A.U. Wells

DOI:
10.1093/med/9780198746690.003.0418
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date: 05 March 2021

Lung involvement in vasculitic disease can manifest as diffuse alveolar haemorrhage or as other pulmonary vasculopathy. Presenting features of diffuse alveolar haemorrhage 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 suggests the diagnosis, and bronchoalveolar lavage is usually diagnostic. Other pulmonary vasculopathies present with breathlessness on exertion. Limited disease is generally treated with oral corticosteroid, given as monotherapy or in combination with a second-line immunosuppressive agent. Oral corticosteroid with either cyclophosphamide or rituximab are typically used to induce remission of generalized disease. Azathioprine or methotrexate with low-dose oral prednisolone are used to maintain remission.

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