Clinical features

• adolescence to elderly, mean age 40-45

• constitutional symptoms (as for PN)

• lower respiratory symptoms, e.g. cough, dyspnoea

• upper respiratory symptoms: rhinorrhoea, epistaxis, sinus pain

• polyarthritis

• renal involvement—usually not clinically apparent

• chest X-ray points to diagnosis—multiple nodes, cavitations

• antineutrophil antibodies are a useful diagnostic marker (not specific)

• diagnosis confirmed by biopsy, usually an open lung biopsy

• better prognosis with early diagnosis and treatment with cyclophosphamide.

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