Clinical features of granulomatosis with polyangiitis

Common features of granulomatosis with polyangiitis include:

• ear, nose and throat features—sinusitis, nasal disease, subglottic stenosis (almost pathognomonic), otitis media, hearing loss, ear pain
• oral lesions
• pulmonary infiltrates and nodules, haemoptysis, pleuritis
• glomerulonephritis—in about 75% of cases; can occur at any time in the course of the disease and can progress to chronic kidney failure
• ophthalmic features—orbital mass (usually an extension of nasal-sinus disease), necrotising scleritis; these presentations are vision-threatening and require intensive treatment.

Granulomatosis with polyangiitis is usually a multisystem disease; rarely, it can present as limited disease (eg isolated sinus disease) that is not organ- or life-threatening.

Diagnosis of granulomatosis with polyangiitis is based on a suggestive clinical picture, and supportive investigation findings.