29th January 2024

Clinical features

Manifestations are similar to those with granulomatous polyangiitis; however, the nasopharynx is usually not affected. The majority of patients have renal involvement with pauci-immune glomerulonephritis with hypertension. The inflammation also commonly affects the peripheral nervous system causing mononeuritis multiplex with presentations such as foot drop, pins and needles or numbness. Approximately half of the patients have skin involvement, which is typically a dependent palpable purpuric rash. Often, patients will also have constitutional symptoms such as fever and weight loss. Lung involvement can be life-threatening with inflammatory infiltrates and alveolar haemorrhage presenting with haemoptysis.