29th January 2024

Clinical features

Granulomatosis with polyangiitis can affect virtually any site in the body with predisposition to certain organs such as the upper respiratory tract, lungs and kidneys. Constitutional features such as fever, night sweats, weight loss and arthralgia will commonly occur.

Ear, nose and throat involvement are often the first clinical manifestation of disease. Commonly, patients have chronic rhinitis or sinusitis, which are slow to resolve. This causes nasopharyngeal ulcerations and nasal septum perforation, leading to depression of the nasal dorsum (saddle nose deformity). Others can present with chronic otitis (decreased hearing), mastitis, oral ulcers or gingival hyperplasia (strawberry gingivitis). 

Skin lesions such as papules, vesicles and ulcers may occur. Palpable purpura of the lower extremities may lead to Raynaud’s phenomenon, necrotising vasculitis and dry gangrene of digits. Ocular involvement includes conjunctivitis, episcleritis, retinal vasculitis and corneal ulcerations. 

Life-threatening complications of the disease include those with renal involvement causing pauci-immune glomerulonephritis. This can progress to rapidly progressive (crescentic) glomerulonephritis with possible pulmonary-renal syndrome if not aggressively managed. These patients have proteinuria, haematuria, and red cell casts in the urine. 

Patients can also present with pneumonia-like symptoms caused by pulmonary fibrosis, pulmonary hypertension and pulmonary haemorrhage. Cardiac involvement can lead to pericarditis and myocarditis. Disease in the coronary arteries are complicated by myocardial infarction and death. 

Image Source: Wikipedia (2021). Granulomatosis with polyangiiti, Licensed under a creative commons licence. Available for free at: https://en.wikipedia.org/wiki/Granulomatosis_with_polyangiitis (Accessed:16 February 2022).