Granulomatosis with polyangiitis

Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) is a rare form of systemic vasculitis.

Antineutrophil cytoplasmic antibody testing may help with diagnosis, but is not reliable for monitoring disease activity.

Treatment is usually considered in two stages: remission induction and remission maintenance. Patients with active disease are treated with corticosteroids and a second immunosuppressive agent. Disease severity is the principal determinant of the most appropriate additional agent.

With appropriate therapy, patients generally experience prolonged survival with a chronic relapsing course. Most patients develop disease- and/or treatment-related morbidity during the course of their disease.

Long-term management requires careful assessment of disease activity, judicious use of immunosuppressive therapies, and referral to specialised centers with multidisciplinary treatment.

Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) is a systemic vasculitis that typically involves small and medium vessels. Although any organ may be targeted, the classic triad consists of upper and lower respiratory tract involvement and pauci-immune glomerulonephritis. Involvement of cutaneous, ocular, musculoskeletal, and peripheral nervous system tissue is also common.