The spectrum of granulomatous vasculitides

Abstract

Granulomatous vasculitides include some primary systemic vasculitides, but can also be secondary to other systemic diseases (e.g., systemic lupus erythematosus and rheumatoid arthritis), other granulomatous diseases (Crohn’s disease, sarcoidosis or lymphomatoid granulomatosis), and infections (tuberculosis or fungal infections) or lymphoma or lymphoproliferative hemopathic diseases. Wegener’s granulomatosis and Churg–Strauss syndrome are the two main primary granulomatous vasculitides that affect small vessels. Granulomatous vasculitis can also be observed on histology in giant cell (temporal) arteritis and, more occasionally, in Takayasu’s arteritis (the two primary large-vessel vasculitides), but only very rarely in polyarteritis nodosa, a medium-sized vessel vasculitis. Treatment for primary necrotizing vasculitides is now fairly well established, although there remain some issues to be clarified, such as the optimal maintenance therapy in Wegener’s granulomatosis or the exact indications of biotherapy...