Wegener's Granulomatosis

Wegener's granulomatosis (WG), more recently granulomatosis with polyangiitis (Wegener's) (GPA), is an incurable form of vasculitis (inflammation of blood vessels) that affects small- and medium-sized vessels in the nose, lungs, kidneys, and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression. Five-year survival is up to 87%, with some of the mortality due to toxicity of treatment. It is named after Dr. Friedrich Wegener, who described the disease in 1936. In 2011, three professional bodies proposed a more descriptive name.

Wegener's granulomatosis is part of a larger group of vasculitic syndromes, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels. Apart from Wegener's, this category includes Churg–Strauss syndrome and microscopic polyangiitis. Although Wegener's granulomatosis affects small and medium-sized vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.