Granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), after Nazi German physician Friedrich Wegener, is a rare, long-term, systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is an autoimmune disease and a form of vasculitis that affects small- and medium-sized vessels in many organs, but most commonly affects the upper respiratory tract, lungs, and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. Damage to the heart, lungs, and kidneys can be fatal.

The cause of GPA is unknown. Genetics have a role in GPA, though the risk of inheritance appears to be low.

GPA treatment depends on the severity of the disease. Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease, and azathioprine, methotrexate, or rituximab to keep the disease under control. Plasma exchange is also used in severe cases with damage to the lungs, kidneys, or intestines.

The number of new cases of GPA each year is estimated to be between 2.1 and 14.4 new cases per million people in Europe. GPA is rare in Japanese and African-American populations but occurs more often in people of Northern European descent. GPA is estimated to affect three cases per 100,000 people in the United States and affects men and women equally. GPA has infrequently been reported in minors.