User:Mr. Ibrahem/Granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a long-term disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It affects small- and medium-size blood vessels, mostly commonly in the upper respiratory tract, lungs, and kidneys. Typical symptoms include a stuffy nose, nosebleeds, and inflammation within the eye. Other symptoms may include fever, coughing up blood, leg swelling, and weight loss. Onset of symptoms may be gradual or rapid. Complications may include hearing loss, blindness, infections, and kidney failure.

The exact cause is unclear, though it is believed to be related to a combination of genetics and environmental exposures. It; however, is not inherited from a person's parents. It is a type of autoimmune disorder, specifically a ANCA-associated vasculitis, along with microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. The diagnosis may be suspected based on symptoms and laboratory tests and confirmed by tissue biopsy.

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 bring about remission of symptoms. To keep the disease under control azathioprine, methotrexate, or rituximab may be used. Plasma exchange may be used in cases with kidney injury. Without treatment survival is poor; however, with treatment most live more than 8 years.

The number of new cases a year is estimated at 2–14 per million people in Europe. In the United States about 3 per 100,000 people are affect. Onset is most often between the age of 40 and 60. Males and females are affected with similar frequency. GPA is rare in Japanese and African-American populations. While the condition was first described in 1931 by Heinz Klinger, it was not identified as a separate condition until 1936 by Friedrich Wegener.