test positive for anti-PR3 ANCA antibodies and is higher than the relapse risk for microscopic polyangiitis. Today, medication toxicity is managed more carefully and long-term remissions are possible. Some affected individuals are able to lead relatively normal lives and remain in remission for 20+ years after treatment. Epidemiology The incidence is 10–20 cases per million per year. It is exceedingly rare in Japan and in African Americans. History Scottish otolaryngologist Peter McBride (1854–1946) first described the condition in 1897 in a BMJ article entitled "Photographs of a case of rapid destruction of the nose and face". Heinz Karl Ernst Klinger (born 1907) added information on the anatomical pathology. An early name for the disease was pathergic granulomatosis. The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas. The full clinical picture was first presented by Friedrich Wegener (1907–1990), a German pathologist, in two reports in 1936 and 1939, leading to the eponymous name Wegener's granulomatosis or Wegener granulomatosis (). In 2011, the American College of Rheumatology (ACR), the American Society of Nephrology (ASN) and the European League Against Rheumatism (EULAR) resolved to change the name to granulomatosis with polyangiitis, given Wegener's association with the Nazi Party. See also List of medical eponyms with Nazi associations References External links Arthritis Autoimmune diseases Disorders of fascia Lung disorders Nephrology Rheumatology Steroid-responsive inflammatory conditions Systemic connective tissue disorders Vascular-related cutaneous conditions