Granulomatosis With Polyangiitis

Excerpt

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a necrotizing vasculitis affecting small to medium-sized vessels. GPA is part of a spectrum of disorders known as antineutrophil cytoplasmic antibody (ANCA)–associated vasculitides. The 3 main ANCA-associated vasculitides are GPA, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA or Churg-Strauss syndrome). This classification was established at the 2012 Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides. GPA is characterized by a pulmonary-renal syndrome associated with otorhinolaryngological manifestations.

The first case of GPA was described by German medical student Heinz Klinger in 1931. In 1936, a German pathologist, Friedrich Wegener, described 3 cases of peculiar small-medium vessel vasculitis with granulomatous inflammation and identified the disorder as a distinct form of vasculitis. In 1954, Godman and Churg published a review involving 22 cases, and the disease was universally known as Wegener's granulomatosis. In 1989, Wegener was awarded a Master Clinician Prize by the American College of Chest Physicians. In 2000, Wegener's Nazi ties came to light, and a movement began to rename the disease in the clinical community. The board of directors of the American College of Rheumatology (ACR), the American Society of Nephrology, and the European League Against Rheumatism recommended a switch to disease-descriptive nomenclature. Hence, the disease was renamed GPA.

Please see StatPearls' companion resources, "ANCA Positive Vasculitis," "Microscopic Polyangiitis," and "Eosinophilic Granulomatosis with Polyangiitis," for more information.