Vaskulárna medicína 2/2015
Granulomatosis with polyangiitis
Granulomatosis with polyangiitis (GPA) (previous name Wegener´s granulomatosis) is a primary systemic necrotizing small-vessel vasculitis that usually affects the upper and lower respiratory tract and the kidney. Limited clinical forms of GPA occur, especially when the disease is confined to the respiratory system or the eye. Most patients have circulating antineutrophil cytoplasmic antibodies (ANCA) against proteinase 3. In the article the etiology, histopathology, clinical presentation, diagnostic methods as well as therapeutic principles of GPA are described.
Keywords: granulomatosis with polyangiitis, Wegener´s granulomatosis, diagnostic, treatment.
