granulomatosis with polyangiitis

An autoimmune disease that is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis, vasculitis, and the presence of antineutrophil cytoplasmatic autoantibodies (ANCAs) in patient sera, and is loca

An autoimmune disease that is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis, vasculitis, and the presence of antineutrophil cytoplasmatic autoantibodies (ANCAs) in patient sera, and is located_in lung, located_in kidney, located_in skin resulting from an autoimmune attack by antineutrophil cytoplasmic antibodies against small and medium-size blood vessels.

Signs and symptoms

• Cytoplasmic antineutrophil antibody positivity
• Pleuritis
• Hemosiderin-laden macrophages in bronchoalveolar fluid
• Diffuse alveolar hemorrhage
• Multiple mononeuropathy
• Elevated bronchoalveolar lavage fluid neutrophil proportion
• Concave nasal ridge
• Granulomatosis
• Fever
• Weight loss

Medications that may treat it

cyclophosphamide rituximab

Also known as: Necrotizing respiratory granulomatosis; Wegener granulomatosis, formerly