Granulomatosis with Polyangiitis
Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's Granulomatosis, is a small-vessel vasculitis that primarily affects the upper airways, the lungs, and kidneys.
Inflammation of the upper respiratory tract and present as chronic sinusitis, otitis media, mastoiditis, with the potential complication of perforation of the nasal septum. Inflammation may also involve mucosal surfaces, with findings like strawberry gums.
Inflammation of the lungs leads to hemoptysis, cough and dyspnea. Chest X-ray will reveal large nodular densities.
Inflammation of the kidneys results in glomerulonephritis, presenting with hematuria. Renal biopsy reveals a rapidly progressive (crescentic) glomerulonephritis, with a pauci-immune pattern on immunofluorescence.
GPA is a focal necrotizing vasculitis, with development of granulomas. Patients may have anti-neutrophilic cytoplasmic antibodies (ANCAs) against proteinase 3 (PR3-ANCA), also known as cytoplasmic ANCA (cANCA).
Treatment involves administration of cyclophosphamide and corticosteroids.
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NOTE: Wegener was a member of the Nazi party before and during World War II. The disease should be referred to by its correct name, Granulomatosis with Polyangiitis (GPA) -- Wegener's granulomatosis is not an acceptable eponym. Click here for further reading.