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Pathophysiology: Wegener’s granulomatosis (WG) is a rare multisystemic autoimmune disease of unknown aetiology, characterized by necrotizing granulomatous inflammatory and pauci-immune vasculitis in small- and medium-sized blood vessels (capillaries, venules, arterioles and arteries) associated with antineutrophil cytoplasmic antibodies directed against proteinase 3 (PR3), a neutrophil serine protease, presented in primary azurophil granules of polymorphonuclear neutrophils and lysosomes of monocytes.
Diagnosis: Whenever possible, it is important to confirm the diagnosis of Wegener’s by biopsying an involved organ and finding the pathologic features of this disease under the microscope. Because many diseases may mimic Wegener’s (and vice versa), before starting a treatment regimen it is essential to be as certain of the diagnosis as possible.
The treatment of GPA can be divided into two stages: first, the induction of disease remission, and secondly, the maintenance of disease remission. Medication usually consists of cytotoxic agents (a form of chemotherapy), using relatively low doses of Cytoxan, and/or methotrexate and/or azathioprine (Imuran), and glucocorticoids (prednisone). Treatment will vary based on patient symptoms, disease activity, organ involvement and lab test results.
Research: According to recent research of Mannheim, Germany, “Since Wegener’s Granulomatosis represents a relapsing disease; efforts have been made to reliably to predict relapses using blood tests. Follow up measures such as conventionally determined C-reactive protein, antineutrophil antbody titer, proteinase-3 ELISA are applied.”