Pathophysiology: Wegener's granulomatosis is a systemic autoimmune disease characterized by granulomatous vasculitis of the upper and lower respiratory tracts, glomerulonephritis and small vessel vasculitis. 90% of patients have pulmonary involvement. As the affected vessels are occluded, stroke developed with subsequent radiographic manifestations of ill-defined multiple bilateral opacities and delimit as myocardial is organized.
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.
Statistics: A retrospective cohort study using hospital discharge registers with a review of hospital case records. Analysis of causes of death in Finland up to 30 July 2005 A total of 492 patients received a verified diagnosis of WG between 1981 and 2000. Of these, 203 died before the end of June 2005. The overall 1-year survival rate was 83.3% and the 5-year survival rate was 74.2%.