J Clin Outcomes Manage
Management of Wegener's granulomatosis and microscopic polyangiitis
Springer JM, Langford CA, Koening CL
Abstract Objectives: To present key concepts in diagnosing and treating Wegener’s granulomatosis and microscopic polyangiitis. Methods: Review of the literature and case-based discussion. Results: Wegener’s granulomatosis and microscopic polyangiitis are rare systemic inflammatory diseases characterized by vasculitis of small and medium-sized blood vessels. Both diseases may affect any organ and can usually be differentiated by clinical history and physical exam. The kidneys and lungs are commonly affected in both diseases, and patients may present with symptoms of acute renal failure or hemoptysis secondary to pulmonary capillaritis. Recognizing the presenting signs and symptoms of these diseases may avoid delays in diagnosis and in initiating appropriate treatment, which can be organ- and life-saving. Prednisone used in combination with immunosuppressive medications such as cyclophosphamide has converted these conditions from universally fatal diseases into manageable chronic conditions. Conclusion: Familiarity with the presenting signs and symptoms together with knowledge of treatment strategies and potential drug toxicities are important in providing excellent care for patients with these rare conditions.
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