TAILIEUCHUNG - Báo cáo y học: "Wegener’s granulomatosis: current and upcoming therapies"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Wegener’s granulomatosis: current and upcoming therapies. | Arthritis Research Therapy Vol 5 No 4 Langford Review Wegener s granulomatosis current and upcoming therapies Carol A Langford Immunologic Diseases Section Laboratory of Immunoregulation National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda Maryland USA Corresponding author Carol A Langford e-mail clangford@ Received 4 Mar 2003 Revisions requested 26 Mar 2003 Revisions received 14 Apr 2003 Accepted 23 Apr 2003 Published 29 May 2003 Arthritis Res Ther 2003 5 180-191 DOI ar771 Abstract Wegener s granulomatosis is a complex multisystem disease that can be associated with morbidity and mortality. The introduction of cyclophosphamide and glucocorticoids brought about the potential for long-term survival and provided the opportunity and impetus to explore treatment options that can reduce the toxicity of therapy and lessen the likelihood of relapse. With the growth of knowledge regarding disease pathophysiology and the increasing ability to selectively target the immune system the potential options for therapeutic investigation have continued to expand. Careful study of new agents through rigorously designed trials is essential to answering questions of safety and efficacy in Wegener s granulomatosis. Keywords granulomatosis treatment vasculitis Wegener Introduction Wegener s granulomatosis WG is a unique clinicopatho-logical disease entity characterized by necrotizing granulomatous vasculitis of the upper and lower respiratory tract pauci-immune segmental necrotizing glomerulonephritis and small vessel vasculitis. Because of its wide range of manifestations WG has a broad spectrum of severity that includes the potential for alveolar hemorrhage or rapidly progressive glomerulonephritis which are immediately life threatening. It has long been appreciated that the immune system plays a critical role in the pathogenesis of WG. Among the earliest supportive evidence was the effectiveness of cyclophosphamide CYC and

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