Wegener's granulomatosis and microscopic polyangiitis

被引:0
作者
de Groot, K. [1 ]
Reinhold-Keller, E. [2 ]
机构
[1] Klinikum Offenbach GmbH, Med Klin Innere Med Nephrol Rheumatol 3, D-63069 Offenbach, Germany
[2] Hamburg Klinikum Bad Bramstedt, Internist Rheumatol Gemeinschaftspraxis, Hamburg, Germany
来源
ZEITSCHRIFT FUR RHEUMATOLOGIE | 2009年 / 68卷 / 01期
关键词
Wegener's granulomatosis; Microscopic polyangiitis; Vasculitis; ANCA; ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES; ANTIBODY-ASSOCIATED VASCULITIS; RANDOMIZED-TRIAL; SYSTEMIC VASCULITIDES; GLOMERULONEPHRITIS; THERAPY;
D O I
10.1007/s00393-008-0425-z
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are primary systemic small vessel vasculitides, associated with a positive C/PR3-ANCA in WG and P/MPO-ANCA in MPA. The most prominently involved organs are the upper (only in WG) and lower respiratory tract and the kidneys. The diagnostic work-up is an interdisciplinary approach assessing disease stage and extent. Treatment is adapted to disease stage and extent and relies on a combination of a cytotoxic plus a tapering regimen of corticosteroids. Induction of remission in "early systemic" disease can be achieved with low-dose methotrexate. In severe generalized vasculitis cyclophosphamide (CYC) is the mainstay of therapy, in rapidly progressive glomerulonephritis in combination with plasmapheresis. After 3-6 months of induction CYC is switched to a maintenance treatment with azathioprine. Alternatives are leflunomide, mycophenolate or methotrexate (creatinine < 150 mu mol/l). Age >= 50 at diagnosis, renal dysfunction and pulmonary involvement are associated with higher mortality rates. The relapse rate is approximately 50% within 5 years, being higher in WG than MPA.
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收藏
页码:49 / 63
页数:15
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