Present And Future Management Of Anti-Neutrophil Cytoplasmic Antibody Associated Vasculitis: How Therapy Changed The Prognosis

Massimo L Andolina,Giovanni Forte,N Marigliano, Salvatore Galasso,Francesca Mazzei,Domenico Galasso

ITALIAN JOURNAL OF MEDICINE(2013)

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摘要
Anti-neutrophil cytoplasmic antibody associated vasculitis is part of a multi-systemic idiopathic, small vessel pouc-tune vasculitis. Given the heterogeneous spectrum of the disease, and thc nccd to update therapeutic protocols, thc aim of this review was to evaluate clinical-diagnostic approaches. We examined statistical data available in the literature, in particular the 2010 review of St. Flamour et al. Management of Anca-associated Vasculitis, published in Therapeutics and Clinical Risk -Management. Acute immunosuppressive therapy and long-term maintenance, with the use of prednisolone, have significantly changed the prognosis of this disease, particularly compared with the 1970s before the introductions of steroids and cyclophosphamide. New drugs such as rituximab, monoclonal antibodies and other modulating immune system molecules are entering clinical use, and experience will confirm whether or not therapeutic guidelines are appropriate. The current diagnostic tools, ranging from laboratory and autoimmune tests, chest X-ray, broncho-alveolar lavage to capillaroseopy, allow prompt diagnosis and early treatment through a first phase of induction-remission, and a second phase of maintenance. There arc, however, recurrent and refractory forms of the disease that require long-tenn immunosuppressi on and further research into this is merited. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, have meant that outcomes have been maintained while toxicity has been reduced.
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关键词
vasculitis, Wegener's disease, Churg-Strauss syndrome
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