系统性坏死性血管炎1例

2006-01-10 00:00 来源:丁香园神经科学专业讨论版 作者:shenren 编译
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NEJM Volume 353:2516 December 8, 2005 Number 23
Case 28-2005: A Case of Systemic Necrotizing Vasculitis
To the Editor: In Case 28-2005 (Sept. 15 issue),1 the authors describe a 42-year-old man who had a systemic necrotizing vasculitis secondary to the Churg–Strauss syndrome with rapidly progressive glomerulonephritis (with a serum creatinine level of 5.7 mg per deciliter at admission), systemic symptoms, eosinophilia, asthma, hemoptysis, sinusitis, purpura, and peripheral-nerve disease. The patient was successfully treated with corticosteroids and oral cyclophosphamide, with normalization of the serum creatinine level, erythrocyte sedimentation rate, urine sediment, and blood eosinophil count. The patient felt well, he was able to return to work, and tests for antineutrophil cytoplasmic antibodies became negative. Yet the patient continued to receive cyclophosphamide well over a year after treatment was begun. In view of the potential toxicity of cyclophosphamide, this approach is no longer the norm for the treatment of a systemic necrotizing vasculitis.2,3 The preferred current approach is to induce remission with three to six months of cyclophosphamide therapy and then to use a less toxic maintenance regimen of azathioprine, methotrexate, or mycophenolate mofetil. Since relapses are common, the current induction–maintenance approach, by minimizing the initial exposure to cyclophosphamide, allows for further cyclophosphamide treatment for late relapses.
The discussant replies: I agree that a shorter course is desirable, but two factors led us to prolong this patient's cytoxan therapy for the Churg–Strauss syndrome. The first was that the patient had prolonged multisystem problems, including persistent neuropathy and recurrent asthma; the second factor was that the patient declined to transition to azathioprine.

NEJM Volume 353:2516 December 8, 2005 Number 23


Case 28-2005:系统性坏死性血管炎1例
编辑:在28-2005病理报告 (Sept. 15 issue)中,作者报道1例42岁男性,因Churg–Strauss综合征继发的系统性坏死性血管炎,合并快速进展的肾小球肾炎(入院时血清肌酐水平 5.7 mg/dl),全身受损症状,嗜酸性粒细胞增多,哮喘,咯血,鼻窦炎,紫癜,周围神经病。经给予皮质激素和环磷酰胺后成功治愈,血清肌酐、血沉、尿酸、血嗜酸细胞计数等恢复正常。病人自觉良好,重返工作,抗中性粒细胞细胞质抗体检测阴性。以后病人继续在1年内服用环磷酰胺。关于环磷酰胺的潜在毒性,这种手段不再是治疗系统性坏死性血管炎的标准。目前公认的方法是采用环磷酰胺治疗3-6个月缓解后,以后用低毒性的硫唑嘌呤、甲氨蝶呤、骁悉等药物维持治疗。复发常见,所以现有的诱导-维持方法应在初期给予最小化的环磷酰胺治疗,以便在复发时再次给予环磷酰胺治疗。
我同意应该采用较短的疗程,但2个因素使我们延长了环磷酰胺治疗Churg–Strauss综合征。首先,病人有长期多系统受损,包括长期神经病和反复的哮喘;其次,换用硫唑嘌呤后,病人衰退。

编辑: Zhu

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