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成功的免疫吸附治疗大疱性自身免疫性疾病:寻常型天疱疮和妊娠疱疹。

Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis.

机构信息

Department of Dermatology, Krankenanstalt Rudolfstiftung, Vienna, Austria.

出版信息

J Dtsch Dermatol Ges. 2011 Jan;9(1):27-31. doi: 10.1111/j.1610-0387.2010.07500.x. Epub 2010 Aug 31.

Abstract

BACKGROUND

Immunoapheresis/immunoadsorption is a specific tool to remove immunoglobulins and immune complexes from the circulation. Immunoapheresis is successfully used in various autoantibody-mediated diseases (such as autoimmune renal disease and others). In dermatology immunoapheresis is increasingly applied as an adjuvant treatment for severe autoimmune bullous diseases.

CASE REPORT

We successfully employed adjuvant immunoapheresis to treat a 57-year-old man with life-threatening pemphigus vulgaris and a 30-year-old pregnant woman with severe pemphigoid gestationis. Immunoapheresis induced a rapid improvement and almost complete clearance of clinical symptoms without notable side effects. The clinical improvement was paralleled by a decline of the pathologic circulating autoantibodies. Following stabilization of his disease with immunoapheresis, the pemphigus vulgaris patient was continued on rituximab and remained almost free of symptoms for the next 12 months. The patient with pemphigoid gestationis was subsequently treated with systemic corticosteroids until the symptoms of her self-limited disease ceased.

CONCLUSION

Immunoapheresis might represent an excellent therapy for certain patients with severe pemphigus vulgaris or pemphigoid gestationis, unresponsive to conventional treatment regimens. We observed rapid improvement of clinical symptoms and no notable side effects.

摘要

背景

免疫吸附/免疫清除是一种从循环中去除免疫球蛋白和免疫复合物的特定工具。免疫吸附已成功应用于各种自身抗体介导的疾病(如自身免疫性肾病等)。在皮肤病学中,免疫吸附越来越多地被用作严重自身免疫性大疱性疾病的辅助治疗。

病例报告

我们成功地采用辅助性免疫吸附治疗了一名 57 岁患有危及生命的寻常型天疱疮的男性和一名 30 岁患有严重妊娠疱疹的孕妇。免疫吸附诱导了快速的临床症状改善,几乎完全清除,没有明显的副作用。临床改善与病理循环自身抗体的下降平行。在免疫吸附稳定了他的疾病后,该寻常型天疱疮患者继续接受利妥昔单抗治疗,在接下来的 12 个月内几乎没有症状。妊娠疱疹患者随后接受全身皮质类固醇治疗,直到她的自限性疾病症状停止。

结论

免疫吸附可能是对常规治疗方案反应不佳的某些严重寻常型天疱疮或妊娠疱疹患者的极好治疗方法。我们观察到临床症状的快速改善,没有明显的副作用。

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