Long-Term Remissions of Severe Pemphigus After Rituximab Therapy Are Associated with Prolonged Failure of Desmoglein B Cell Response
Long-Term Remissions of Severe Pemphigus After Rituximab Therapy Are Associated with Prolonged Failure of Desmoglein B Cell Response
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DOI：
10.1126/scitranslmed.3005166
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发表时间：
2013-03-06
影响因子：
17.1
通讯作者：
Musette, Philippe
中科院分区：
文献类型：
--
作者：
Colliou, Natacha;Picard, Damien;Musette, Philippe
天疱疮是由针对桥粒核心糖蛋白的自身抗体引起的皮肤和粘膜的严重起泡病症，所述桥粒核心糖蛋白是一种类型的角质形成细胞粘附蛋白。利妥昔单抗清除B细胞对天疱疮有短期疗效。我们的目的是评估天疱疮患者B细胞耗竭后的长期病程，并了解介导长期缓解的免疫学机制。我们评估了22例天疱疮患者在中位随访79个月后接受利妥昔单抗治疗的临床过程，并比较了达到完全缓解（CR）和不完全缓解（IR）患者之间的抗桥粒芯糖蛋白B细胞应答以及B和T淋巴细胞亚群和库。13例患者（59%）在研究期间发生CR，包括10例停止治疗的患者和3例接受泼尼松剂量的患者
Pemphigus is a severe blistering condition of the skin and mucosa caused by autoantibodies directed against desmogleins, which are a type of keratinocyte adhesion protein. B cell depletion by rituximab has short-term efficacy against pemphigus. We aimed to assess the long-term course of pemphigus patients after B cell depletion and to understand the immunological mechanisms that mediate long-lasting remissions. We evaluated the clinical course of 22 pemphigus patients treated with rituximab after a 79-month median follow-up and compared the anti-desmoglein B cell response and B and T lymphocyte subpopulations and repertoire between patients who achieved complete remission (CR) and those who had incomplete remission (IR). Thirteen patients (59%) experienced CR during the study, including 10 patients off treatment and 3 patients with prednisone doses