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重症多重耐药寻常型天疱疮:利妥昔单抗单周期治疗实现长期缓解

Severe multi-resistant pemphigus vulgaris: prolonged remission with a single cycle of rituximab.

作者信息

Corral Isabela Soubhia, Freitas Thais Helena Proença de, Aquino Renata Telles Rudge de, Koller Daniella Abbruzzini S, Magliari Maria Elisa Ruffolo, Muller Helena

机构信息

Dermatology Clinic, Santa Casa de Misericórdia de São Paulo, São Paulo(SP), Brazil.

出版信息

An Bras Dermatol. 2013 Jul-Aug;88(4):639-42. doi: 10.1590/abd1806-4841.20131990.

Abstract

Pemphigus vulgaris is an autoimmune bullous disease whose therapy is based on systemic corticosteroids, with or without immunosuppressants. Rituximab is a chimeric monoclonal antibody of the IgG class, directed at a specific CD20 B cell surface antigen, used in pemphigus vulgaris empirically since 2002, with success in 90% of the cases and long periods of remission. Male patient, 33 years old, diagnosed with pemphigus vulgaris, confirmed by histopathology and direct immunofluorescence. He was treated for seven months with numerous treatments, including immunosuppressive drugs, with an unsatisfactory response, until he had complete remission with the use of rituximab. During a 34-month follow-up period, the patient presented a slight clinical relapse, which was successfully controlled with prednisone in a daily dose of 120 mg, soon reduced to 20mg.

摘要

寻常型天疱疮是一种自身免疫性大疱性疾病,其治疗基于全身用糖皮质激素,可联合或不联合免疫抑制剂。利妥昔单抗是一种IgG类嵌合单克隆抗体,靶向特定的CD20 B细胞表面抗原,自2002年起经验性用于寻常型天疱疮治疗,90%的病例取得成功且缓解期长。男性患者,33岁,诊断为寻常型天疱疮,经组织病理学和直接免疫荧光检查确诊。他接受了包括免疫抑制药物在内的多种治疗7个月,反应不佳,直至使用利妥昔单抗后完全缓解。在34个月的随访期内,患者出现轻微临床复发,每日口服120mg泼尼松成功控制,随后很快减至20mg。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c30d/3760947/087855bff464/abd-88-0639-g01.jpg

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