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利妥昔单抗和静脉注射免疫球蛋白作为长期全身性皮质类固醇治疗天疱疮的替代方案:一项63例患者的单中心病例系列研究

Rituximab and intravenous immunoglobulin as alternatives to long-term systemic corticosteroids in the treatment of pemphigus: a single center case series of 63 patients.

作者信息

Brown Ashley E, Motaparthi Kiran, Hsu Sylvia

机构信息

Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida.

出版信息

Dermatol Online J. 2018 Jan 19;23(12):13030/qt96v387cj.

PMID:29447653
Abstract

Rituximab and intravenous immunoglobulin [IVIg] have recently emerged as effective treatments for pemphigus refractory to corticosteroids [CS]. This case series sought to compare the clinical, serologic,and adverse effects of CS, IVIg, and rituximab in patients with pemphigus. A retrospective review of 63 patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), or paraneoplastic pemphigus (PNP)was performed. Clinical remission (CR), serologic remission (SR), and adverse effects were evaluated. Three study groups were compared: patients treated with systemic CS, refractory patients treated withIVIg, and refractory patients treated with rituximab. The overall number of adverse effects was not significantly different between the groups but those observed in patients treated with systemic CS weremore severe. CR was less likely in the patients treated with systemic CS than in patients treated with IVIg or rituximab, P-value = 0.000467. SR was more likely in patients treated with systemic CS or rituximab thanin patients treated with IVIg, P-value = 0.002118. These results suggest that the clinical efficacy of IVIg is not correlated with an expected concomitant SR. Frequently reserved for refractory pemphigus,IVIg and rituximab are significantly more likely to produce clinical remission than systemic CS therapy, suggesting their utility as first-line treatments.

摘要

利妥昔单抗和静脉注射免疫球蛋白(IVIg)最近已成为治疗对皮质类固醇(CS)难治的天疱疮的有效方法。本病例系列旨在比较CS、IVIg和利妥昔单抗在天疱疮患者中的临床、血清学及不良反应。对63例寻常型天疱疮(PV)、落叶型天疱疮(PF)或副肿瘤性天疱疮(PNP)患者进行了回顾性研究。评估了临床缓解(CR)、血清学缓解(SR)及不良反应。比较了三个研究组:接受全身CS治疗的患者、接受IVIg治疗的难治性患者和接受利妥昔单抗治疗的难治性患者。各组间不良反应的总数无显著差异,但全身CS治疗患者中观察到的不良反应更严重。接受全身CS治疗的患者比接受IVIg或利妥昔单抗治疗的患者CR可能性更低,P值 = 0.000467。接受全身CS或利妥昔单抗治疗的患者比接受IVIg治疗的患者SR可能性更高,P值 = 0.002118。这些结果表明,IVIg的临床疗效与预期的伴随SR不相关。IVIg和利妥昔单抗常用于难治性天疱疮,它们产生临床缓解的可能性显著高于全身CS治疗,表明它们作为一线治疗的效用。

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