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静脉注射免疫球蛋白治疗无肌肉病变的皮肌炎皮肤病变的临床疗效。

Clinical efficacy of intravenous immunoglobulins for the treatment of dermatomyositis skin lesions without muscle disease.

作者信息

Bounfour T, Bouaziz J-D, Bézier M, Cordoliani F, Saussine A, Petit A, Juillard C, Bagot M, Rybojad M

机构信息

Université Paris Diderot, Sorbonne Paris Cité; AP-HP; Service de Dermatologie, Hôpital Saint Louis, Paris, France.

出版信息

J Eur Acad Dermatol Venereol. 2014 Sep;28(9):1150-7. doi: 10.1111/jdv.12223. Epub 2013 Aug 1.

Abstract

BACKGROUND

Treating dermatomyositis (DM) with isolated skin involvement is difficult and inconsistently performed. Intravenous immunoglobulins (IVIg) are recommended for corticoresistant or corticodependant DM, but only a few cases of IVIg use in DM with isolated skin involvement have been reported.

DESIGN

We performed a retrospective monocentric study of 27 patients who were treated with IVIg for severe DM skin lesions (no or minor muscle involvement) after failure of photoprotection and at least one line of treatment.

RESULTS

Nineteen patients (70%) exhibited a major response, four patients exhibited a partial response and four patients exhibited no response, including two patients with grade 3 side effects (headaches). The mean number of IVIg courses was 4.8 (range 1-15). Ten patients (53%) relapsed, with a median time of 6.2 months after the last IVIg course. Six of these patients were successfully treated with a new IVIg course. Muscle disease developed in six patients.

CONCLUSION

IVIg may be an effective and safe treatment for DM with isolated skin involvement. Relapse occurred frequently, but treatment with a new course of IVIg was successful. Controlled studies are required to confirm these results.

摘要

背景

治疗仅有皮肤受累的皮肌炎(DM)具有挑战性,且治疗方法并不统一。静脉注射免疫球蛋白(IVIg)被推荐用于治疗对皮质类固醇耐药或依赖的DM,但仅有少数关于IVIg用于仅有皮肤受累的DM的病例报道。

设计

我们对27例患者进行了一项回顾性单中心研究,这些患者在光防护及至少一种治疗方案失败后,接受IVIg治疗严重的DM皮肤病变(无或仅有轻微肌肉受累)。

结果

19例患者(70%)有显著反应,4例患者有部分反应,4例患者无反应,其中2例患者出现3级副作用(头痛)。IVIg疗程的平均次数为4.8次(范围1 - 15次)。10例患者(53%)复发,末次IVIg疗程后中位复发时间为6.2个月。其中6例患者再次接受IVIg疗程治疗成功。6例患者出现肌肉病变。

结论

IVIg可能是治疗仅有皮肤受累的DM的一种有效且安全的治疗方法。复发很常见,但再次进行IVIg疗程治疗是成功的。需要对照研究来证实这些结果。

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