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[肌炎的治疗]

[Therapy of myositis].

作者信息

Keck A D, Walker U A

机构信息

Rheumatologische Universitätspoliklinik Basel, Felix Platter-Spital, Burgfelderstr. 101, 4012, Basel, Schweiz.

出版信息

Z Rheumatol. 2013 Apr;72(3):227-35. doi: 10.1007/s00393-012-1080-y.

DOI:10.1007/s00393-012-1080-y
PMID:23456367
Abstract

Idiopathic inflammatory myopathy consists of dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). At all stages of myositis, physiotherapy is effective in improving muscle strength, endurance and in maintaining joint motion. In DM and PM the therapy is initiated with glucocorticosteroids. Steroid-sparing agents (azathioprine, methotrexate and cyclosporin A) are added to prevent Cushing's syndrome or an unsatisfactory response. Therapy can also be escalated with intravenous immunoglobulins. Tacrolimus and mycophenolate mofetil (MMF) were effective in small case series. Cyclophosphamide is restricted to patients not responding to previous agents. For treatment intensification immunoglobulins can also be combined with MMF. There is not enough evidence to routinely recommend rituximab. The results with TNF-alpha inhibitors and plasmapheresis were negative or inconsistent. In DM skin involvement responds to sun blockers, antimalarials, topical corticosteroids or calcineurin inhibitors. In NAM statins should be discontinued and treatment with prednisone and immunosuppressants initiated. In IBM a therapeutic trial with prednisone, methotrexate or azathioprine may be warranted, especially in cases in which the serum creatine kinase (CK) is elevated or an inflammatory infiltrate is present in the muscle biopsy.

摘要

特发性炎性肌病包括皮肌炎(DM)、多发性肌炎(PM)、包涵体肌炎(IBM)和坏死性自身免疫性肌病(NAM)。在肌炎的各个阶段,物理治疗对于提高肌肉力量、耐力以及维持关节活动均有效。在DM和PM中,治疗始于糖皮质激素。添加免疫抑制剂(硫唑嘌呤、甲氨蝶呤和环孢素A)以预防库欣综合征或疗效不佳。治疗也可通过静脉注射免疫球蛋白加强。他克莫司和霉酚酸酯(MMF)在小样本病例系列中有效。环磷酰胺仅限于对先前药物无反应的患者。为加强治疗,免疫球蛋白也可与MMF联合使用。尚无足够证据常规推荐利妥昔单抗。肿瘤坏死因子-α抑制剂和血浆置换的结果为阴性或不一致。在DM中,皮肤受累对防晒剂、抗疟药、外用糖皮质激素或钙调神经磷酸酶抑制剂有反应。在NAM中,应停用他汀类药物,并开始使用泼尼松和免疫抑制剂治疗。在IBM中,可能有必要进行泼尼松、甲氨蝶呤或硫唑嘌呤的治疗试验,尤其是在血清肌酸激酶(CK)升高或肌肉活检中有炎性浸润的病例中。

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本文引用的文献

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Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial.利妥昔单抗治疗难治性成人及青少年皮肌炎和成人多发性肌炎:一项随机、安慰剂对照试验。
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Therapeutic advances in myositis.肌炎的治疗进展。
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Pathogenesis and therapy of inclusion body myositis.包涵体肌炎的发病机制与治疗。
Curr Opin Neurol. 2012 Oct;25(5):630-9. doi: 10.1097/WCO.0b013e328357f211.
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Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI).成人及青少年皮肌炎、多发性肌炎和包涵体肌炎的评估指标:医生及患者/家长整体活动度、徒手肌力测试(MMT)、健康评估问卷(HAQ)/儿童健康评估问卷(C-HAQ)、儿童肌炎评估量表(CMAS)、肌炎疾病活动评估工具(MDAAT)、疾病活动评分(DAS)、简明健康状况调查问卷(SF-36)、儿童健康问卷(CHQ)、医生整体损伤程度、肌炎损伤指数(MDI)、定量肌肉测试(QMT)、肌炎功能指数-2(FI-2)、肌炎活动概况(MAP)、包涵体肌炎功能评定量表(IBMFRS)、皮肤型皮肌炎疾病面积和严重程度指数(CDASI)、皮肤评估工具(CAT)、皮肌炎皮肤严重程度指数(DSSI)、皮肤指数及皮肤病生活质量指数(DLQI)。
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Mod Rheumatol. 2012 Jun;22(3):382-93. doi: 10.1007/s10165-011-0534-4. Epub 2011 Oct 5.
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Safety and efficacy of exercise training in patients with an idiopathic inflammatory myopathy--a systematic review.特发性炎性肌病患者运动训练的安全性和有效性——系统评价。
Rheumatology (Oxford). 2011 Nov;50(11):2113-24. doi: 10.1093/rheumatology/ker292. Epub 2011 Sep 2.