Keck A D, Walker U A
Rheumatologische Universitätspoliklinik Basel, Felix Platter-Spital, Burgfelderstr. 101, 4012, Basel, Schweiz.
Z Rheumatol. 2013 Apr;72(3):227-35. doi: 10.1007/s00393-012-1080-y.
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.
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。