Cherin P, Piette J C, Wechsler B, Bletry O, Ziza J M, Laraki R, Godeau P, Herson S
Service de Médecine Interne, Groupe Hospitalier Pitié-Salpétrière, Paris, France.
J Rheumatol. 1994 Jun;21(6):1092-7.
Polymyositis (PM) and dermatomyositis (DM) are inflammatory muscle diseases of presumed autoimmune origin. Many possible interventions are available to treat these patients: corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. But these therapies are not always effective and may be responsible for certain serious side effects. Polyvalent intravenous immunoglobulin (IVIG) has been tried with success in inflammatory myopathies after failure of traditional treatment. An attempt was made to evaluate the efficacy of IVIG as first line therapy in patients with PM or DM.
Eleven Caucasian patients [6 women, 5 men, mean age 55.6 (SD 10.1) years], with active recent inflammatory myopathy, were treated by high doses of IVIG as first choice. The average duration of inflammatory myopathy before IVIG was 9.6 months (SD 10.2 months, with a range of 1 month to 3 years). Five patients had PM and 6 had DM. None had myositis associated with connective tissue disease. Two patients had a history of malignant disease: 1 lymphoma and 1 breast tumor with relapse of the malignancy during the study. One patient had a probable lung carcinoma and in another patient, ovarian carcinoma was diagnosed a few months after the onset of IVIG. We used preparations of polyvalent human i.v. gamma globulins with intact IgG. All patients received 1 g/kg daily for 2 days each month. The mean course of treatment was 4 months.
Clinical assessment, evaluated by proximal muscle power and biochemical tests, was carried out before each treatment period. Significant clinical improvement was noted in only 3 of the 11 patients (one with acute coxsackie virus B infection, and one with possible drug induced myopathy). Mean muscle power estimated for the 11 patients before and after IVIG therapy was not significantly improved. Eight patients showed significant biochemical improvement (more than 50%). Mean CK levels for the 11 patients showed a statistically significant decrease during IVIG therapy (p < 0.01). Minor IVIG side effects were noted in one patient.
IVIG therapy seems effective rarely as first therapy in patients with inflammatory myopathy but may be considered especially in viral or drug induced myopathy. IVIG therapy as the first treatment may also be tried in patients with contraindication for steroids, and in mild myopathy, especially in the elderly, to avoid steroid induced side effects.
多发性肌炎（PM）和皮肌炎（DM）是推测为自身免疫性起源的炎性肌肉疾病。有许多可能的干预措施可用于治疗这些患者：皮质类固醇、免疫抑制药物、血浆置换和全身照射。但这些疗法并不总是有效，且可能导致某些严重的副作用。在传统治疗失败后，已尝试使用多价静脉注射免疫球蛋白（IVIG）成功治疗炎性肌病。本研究旨在评估IVIG作为PM或DM患者一线治疗的疗效。
11名患有近期活动性炎性肌病的白种人患者（6名女性，5名男性，平均年龄55.6岁（标准差10.1岁）），首选大剂量IVIG治疗。IVIG治疗前炎性肌病的平均病程为9.6个月（标准差10.2个月，范围为1个月至3年）。5例为PM，6例为DM。均无与结缔组织病相关的肌炎。2例有恶性疾病史：1例淋巴瘤，1例乳腺癌，在研究期间恶性肿瘤复发。1例可能患有肺癌，另1例患者在IVIG治疗开始后几个月被诊断为卵巢癌。我们使用了具有完整IgG的多价人静脉注射丙种球蛋白制剂。所有患者每月接受1 g/kg，连续2天。平均疗程为4个月。
在每个治疗期之前，通过近端肌肉力量和生化检查进行临床评估。11例患者中仅有3例（1例急性柯萨奇病毒B感染，1例可能为药物性肌病）有显著的临床改善。IVIG治疗前后11例患者的平均肌肉力量无显著改善。8例患者有显著的生化改善（超过50%）。11例患者的平均肌酸激酶水平在IVIG治疗期间有统计学显著下降（p<0.01）。1例患者出现轻微的IVIG副作用。
IVIG治疗作为炎性肌病患者的一线治疗似乎很少有效，但在病毒或药物性肌病中可特别考虑。对于有类固醇禁忌证的患者以及轻度肌病患者，尤其是老年人，为避免类固醇引起的副作用，也可尝试将IVIG治疗作为首选治疗方法。
