Saeki T, Suzuki E, Watanabe T, Saito Y, Itou S, Honma T, Sato T, Nakano M, Arakawa M
Department of Medicine (II), Niigata University School of Medicine.
Ryumachi. 1994 Feb;34(1):16-21.
IP, the major complication of PM/DM, is closely related to the life of these patients. In this study the course and prognosis of IP associated with PM/DM were investigated. Thirty IP patients with PM/DM were divided into 4 groups according to the course of IP. Clinical manifestations, laboratory data, chest radiographs and treatment were examined retrospectively. Eleven cases improved (Group I), 11 cases were exacerbated (Group E), 3 cases were unchanged (Group U), 2 cases died due to rapid progression of IP (Group D) and 3 cases dropped out. Both Group D patients were middle aged female DM patients without CPK elevation. All cases were treated with corticosteroids. The clinical manifestations, laboratory data, chest radiographs and initial dose of corticosteroid were not markedly different between Group I and E. The total dose of corticosteroids during the first 3 months tended to be higher in Group I than in Group E. Unlike idiopathic interstitial pneumonitis, the decrease in lung volume on chest radiographs improved after the treatment in 5 cases. We conclude that IP associated with PM/DM should be treated initially with high doses of corticosteroids.
间质性肺炎(IP)是皮肌炎(PM)/多发性肌炎(DM)的主要并发症,与这些患者的生命密切相关。在本研究中,对与PM/DM相关的IP的病程和预后进行了调查。30例PM/DM合并IP的患者根据IP病程分为4组。对临床表现、实验室数据、胸部X光片和治疗情况进行回顾性检查。11例病情改善(I组),11例病情加重(E组),3例病情无变化(U组),2例因IP快速进展死亡(D组),3例退出研究。D组的两名患者均为中年女性DM患者,肌酸磷酸激酶(CPK)未升高。所有病例均接受皮质类固醇治疗。I组和E组在临床表现、实验室数据、胸部X光片和皮质类固醇初始剂量方面无明显差异。I组前3个月皮质类固醇的总剂量往往高于E组。与特发性间质性肺炎不同,5例患者治疗后胸部X光片上肺容积减少的情况有所改善。我们得出结论,PM/DM相关的IP最初应以高剂量皮质类固醇治疗。