Takizawa H, Suzuki N, Yanagawa T, Okazaki H, Satoh M, Akiyama N, Kohyama T, Kawasaki S, Ito K, Oka T
Department of Internal Medicine & Physical Therapy, University of Tokyo, School of Medicine, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1996 Oct;34(10):1093-7.
To determine the role of interstitial lung disease (ILD) in the outcome of polymyositis-dermatomyositis (PM/DM), we studied 41 patients who had radiographic evidence of ILD among 51 patients with PM/DM. The patients were divided into 3 groups according to therapy: progressive, no change, or improved. Those classified as progressive had significantly higher rates of skin and lung symptoms, milder muscle symptoms, and lower levels of muscle enzymes than the other 2 groups, and none was positive for the anti-Jo-1 autoantibody. Nine patients with DM died of respiratory failure; they had low serum creatine kinase levels, no anti-Jo-1 antibodies, and a rapidly progressive course before death. Autopsy findings were obtained in 7 patients, and all had diffuse alveolar damage as the main pulmonary histological finding. A subgroup of patients with PM/DM-ILD have a poor prognosis, and ILD in PM/DM is an important prognostic factor.
为了确定间质性肺疾病（ILD）在多发性肌炎-皮肌炎（PM/DM）预后中的作用，我们研究了51例PM/DM患者中41例有ILD影像学证据的患者。根据治疗情况将患者分为3组：病情进展、无变化或改善。那些被归类为病情进展的患者皮肤和肺部症状发生率显著更高，肌肉症状较轻，肌肉酶水平低于其他两组，且无一例抗Jo-1自身抗体呈阳性。9例皮肌炎患者死于呼吸衰竭；他们血清肌酸激酶水平低，无抗Jo-1抗体，且死亡前病程进展迅速。7例患者进行了尸检，所有患者主要肺部组织学表现均为弥漫性肺泡损伤。PM/DM-ILD患者亚组预后较差，PM/DM中的ILD是一个重要的预后因素。
