Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Sakyo-ku, Japan.
Curr Rheumatol Rep. 2012 Jun;14(3):264-74. doi: 10.1007/s11926-012-0246-6.
Interstitial lung disease (ILD) is the most frequent organ involvement (found in nearly half) of myositis patients, but it reveals various clinical courses and therapeutic responsiveness according to clinical and serological subsets. Autoantibodies, as well as imaging and histopathological studies, are useful for the classification of ILD in myositis and provide useful information for predicting prognosis and determining treatment. Antisynthetase antibodies are correlated with chronic and recurrent ILD, whereas anti-CADM-140 (MDA5/IFIH1) antibodies are a marker of acute progressive ILD in clinically amyopathic dermatomyositis. Serum KL-6, SP-D, and ferritin are useful biomarkers for monitoring the activity and severity of ILD. Regarding treatment, glucocorticoids are the first-line drug, but additional immunomodulating drugs are also used in refractory patients. Cyclophosphamide and calcineurin inhibitors (cyclosporine and tacrolimus) appear to be the key drugs in the treatment of refractory myositis-ILD. Rituximab may become another candidate if these drugs are not effective.
间质性肺病（ILD）是肌炎患者最常见的器官受累（近半数患者），但根据临床和血清学亚组，ILD 呈现不同的临床病程和治疗反应。自身抗体以及影像学和组织病理学研究有助于肌炎患者的 ILD 分类，并为预测预后和确定治疗提供有用信息。抗合成酶抗体与慢性和复发性 ILD 相关，而抗 CADM-140（MDA5/IFIH1）抗体是临床无肌病性皮肌炎中急性进行性 ILD 的标志物。血清 KL-6、SP-D 和铁蛋白是监测 ILD 活动度和严重程度的有用生物标志物。关于治疗，糖皮质激素是一线药物，但在难治性患者中也使用其他免疫调节药物。环磷酰胺和钙调神经磷酸酶抑制剂（环孢素和他克莫司）似乎是治疗难治性肌炎-ILD 的关键药物。如果这些药物无效，利妥昔单抗可能成为另一个候选药物。
