From the Department of Clinical Immunology and Rheumatology, University of Alabama at Birmingham, Birmingham, Alabama; Department of Internal Medicine, and Section of Pulmonary and Critical Care Medicine, and Section of Rheumatology, University of Chicago, Chicago, Illinois, USA.
Dr. Dua serves on the advisory board for Genentech and Janssen. Dr. Strek has served as the PI for her institution for studies of therapy for idiopathic pulmonary fibrosis with Gilead, InterMune, and MedImmune. She has served as a member of the Data Safety Committee for Boehringer-Ingelheim. Dr. Vij has received grant funding from Genentech for the study of interstitial lung diseases.
J Rheumatol. 2017 Nov;44(11):1612-1618. doi: 10.3899/jrheum.161217. Epub 2017 Sep 1.
Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases.
A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. In those who failed conventional treatment and received adjunctive tacrolimus, response to tacrolimus was measured by the improvement in myositis, ILD, and change in the dose of glucocorticoids.
Thirty-one of 54 patients (57%) responded to conventional treatment based on the predefined variables of improvement in myositis and/or ILD. Patients with polymyositis (PM)-ILD were more likely to respond to conventional treatment than those with dermatomyositis (DM)-ILD (67% vs 35%, p = 0.013). Twenty-three patients failed conventional treatment, 18 of whom subsequently received adjunctive tacrolimus. Ninety-four percent had improvements in ILD and 72% showed improvement in both myositis and ILD. The mean doses of prednisone decreased from baseline by 65% at 3-6 months (p = 0.002) and 81% at 1 year (p < 0.001).
Patients with PM-ILD were more likely to respond to conventional treatment than patients with DM-ILD, but clinical characteristics and serology did not otherwise predict response to therapy. A majority of patients with MA-ILD refractory to conventional therapy improved while receiving tacrolimus and were able to decrease their dose of both glucocorticoids and other disease-modifying antirheumatic drugs.
肌炎相关性间质性肺病（MA-ILD）患者常对常规治疗无效，且预测其治疗反应具有挑战性。最近的病例报告和小系列研究表明，他克莫司在难治性病例中可能有用。
一项回顾性队列研究，比较了对常规治疗有反应和无反应的 MA-ILD 患者的临床特征。在常规治疗失败且接受辅助他克莫司治疗的患者中，通过肌炎、ILD 的改善以及糖皮质激素剂量的变化来衡量对他克莫司的反应。
根据肌炎和/或ILD 改善的预定变量，54 例患者中有 31 例（57%）对常规治疗有反应。皮肌炎相关性间质性肺病（DM-ILD）患者比多发性肌炎相关性间质性肺病（PM-ILD）患者更有可能对常规治疗有反应（67%比 35%，p=0.013）。23 例患者常规治疗失败，其中 18 例随后接受了辅助他克莫司治疗。94%的患者ILD 改善，72%的患者肌炎和ILD 均改善。泼尼松的平均剂量在 3-6 个月时从基线下降 65%（p=0.002），在 1 年时下降 81%（p<0.001）。
PM-ILD 患者比 DM-ILD 患者更有可能对常规治疗有反应，但临床特征和血清学并不能预测治疗反应。大多数对常规治疗无效的 MA-ILD 患者在接受他克莫司治疗后得到改善，并能够减少糖皮质激素和其他疾病修饰抗风湿药物的剂量。
