Department of Internal Medicine, Vall d´Hebron General Hospital. Universitat Autonoma de Barcelona, Spain.
Clin Exp Rheumatol. 2013 May-Jun;31(3):436-9. Epub 2013 Mar 7.
The aim of this paper is to assess the effect of calcineurin inhibitors (tacrolimus or cyclosporine) for treating patients with interstitial lung disease (ILD) associated with antisynthetase autoantibodies.
Sixty patients with antisynthetase autoantibodies were identified in our myositis cohort of 179 patients. The medical records of 15 patients with antisynthetase autoantibody-associated ILD treated with tacrolimus/cyclosporine (11 for refractory disease and 4 as first-line therapy) between 1980 and 2011 were retrospectively reviewed. Serial pulmonary function tests were used to assess the clinical response. Qualitative data are presented as a number and percentage, and quantitative data as the median and interquartile range (IQR).
Patients were classified as having probable or definite idiopathic inflammatory myopathy (8 dermatomyositis and 4 polymyositis), and pure interstitial lung disease (3 cases). The 15 patients had received tacrolimus/cyclosporine for an average of 19 (IQR 14-30) months. Median age at onset of ILD was 42.3 (IQR 32.4-56.8) years and median duration of lung disease before administration of calcineurin inhibitors was 11 (IQR: 5-49) months. Median duration of follow-up was 24 (IQR 12-32) months. Thirteen patients had anti-histidyl-transfer RNA synthetase autoantibody (anti-Jo-1) and two had anti-alanyl-transfer RNA synthetase autoantibody (anti-PL-12). A more than 10% increase in FVC or stabilisation was observed in 13 (87%; 95%CI 56-98) patients who received calcineurin inhibitors (9 [81%] refractory cases and 4 [100%] as first-line therapy).
Calcineurin inhibitors seem to be a good therapeutic option for managing ILD associated with antisynthetase autoantibodies, not only in refractory cases, but also as first-line treatment.
本文旨在评估钙调神经磷酸酶抑制剂（他克莫司或环孢素）治疗抗合成酶抗体相关间质性肺病（ILD）患者的疗效。
在我们的 179 例肌炎患者队列中，共发现 60 例抗合成酶抗体患者。回顾性分析了 1980 年至 2011 年间 15 例接受他克莫司/环孢素治疗的抗合成酶抗体相关ILD 患者（11 例为难治性疾病，4 例为一线治疗）的病历。采用系列肺功能检测评估临床疗效。定性数据以例数和百分比表示，定量数据以中位数和四分位距（IQR）表示。
患者分为可能或明确的特发性炎症性肌病（8 例皮肌炎和 4 例多发性肌炎）和单纯间质性肺病（3 例）。15 例患者接受他克莫司/环孢素治疗，平均时间为 19（IQR 14-30）个月。ILD 发病中位年龄为 42.3（IQR 32.4-56.8）岁，开始使用钙调神经磷酸酶抑制剂前肺部疾病中位持续时间为 11（IQR：5-49）个月。中位随访时间为 24（IQR 12-32）个月。13 例患者抗组氨酰-tRNA 合成酶抗体（抗 Jo-1）阳性，2 例抗丙氨酰-tRNA 合成酶抗体（抗 PL-12）阳性。接受钙调神经磷酸酶抑制剂治疗的 13 例（87%；95%CI 56-98）患者 FVC 增加 10%以上或病情稳定，其中 9 例（81%）为难治性病例，4 例（100%）为一线治疗。
钙调神经磷酸酶抑制剂似乎是治疗抗合成酶抗体相关ILD 的一种有效治疗选择，不仅适用于难治性病例，也适用于一线治疗。
