Department of Clinical and Experimental Medicine, Rheumatology Section, Second University of Naples, Naples, Italy.
Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy.
Autoimmun Rev. 2015 Jul;14(7):575-8. doi: 10.1016/j.autrev.2015.02.002. Epub 2015 Feb 21.
Interstitial lung disease (ILD) affects about 90% of patients with systemic sclerosis (SSc). It is associated with a restrictive lung disease in only 30% of patients and is progressive in an even lower percentage. A low forced vital capacity at presentation, an extent of lung fibrosis >20% as detected by lung high-resolution computed tomography, high serum interleukin-6 levels, anti-topoisomerase I antibody positivity and diffuse cutaneous SSc are each associated with SSc-ILD progression. However, no such association is absolute. Treating patients with a recent deterioration of lung function may allow to capture those with active disease. To date, cyclophosphamide (CYC) is the only drug found to stabilize or improve lung function in randomized clinical trials, but its small beneficial effect is short lived. Therefore, immunosuppressive maintenance therapy after CYC treatment is warranted. At present, however, the best therapeutical strategy after CYC therapy both in responders and in non-responders to CYC is still controversial. Based on a review of the literature, we suggest an approach to the management of SSc-ILD.
间质性肺疾病（ILD）影响约 90%的系统性硬化症（SSc）患者。它仅在 30%的患者中与限制性肺疾病相关，并且在更低的百分比中呈进行性。在发病时，用力肺活量低，肺高分辨率计算机断层扫描检测到的肺纤维化程度>20%，血清白细胞介素-6 水平高，抗拓扑异构酶 I 抗体阳性和弥漫性皮肤 SSc 与 SSc-ILD 进展相关。然而，没有任何关联是绝对的。治疗肺功能近期恶化的患者可能可以发现那些患有活动性疾病的患者。迄今为止，环磷酰胺（CYC）是唯一在随机临床试验中发现稳定或改善肺功能的药物，但它的小有益效果是短暂的。因此，在 CYC 治疗后进行免疫抑制维持治疗是必要的。然而，目前，在 CYC 治疗的应答者和非应答者中，CYC 治疗后最佳的治疗策略仍存在争议。基于对文献的回顾，我们提出了一种管理 SSc-ILD 的方法。
