Volkmann Elizabeth R, Tashkin Donald P, Sim Myung, Kim Grace Hyun, Goldin Jonathan, Clements Philip J
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Department of Radiology, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
J Scleroderma Relat Disord. 2019 Feb;4(1):62-70. doi: 10.1177/2397198318816915. Epub 2018 Dec 17.
Interstitial lung disease occurs in the majority of patients with systemic sclerosis. Although interstitial lung disease is the number one cause of death in systemic sclerosis, interstitial lung disease progression rates vary considerably among patients with systemic sclerosis. Some patients with systemic sclerosis-associated interstitial lung disease have sub-clinical disease and may not derive benefit from immunosuppression, while others have a more aggressive interstitial lung disease phenotype. Reliable predictors of interstitial lung disease progression are lacking. The present review describes our current approach to monitoring systemic sclerosis-associated interstitial lung disease progression in clinical practice. To illustrate the marked heterogeneity that exists in interstitial lung disease progression rates in systemic sclerosis, this review presents the individual disease course of five unique patients with systemic sclerosis-associated interstitial lung disease who participated in the Scleroderma Lung Study II. These cases illustrate that treatment response rates vary in systemic sclerosis-associated interstitial lung disease and more research is needed to determine how to predict treatment response in systemic sclerosis-associated interstitial lung disease and to develop personalized treatment approaches for patients with this devastating disease.
间质性肺疾病在大多数系统性硬化症患者中都会出现。尽管间质性肺疾病是系统性硬化症患者的首要死因，但系统性硬化症患者中间质性肺疾病的进展速度差异很大。一些系统性硬化症相关间质性肺疾病患者有亚临床疾病，可能无法从免疫抑制中获益，而另一些患者则有更具侵袭性的间质性肺疾病表型。目前缺乏间质性肺疾病进展的可靠预测指标。本综述描述了我们目前在临床实践中监测系统性硬化症相关间质性肺疾病进展的方法。为了说明系统性硬化症中间质性肺疾病进展速度存在的显著异质性，本综述介绍了五例参与硬皮病肺部研究II的系统性硬化症相关间质性肺疾病独特患者的个体病程。这些病例表明，系统性硬化症相关间质性肺疾病的治疗反应率各不相同，需要更多研究来确定如何预测系统性硬化症相关间质性肺疾病的治疗反应，并为患有这种毁灭性疾病的患者制定个性化治疗方案。
