Patients with diffuse cutaneous systemic sclerosis (dcSSc) frequently show spontaneous improvement of skin fibrosis. Our aim was to examine whether an improvement in skin fibrosis predicts lower likelihood of visceral organ progression and better survival. Patients from the European Scleroderma Trials and Research (EUSTAR) cohort with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, and valid mRSS at 12±3 months follow up were included. Regression/progression of skin fibrosis was defined as a decrease/increase in mRSS >5 points and ≥25% from baseline to follow up. The outcomes included progression of lung, renal, cardiac and gastrointestinal manifestations using consensus derived definitions and all-cause death. Regressive, stable and progressive patients were compared by univariate, Kaplan-Meier survival curve and Cox regression analysis. Of 1257 included patients, 883 (70.2%) were stable, 282 (22.4%) regressive, and 92 (7.3%) progressive. Regressive patients, adjusted for baseline mRSS, baseline immunosuppression, baseline FVC, and disease duration, showed a significantly lower probability of FVC decline ≥10% than progressive patients (p=0.00003), lower probability of all-cause mortality during follow up (p=0.035) compared to progressive patients. .Improvement of skin fibrosis was not associated with progression of other organ manifestations. We found that regression of skin fibrosis is associated with a lower probability of lung progression and better survival at follow up. The link between the disease course of skin and lung fibrosis in SSc can help to better stratify patients in clinical practice and enrich for ILD progressive patients in clinical trials. • Diffuse SSc patients with improvement of skin fibrosis had a lower probability of lung function progression and all-cause mortality than skin progressive patients. • This allows better risk stratification of SSc patients in clinical practice. • It could help to improve the design of clinical trials in SSc and better enrichment of ILD progressive patients.

中文翻译：

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皮肤增厚的消退是否预测弥漫性皮肤系统性硬化症患者内脏受累和生存率的改善？EUSTAR 分析


弥漫性皮肤系统性硬化症 （dcSSc） 患者经常表现出皮肤纤维化的自发性改善。我们的目的是检查皮肤纤维化的改善是否预示着内脏器官进展的可能性较低和生存率较高。包括来自欧洲硬皮病试验和研究 （EUSTAR） 队列的 dcSSc 、基线改良 Rodnan 皮肤评分 （mRSS） ≥7 和随访 12±3 个月时的有效 mRSS 的患者。皮肤纤维化的消退/进展定义为 mRSS >5 点减少/增加，从基线到随访 ≥25%。结局包括使用共识衍生定义和全因死亡的肺、肾、心脏和胃肠道表现的进展。通过单因素、 Kaplan-Meier 生存曲线和 Cox 回归分析比较回归、稳定和进展患者。在纳入的 1257 例患者中，883 例 （70.2%） 稳定，282 例 （22.4%） 退化，92 例 （7.3%） 进行性。根据基线 mRSS 、基线免疫抑制、基线 FVC 和疾病持续时间进行调整的回归患者显示 FVC 下降的可能性显着降低 ≥10% 于进展患者 （p=0.00003），随访期间全因死亡率的概率较低 （p=0.035） 与进展患者相比。.皮肤纤维化的改善与其他器官表现的进展无关。我们发现皮肤纤维化的消退与较低的肺部进展概率和较高的随访生存率相关。SSc 中皮肤病程与肺纤维化之间的联系有助于在临床实践中更好地对患者进行分层，并在临床试验中丰富 ILD 进展患者。 • 皮肤纤维化改善的弥漫性 SSc 患者肺功能进展和全因死亡率的概率低于皮肤进展患者。• 这允许在临床实践中更好地对 SSc 患者进行风险分层。• 它可能有助于改进 SSc 临床试验的设计，更好地丰富 ILD 进展患者。