Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts.
Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts.
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DOI：
10.1136/annrheumdis-2018-213708
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发表时间：
2019-01
影响因子：
27.4
通讯作者：
SLS I and SLS II study groups
中科院分区：
文献类型：
--
作者：
Volkmann ER;Tashkin DP;Sim M;Li N;Goldmuntz E;Keyes-Elstein L;Pinckney A;Furst DE;Clements PJ;Khanna D;Steen V;Schraufnagel DE;Arami S;Hsu V;Roth MD;Elashoff RM;Sullivan KM;SLS I and SLS II study groups
评估参加硬皮病肺研究（SLS）I和II的系统性硬化症-间质性肺病（SSc-ILD）患者的生存率并确定生存率预测因素。SLS I将158例SSc-ILD患者随机分配至1年口服环磷酰胺（CYC）与安慰剂组。SLS II将142例患者随机分为1年口服CYC组，随后是1年安慰剂组和2年霉酚酸酯（MMF）组。计数过程考克斯比例风险模型确定了与SLS I和II长期死亡率相关的变量。使用联合建模进行内部确认。在中位随访8年后，42%的SLS I患者死亡，当已知时，死亡原因最常归因于SSc。在SLS I或II中，治疗组之间的至死亡时间无显著差异。基线皮肤评分较高、年龄较大、用力肺活量（FVC）和一氧化碳弥散量（DLCO）下降超过2年与SLS I死亡风险增加独立相关。考克斯模型使用SLS II数据确定了相同的死亡率预测变量。除了确定SSc中的传统死亡风险因素（皮肤评分、年龄）外，本研究还证明，与基线FVC和DLCO相比，2年内FVC和DLCO的下降是更好的死亡预测因子。这些结果表明，SSc-ILD进展替代指标的短期变化可能对长期结局产生重要影响。
To assess survival and identify predictors of survival in patients with systemic sclerosis-interstitial lung disease (SSc-ILD) who participated in the Scleroderma Lung Studies (SLS) I and II. SLS I randomized 158 SSc-ILD patients to 1 year of oral cyclophosphamide (CYC) versus placebo. SLS II randomized 142 patients to 1 year of oral CYC followed by 1 year of placebo versus 2 years of mycophenolate (MMF). Counting process cox proportional hazard modeling identified variables associated with long-term mortality in SLS I and II. Internal validation was performed using joint modeling. After a median follow-up of 8 years, 42% of SLS I patients died, and when known, the cause of death was most often attributable to SSc. There was no significant difference in the time to death between treatment arms in SLS I or II. Higher baseline skin score, older age, and a decline in the forced vital capacity (FVC) and the diffusing capacity for carbon monoxide (DLCO) over 2 years were independently associated with an increased risk of mortality in SLS I. The Cox model identified the same mortality predictor variables using the SLS II data. In addition to identifying traditional mortality risk factors in SSc (skin score, age), this study demonstrated that a decline in the FVC and the DLCO over 2 years was a better predictor of mortality than the baseline FVC and DLCO. These findings suggest that short-term changes in surrogate measures of SSc-ILD progression may have important effects on long-term outcomes.
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影响因子：
--
作者：
STEEN, VD;CONTE, C;MEDSGER, TA
通讯作者：
MEDSGER, TA
影响因子：
158.5
作者：
Tashkin, Donald P.;Elashoff, Robert;Metersky, Mark
通讯作者：
Metersky, Mark
DOI：
10.14740/jocmr2606w
发表时间：
2016-09
期刊：
Journal of clinical medicine research
影响因子：
--
作者：
Zeineddine N;Khoury LE;Mosak J
通讯作者：
Mosak J
影响因子：
3
作者：
BOZDOGAN, H
通讯作者：
BOZDOGAN, H
影响因子：
158.5
作者：
Sullivan, K. M.;Goldmuntz, E. A.;Furst, D. E.
通讯作者：
Furst, D. E.