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Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts.

Abstract：

OBJECTIVE: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. METHODS:SLS I randomised 158 patients with SSc-ILD to 1 year of oral cyclophosphamide (CYC) vs placebo. SLS II randomised 142 patients to 1 year of oral CYC followed by 1 year of placebo vs 2 years of mycophenolate mofetil. Counting process Cox proportional hazard modelling identified variables associated with long-term mortality in SLS I and II. Internal validation was performed using joint modelling. RESULTS: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. CONCLUSION:In addition to identifying traditional mortality risk factors in SSc (skin score, age), this study demonstrated that a decline in FVC and DLCO over 2 years was a better predictor of mortality than 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.

journal_name

Ann Rheum Dis

journal_title

Annals of the rheumatic diseases

authors

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.

doi

10.1136/annrheumdis-2018-213708

subject

Has Abstract

pub_date

2019-01-01 00:00:00

pages

122-130

issue

eissn

0003-4967

issn

1468-2060

pii

annrheumdis-2018-213708

journal_volume

pub_type

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Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts.