Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts. Issue 1 (8th November 2018)

• Record Type: Journal Article
• Authors: Volkmann, Elizabeth R; Tashkin, Donald P; Sim, Myung; Li, Ning; Goldmuntz, Ellen; Keyes-Elstein, Lynette; Pinckney, Ashley; Furst, Daniel E; Clements, Philip J; Khanna, Dinesh; Steen, Virginia; Schraufnagel, Dean E; Arami, Shiva; Hsu, Vivien; Roth, Michael D; Elashoff, Robert M; Sullivan, Keith M
• Other Names: Clements PJ author non-byline.; Tashkin DP author non-byline.; Elashoff R author non-byline.; Goldin J author non-byline.; Roth M author non-byline.; Furst D author non-byline.; Bulpitt K author non-byline.; Khanna D author non-byline.; Chung W-LJ author non-byline.; Viasco S author non-byline.; Sterz M author non-byline.; Woolcock L author non-byline.; Yan X author non-byline.; Ho J author non-byline.; Vasunilashorn S author non-byline.; Costa I da author non-byline.; Seibold JR author non-byline.; Riley DJ author non-byline.; Amorosa JK author non-byline.; Hsu VM author non-byline.; McCloskey DA author non-byline.; Wilson JE author non-byline.; Varga J author non-byline.; Schraufnagel D author non-byline.; Wilbur A author non-byline.; Lopata M author non-byline.; Arami S author non-byline.; Cole-Saffold P author non-byline.; Simms R author non-byline.; Theodore A author non-byline.; Clarke P author non-byline.; Korn J author non-byline.; Tobin K author non-byline.; Nuite M author non-byline.; Silver R author non-byline.; Bolster M author non-byline.; Strange C author non-byline.; Schabel S author non-byline.; Smith E author non-byline.; Arnold J author non-byline.; Caldwell K author non-byline.; Bonner M author non-byline.; Wise R author non-byline.; Wigley F author non-byline.; White B author non-byline.; Hummers L author non-byline.; Bohlman M author non-byline.; Polito A author non-byline.; Leatherman G author non-byline.; Forbes E author non-byline.; Daniel M author non-byline.; Steen V author non-byline.; Read C author non-byline.; Cooper C author non-byline.; Wheaton S author non-byline.; Carey A author non-byline.; Ortiz A author non-byline.; Mayes M author non-byline.; Parsley E author non-byline.; Oldham S author non-byline.; Filemon T author non-byline.; Jordan S author non-byline.; Perry M author non-byline.; Connolly K author non-byline.; Golden J author non-byline.; Wolters P author non-byline.; Webb R author non-byline.; Davis J author non-byline.; Antolos C author non-byline.; Maynetto C author non-byline.; Fessler B author non-byline.; Olman M author non-byline.; Sanders C author non-byline.; Heck L author non-byline.; Parkhill T author non-byline.; Rothfield N author non-byline.; Metersky M author non-byline.; Cobb R author non-byline.; Aberles M author non-byline.; Ingenito F author non-byline.; Breen E author non-byline.; Mayes M author non-byline.; Mubarak K author non-byline.; Granda JL author non-byline.; Silva J author non-byline.; Injic Z author non-byline.; Alexander R author non-byline.; Furst D author non-byline.; Springmeyer S author non-byline.; Kirkland S author non-byline.; Molitor J author non-byline.; Hinke R author non-byline.; Mondt A author non-byline.; Thompson T author non-byline.; Rounds S author non-byline.; Weinstein M author non-byline.; Thompson B author non-byline.; Paulus H author non-byline.; Levy S author non-byline.; Martin D author non-byline.; Theodore AC author non-byline.; Simms RW author non-byline.; Kissin E author non-byline.; Cheong FY author non-byline.; Steen VD author non-byline.; Read Jr CA author non-byline.; Fridley C author non-byline.; Zulmatashvili M author non-byline.; Wise RA author non-byline.; Wigley FM author non-byline.; Hummers L author non-byline.; Leatherman G author non-byline.; Silver RM author non-byline.; Strange C author non-byline.; Hant FN author non-byline.; Ham J author non-byline.; Gibson K author non-byline.; Rosson D author non-byline.; Tashkin DP author non-byline.; Elashoff RM author non-byline.; Roth MD author non-byline.; Clements PJ author non-byline.; Furst D author non-byline.; Kafaja S author non-byline.; Kleerup E author non-byline.; Elashoff D author non-byline.; Goldin J author non-byline.; Ariola E author non-byline.; Marlis G author non-byline.; Mason-Berry J author non-byline.; Saffold P author non-byline.; Rodriguez M author non-byline.; Guzman L author non-byline.; Brook J author non-byline.; Golden J author non-byline.; Connolly MK author non-byline.; Eller A author non-byline.; Leong D author non-byline.; Lalosh M author non-byline.; Obata J author non-byline.; Varga J author non-byline.; Dematte J author non-byline.; Hinchcliff M author non-byline.; DeLuca C author non-byline.; Donnelly H author non-byline.; Marlin C author non-byline.; Riley DJ author non-byline.; Hsu VM author non-byline.; McCloskey DA author non-byline.; Phillips K author non-byline.; Khanna D author non-byline.; Martinez FJ author non-byline.; Schiopu E author non-byline.; Konkle J author non-byline.; Mayes M author non-byline.; Patel B author non-byline.; Assassi S author non-byline.; Tan F author non-byline.; Fischer A author non-byline.; Swigris J author non-byline.; Meehan R author non-byline.; Brown K author non-byline.; Warren T author non-byline.; Morrison M author non-byline.; Scholand MB author non-byline.; Frecht T author non-byline.; Carey P author non-byline.; Villegas M author non-byline.; Molitor J author non-byline.; Carlson P author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 78:Issue 1(2019)
• Page Start: 122
• Page End: 130
• Publication Date: 2018-11-08
• Subjects: systemic sclerosis -- interstitial lung disease -- treatment -- survival -- systemic sclerosis -- interstitial lung disease -- treatment -- survival; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-213708
• Languages: English
• ISSNs: 0003-4967

Abstract

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.