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

被引：159

作者：

Volkmann, Elizabeth R. ^{[1
]}

Tashkin, Donald P. ^{[1
]}

Sim, Myung ^{[1
]}

Li, Ning ^{[2
]}

Goldmuntz, Ellen ^{[3
]}

Keyes-Elstein, Lynette ^{[4
]}

Pinckney, Ashley ^{[4
]}

Furst, Daniel E. ^{[1
]}

Clements, Philip J. ^{[1
]}

Khanna, Dinesh ^{[5
]}

Steen, Virginia ^{[6
]}

Schraufnagel, Dean E. ^{[7
,12
]}

Arami, Shiva ^{[7
]}

Hsu, Vivien ^{[8
]}

Roth, Michael D. ^{[1
]}

Elashoff, Robert M. ^{[2
]}

Sullivan, Keith M. ^{[9
]}

Clements, P. J. ^{[10
]}

Tashkin, D. P. ^{[10
]}

Elashoff, R. ^{[10
]}

Goldin, J. ^{[10
]}

Roth, M. ^{[10
]}

Furst, D. ^{[10
,22
]}

Bulpitt, K. ^{[10
]}

Khanna, D. ^{[10
]}

Chung, W-L J. ^{[10
]}

Vasco, S. ^{[10
]}

Sterz, M. ^{[10
]}

Woolcock, L. ^{[10
]}

Yan, X. ^{[10
]}

Ho, J. ^{[10
]}

Vasunilashorn, S. ^{[10
]}

da Costa, I ^{[10
]}

Seibold, J. R. ^{[11
]}

Riley, D. J. ^{[11
]}

Amorosa, J. K. ^{[11
]}

Hsu, V. M. ^{[11
]}

McCloskey, D. A. ^{[11
]}

Wilson, J. E. ^{[11
]}

Varga, J. ^{[12
,30
]}

Schraufnagel, D. ^{[12
]}

Wilbur, A. ^{[12
]}

Lopata, M. ^{[12
]}

Arami, S. ^{[12
]}

Cole-Saffold, P. ^{[12
]}

Simms, R. ^{[13
]}

Theodore, A. ^{[13
]}

Clarke, P. ^{[13
]}

Korn, J. ^{[13
]}

Tobin, K. ^{[13
]}

机构：

[1] Univ Calif Los Angeles, Dept Med, David Geffen Sch Med, Los Angeles, CA 90095 USA

[2] Univ Calif Los Angeles, Dept Biomath, Los Angeles, CA 90095 USA

[3] NIAID, NIH, 9000 Rockville Pike, Bethesda, MD 20892 USA

[4] Rho Fed Syst Inc, Chapel Hill, NC USA

[5] Univ Michigan, Dept Med, Med Sch, Ann Arbor, MI 48109 USA

[6] Georgetown Univ, Dept Med, Washington, DC USA

[7] Univ Illinois Hosp, Dept Med, Chicago, IL USA

[8] Univ Med & Dent New Jersey, Dept Med, New Brunswick, NJ USA

[9] Duke Univ, Dept Med, Durham, NC USA

[10] UCLA, Los Angeles, CA USA

[11] Univ Med & Dent New Jersey, New Brunswick, NJ USA

[12] Univ Illinois, Chicago, IL USA

[13] Boston Univ, Boston, MA 02215 USA

[14] Med Univ South Carolina, Charleston, SC 29425 USA

[15] Johns Hopkins Sch Med, Baltimore, MD USA

[16] Georgetown Univ, Washington, DC USA

[17] Univ Texas Houston, Houston, TX USA

[18] Univ Calif San Francisco, San Francisco, CA 94143 USA

[19] Univ Alabama Birmingham, Birmingham, AL USA

[20] Univ Connecticut, Ctr Hlth, Farmington, CT USA

[21] Wayne State Univ, Detroit, MI USA

[22] Virginia Mason Res Ctr, 1000 Seneca St, Seattle, WA 98101 USA

[23] Harvard Med Sch, Data Safety & Monitoring Board, Boston, MA USA

[24] Brown Univ, Vet Affairs Med Ctr, Providence, RI 02912 USA

[25] Cedars Sinai UCLA, Los Angeles, CA USA

[26] Clin Trials Surveys, Baltimore, MD USA

[27] UCLA, Mortal & Morbid Review Comm, Los Angeles, CA USA

[28] Johns Hopkins Univ, Baltimore, MD USA

[29] Univ Boston, Boston, MA USA

[30] Northwestern Univ, Evanston, IL 60208 USA

[31] Univ Michigan, Ann Arbor, MI 48109 USA

[32] Natl Jewish Hlth, Denver, CO USA

[33] Univ Utah, Salt Lake City, UT 84112 USA

[34] Univ Minnesota, Minneapolis, MN 55455 USA

来源：

ANNALS OF THE RHEUMATIC DISEASES | 2019年 / 78卷 / 01期

关键词：

STEM-CELL TRANSPLANTATION; PULSE CYCLOPHOSPHAMIDE; MODEL SELECTION; SCLERODERMA; MORTALITY; PLACEBO; TRENDS;

D O I：

10.1136/annrheumdis-2018-213708

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：122 / 130

页数：9

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