Evidence map›Paper›PMID 30409830›Full record

Trial reportAnnals of the rheumatic diseases2019

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

Elizabeth R Volkmann, Donald P Tashkin, Myung Sim, Ning Li, Ellen Goldmuntz, Lynette Keyes-Elstein, Ashley Pinckney, Daniel E Furst, Philip J Clements, Dinesh Khanna and 8 more

Abstract readRandomized Controlled Trial
PubMed Full text at PMC Publisher
In one paragraph

Trial report in Annals of the rheumatic diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 94 papers, 6 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
94citing papers in PubMed, 6 pooled it
15.3field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

94 citing papers in PubMed, 6 syntheses or guidelines pooled it, 196 citations in OpenAlex.

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  15. Prevalence and Prognostic Impact of Progressive Pulmonary Fibrosis.Tuberculosis and respiratory diseases · 2026
    Review
  16. Review
  17. Article
  18. Article
  19. Article
  20. Treating connective tissue disease-associated interstitial lung disease - think outside the box: a perspective.European respiratory review : an official journal of the European Respiratory Society · 2025
    Review

34 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

18 authors at 7 institutions in 1 country.

Elizabeth R VolkmannDepartment of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA evolkmann@mednet.ucla.edu.
Donald P TashkinDepartment of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA.
Myung SimDepartment of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA.
Ning LiDepartment of Biomathematics, University of California, Los Angeles, California, USA.
Ellen GoldmuntzNIAID, NIH, Bethesda, Maryland, USA.
Lynette Keyes-ElsteinRho Federal Systems, Inc., Chapel Hill, North Carolina, USA.
Ashley PinckneyRho Federal Systems, Inc., Chapel Hill, North Carolina, USA.
Daniel E FurstDepartment of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA.
Philip J ClementsDepartment of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA.
Dinesh KhannaDepartment of Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Virginia SteenDepartment of Medicine, Georgetown University, Washington, District of Columbia, USA.
Dean E SchraufnagelDepartment of Medicine, University of Illinois Hospital, Chicago, Illinois, USA.
Shiva AramiDepartment of Medicine, University of Illinois Hospital, Chicago, Illinois, USA.
Vivien HsuDepartment of Medicine, Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
Michael D RothDepartment of Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, California, USA.
Robert M ElashoffDepartment of Biomathematics, University of California, Los Angeles, California, USA.
Keith M SullivanDepartment of Medicine, Duke University, Durham, North Carolina, USA.
SLS I and SLS II study groups
University of California, Los Angeles · USRho (United States) · USUniversity of Illinois Chicago · USDuke University · USGeorgetown University · USJohnson University · USUniversity of Michigan–Ann Arbor · US

Funding

Transplantation GroupUM2AI117870 · NIAID · RHO FEDERAL SYSTEMS DIVISION, INC. · PI DAVID, GLORIA · 2015 to 2023
$208.1M
MOCSLID-DCCR01HL089901 · NHLBI · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI ELASHOFF, ROBERT M · 2008 to 2012
$9.1M
STEM CELL TRANSPLANTATION FOR TX OF AUTOIMMUNE DISEASES-N01AI5419N01AI005419 · NIAID · DUKE UNIVERSITY · PI SULLIVAN, KEITH M · 2000 to 2006
$6.2M
SCLERODERMA LUNG STUDYU01HL060587 · NHLBI · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI TASHKIN, DONALD P · 1999 to 2003
$2.3M
TRPV4-PI3K Axis Mediates Pulmonary and Cardiac FibrosisR01HL133721 · NHLBI · CLEVELAND CLINIC LERNER COM-CWRU · PI OLMAN, MITCHELL ALAN, PRASAD, SATHYAMANGLA V · 2017 to 2020
$2.2M
MOCSLID-CCCR01HL089758 · NHLBI · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI TASHKIN, DONALD P · 2008 to 2012
$2.0M
Myofibroblast differentiation and fibrosis are mediated by TRPV4 mechanosensingR01HL119792 · NHLBI · CLEVELAND CLINIC LERNER COM-CWRU · PI OLMAN, MITCHELL ALAN · 2014 to 2017
$1.6M
SCLERODERMA LUNG STUDYU01HL060606 · NHLBI · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI ELASHOFF, ROBERT M · 1999 to 2006
$1.3M
Systemic Sclerosis-Associated Interstitial Lung Disease Response IndexR01AR070470 · NIAMS · UNIVERSITY OF MICHIGAN AT ANN ARBOR · PI KHANNA, DINESH · 2017 to 2019
$642k
NHLBI NIH HHS R01 HL089758NHLBI NIH HHS R01 HL089901NHLBI NIH HHS R01 HL119792NHLBI NIH HHS R01 HL133721NHLBI NIH HHS U01 HL060587NHLBI NIH HHS U01 HL060606NIAID NIH HHS HHSN272201100025CNIAID NIH HHS N01 AI005419NIAID NIH HHS UM2 AI117870NIAMS NIH HHS R01 AR070470
6 · The paper itself

Abstract

objectiveTo 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.

methodsSLS 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.

resultsAfter 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.

conclusionIn 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.

Indexed as

AdultCarbon MonoxideCyclophosphamideDisease ProgressionDrug Administration ScheduleFemaleHumansImmunosuppressive AgentsLung Diseases, InterstitialMaleMiddle AgedMycophenolic AcidProportional Hazards ModelsPulmonary Diffusing CapacityRisk FactorsScleroderma, SystemicCarbon MonoxideCyclophosphamideImmunosuppressive AgentsMycophenolic Acidinterstitial lung diseasesurvivalsystemic sclerosistreatment

Identifiers

PMID30409830
PMCPMC6311344
OpenAlexW2900158663

What OpenQuestion holds

Textmetadata
LicenceTDM
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.