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Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort

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@article{7341537064224596834800d079dca482,
title = "Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort",
abstract = "OBJECTIVES: To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc).METHODS: We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25{\%} from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression.RESULTS: Of 1021 included patients, 78 (7.6{\%}) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10{\%} (53.6{\%} vs 34.4{\%}; p<0.001) and all-cause death (15.4{\%} vs 7.3{\%}; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10{\%} (HR 1.79, 95{\%} CI 1.20 to 2.65) and all-cause death (HR 2.58, 95{\%} CI 1.31 to 5.09).CONCLUSIONS: Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.",
keywords = "Adult, Cohort Studies, Databases, Factual, Disease Progression, Europe, Female, Fibrosis, Humans, Kaplan-Meier Estimate, Lung/physiopathology, Male, Middle Aged, Scleroderma, Diffuse/complications, Severity of Illness Index, Skin/pathology, Skin Diseases/etiology, Survival Analysis, Time Factors",
author = "Wanlong Wu and Suzana Jordan and Nicole Graf and {de Oliveira Pena}, Janethe and John Curram and Yannick Allanore and Marco Matucci-Cerinic and Pope, {Janet E} and Denton, {Christopher P} and Dinesh Khanna and Oliver Distler and {EUSTAR Collaborators} and Susanne Ullman",
note = "{\circledC} Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.",
year = "2019",
month = "5",
doi = "10.1136/annrheumdis-2018-213455",
language = "English",
volume = "78",
pages = "648--656",
journal = "Annals of the Rheumatic Diseases",
issn = "0003-4967",
publisher = "B M J Group",
number = "5",

}

RIS

TY - JOUR

T1 - Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort

AU - Wu, Wanlong

AU - Jordan, Suzana

AU - Graf, Nicole

AU - de Oliveira Pena, Janethe

AU - Curram, John

AU - Allanore, Yannick

AU - Matucci-Cerinic, Marco

AU - Pope, Janet E

AU - Denton, Christopher P

AU - Khanna, Dinesh

AU - Distler, Oliver

AU - EUSTAR Collaborators

AU - Ullman, Susanne

N1 - © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

PY - 2019/5

Y1 - 2019/5

N2 - OBJECTIVES: To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc).METHODS: We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression.RESULTS: Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09).CONCLUSIONS: Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.

AB - OBJECTIVES: To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc).METHODS: We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression.RESULTS: Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09).CONCLUSIONS: Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.

KW - Adult

KW - Cohort Studies

KW - Databases, Factual

KW - Disease Progression

KW - Europe

KW - Female

KW - Fibrosis

KW - Humans

KW - Kaplan-Meier Estimate

KW - Lung/physiopathology

KW - Male

KW - Middle Aged

KW - Scleroderma, Diffuse/complications

KW - Severity of Illness Index

KW - Skin/pathology

KW - Skin Diseases/etiology

KW - Survival Analysis

KW - Time Factors

U2 - 10.1136/annrheumdis-2018-213455

DO - 10.1136/annrheumdis-2018-213455

M3 - Journal article

VL - 78

SP - 648

EP - 656

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

SN - 0003-4967

IS - 5

ER -

ID: 59237230