Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis

A 10-year longitudinal study from the EUSTAR database

Elina G. Wirz, Veronika K. Jaeger, Yannick Allanore, Gabriela Riemekasten, Eric Hachulla, Oliver Distler, Paolo Airó, Patricia E. Carreira, Mohammed Tikly, Serena Vettori, Alexandra Balbir Gurman, Nemanja Damjanov, Ulf Müller-Ladner, Jörg Hw Distler, Mangtao Li, Peter Häusermann, Ulrich A. Walker, Raffaella Scorza

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud's phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan-Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.

Original languageEnglish
Pages (from-to)1285-1292
Number of pages8
JournalAnnals of the Rheumatic Diseases
Volume75
Issue number7
DOIs
Publication statusPublished - Jul 1 2016

Fingerprint

Skin Manifestations
Systemic Scleroderma
antineoplaston A10
Raynaud Disease
Longitudinal Studies
Skin
Databases
Autoantibodies
Incidence
Research
Ulcer
Diffuse Scleroderma
RNA Polymerase III
Observation
Regression analysis
Hazards
Sclerosis
Kinetics
Leg
Arm

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis : A 10-year longitudinal study from the EUSTAR database. / Wirz, Elina G.; Jaeger, Veronika K.; Allanore, Yannick; Riemekasten, Gabriela; Hachulla, Eric; Distler, Oliver; Airó, Paolo; Carreira, Patricia E.; Tikly, Mohammed; Vettori, Serena; Gurman, Alexandra Balbir; Damjanov, Nemanja; Müller-Ladner, Ulf; Distler, Jörg Hw; Li, Mangtao; Häusermann, Peter; Walker, Ulrich A.; Scorza, Raffaella.

In: Annals of the Rheumatic Diseases, Vol. 75, No. 7, 01.07.2016, p. 1285-1292.

Research output: Contribution to journalArticle

Wirz, EG, Jaeger, VK, Allanore, Y, Riemekasten, G, Hachulla, E, Distler, O, Airó, P, Carreira, PE, Tikly, M, Vettori, S, Gurman, AB, Damjanov, N, Müller-Ladner, U, Distler, JH, Li, M, Häusermann, P, Walker, UA & Scorza, R 2016, 'Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: A 10-year longitudinal study from the EUSTAR database', Annals of the Rheumatic Diseases, vol. 75, no. 7, pp. 1285-1292. https://doi.org/10.1136/annrheumdis-2015-207271
Wirz, Elina G. ; Jaeger, Veronika K. ; Allanore, Yannick ; Riemekasten, Gabriela ; Hachulla, Eric ; Distler, Oliver ; Airó, Paolo ; Carreira, Patricia E. ; Tikly, Mohammed ; Vettori, Serena ; Gurman, Alexandra Balbir ; Damjanov, Nemanja ; Müller-Ladner, Ulf ; Distler, Jörg Hw ; Li, Mangtao ; Häusermann, Peter ; Walker, Ulrich A. ; Scorza, Raffaella. / Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis : A 10-year longitudinal study from the EUSTAR database. In: Annals of the Rheumatic Diseases. 2016 ; Vol. 75, No. 7. pp. 1285-1292.
@article{155c6bea3a58488db2ec9d1a1ea27bb8,
title = "Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: A 10-year longitudinal study from the EUSTAR database",
abstract = "Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud's phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan-Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69{\%} and 25{\%}, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36{\%} during the subsequent 2 years. Only 6{\%} of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70{\%} at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.",
author = "Wirz, {Elina G.} and Jaeger, {Veronika K.} and Yannick Allanore and Gabriela Riemekasten and Eric Hachulla and Oliver Distler and Paolo Air{\'o} and Carreira, {Patricia E.} and Mohammed Tikly and Serena Vettori and Gurman, {Alexandra Balbir} and Nemanja Damjanov and Ulf M{\"u}ller-Ladner and Distler, {J{\"o}rg Hw} and Mangtao Li and Peter H{\"a}usermann and Walker, {Ulrich A.} and Raffaella Scorza",
year = "2016",
month = "7",
day = "1",
doi = "10.1136/annrheumdis-2015-207271",
language = "English",
volume = "75",
pages = "1285--1292",
journal = "Annals of the Rheumatic Diseases",
issn = "0003-4967",
publisher = "BMJ Publishing Group",
number = "7",

}

TY - JOUR

T1 - Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis

T2 - A 10-year longitudinal study from the EUSTAR database

AU - Wirz, Elina G.

AU - Jaeger, Veronika K.

AU - Allanore, Yannick

AU - Riemekasten, Gabriela

AU - Hachulla, Eric

AU - Distler, Oliver

AU - Airó, Paolo

AU - Carreira, Patricia E.

AU - Tikly, Mohammed

AU - Vettori, Serena

AU - Gurman, Alexandra Balbir

AU - Damjanov, Nemanja

AU - Müller-Ladner, Ulf

AU - Distler, Jörg Hw

AU - Li, Mangtao

AU - Häusermann, Peter

AU - Walker, Ulrich A.

AU - Scorza, Raffaella

PY - 2016/7/1

Y1 - 2016/7/1

N2 - Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud's phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan-Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.

AB - Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud's phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan-Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.

UR - http://www.scopus.com/inward/record.url?scp=84940202098&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84940202098&partnerID=8YFLogxK

U2 - 10.1136/annrheumdis-2015-207271

DO - 10.1136/annrheumdis-2015-207271

M3 - Article

VL - 75

SP - 1285

EP - 1292

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

SN - 0003-4967

IS - 7

ER -