title: Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: a 10-year longitudinal study from the EUSTAR database.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
  
  
  
  Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.
authors.affiliation: Department of Rheumatology, University Hospital Basel, Basel, Switzerland Department of Dermatology, University Hospital Basel, Basel, Switzerland.
authors.forename: Elina G
authors.initials: EG
authors.lastname: Wirz
authors.affiliation: Department of Rheumatology, University Hospital Basel, Basel, Switzerland.
authors.forename: Veronika K
authors.initials: VK
authors.lastname: Jaeger
authors.affiliation: Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Paris, France.
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
authors.affiliation: Department of Rheumatology, Charité University Hospital, Berlin, Germany German Rheumatism Research Centre (DRFZ), Leibniz Institute, Berlin, Germany.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Riemekasten
authors.affiliation: Department of Internal Medicine, Hôpital Claude Huriez, University Lille, Lille Cedex, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.affiliation: Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.affiliation: Rheumatology and Clinical Immunology Service, Spedali Civili di Brescia, Brescia, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Airò
authors.affiliation: Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain.
authors.forename: Patricia E
authors.initials: PE
authors.lastname: Carreira
authors.affiliation: Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Mohammed
authors.initials: M
authors.lastname: Tikly
authors.affiliation: Rheumatology Department, Second University of Naples, Naples, Italy.
authors.forename: Serena
authors.initials: S
authors.lastname: Vettori
authors.affiliation: B. Shine Rheumatology Unit, Rappaport Faculty of Medicine, Rambam Health Care Campus, Technion-Institute of Technology, Haifa, Israel.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Balbir Gurman
authors.affiliation: Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia.
authors.forename: Nemanja
authors.initials: N
authors.lastname: Damjanov
authors.affiliation: Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim, Germany.
authors.forename: Ulf
authors.initials: U
authors.lastname: Müller-Ladner
authors.affiliation: Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany.
authors.forename: Jörg
authors.initials: J
authors.lastname: Distler
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Mangtao
authors.initials: M
authors.lastname: Li
authors.affiliation: Department of Dermatology, University Hospital Basel, Basel, Switzerland.
authors.forename: Peter
authors.initials: P
authors.lastname: Häusermann
authors.affiliation: Department of Rheumatology, University Hospital Basel, Basel, Switzerland.
authors.forename: Ulrich A
authors.initials: UA
authors.lastname: Walker
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood; D016208:Databases, Factual; D005260:Female; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012307:Risk Factors; D045743:Scleroderma, Diffuse / Q000453:epidemiology / Q000209:etiology; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications* / Q000473:pathology; D012720:Severity of Illness Index; D012737:Sex Factors; D012883:Skin Ulcer / Q000453:epidemiology* / Q000209:etiology*; D013997:Time Factors
chemical_list: D001323:Autoantibodies
keywords: Autoantibodies; Epidemiology; Systemic Sclerosis