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; Airò, Paolo; Carreira, Patricia E; Tikly, Mohammed; Vettori, Serena; Balbir Gurman, Alexandra; Damjanov, Nemanja; Müller-Ladner, Ulf; Distler, Jörg; Li, Mangtao; Häusermann, Peter; Walker, Ulrich A; Villiger, Peter (2015). 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, 75(7), pp. 1285-1292. BMJ Publishing Group 10.1136/annrheumdis-2015-207271

[img] Text
annrheumdis-2015-207271.full.pdf - Published Version
Restricted to registered users only
Available under License Publisher holds Copyright.

Download (2MB) | Request a copy

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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology, Clinical Immunology and Allergology

UniBE Contributor:

Villiger, Peter

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0003-4967

Publisher:

BMJ Publishing Group

Language:

English

Submitter:

Stefan Kuchen

Date Deposited:

13 Apr 2016 12:00

Last Modified:

26 Jun 2016 02:15

Publisher DOI:

10.1136/annrheumdis-2015-207271

PubMed ID:

26232495

Uncontrolled Keywords:

Autoantibodies, Epidemiology, Systemic Sclerosis

BORIS DOI:

10.7892/boris.80950

URI:

https://boris.unibe.ch/id/eprint/80950

Actions (login required)

Edit item Edit item
Provide Feedback