Natural history of ischemic digital ulcers in systemic sclerosis: single-center retrospective longitudinal study.
OBJECTIVE
To describe the natural history of ischemic digital ulcers (DU) in systemic sclerosis (SSc).


METHODS
This single-center, retrospective, longitudinal study identified patients by demographic data, SSc history and type, Rodnan score, tobacco use, presence of autoantibodies, ongoing treatment, and DU history.


RESULTS
One hundred three patients were enrolled, 46 with DU history and 57 without; 2 with DU were excluded. The mean duration of followup from the first non-Raynaud SSc symptoms was 12.3 +/- 6.3 years in patients with DU history and 12.1 +/- 7.0 years in patients without. In 43% of cases, first DU occurred within 1 year following first non-Raynaud SSc symptoms, and within 5 years in 73% of cases. In a multivariate analysis, younger patients at occurrence of first non-Raynaud SSc symptoms (HR = 0.77 per each 5 years older, 95% CI 0.66-0.90) with higher Rodnan scores (HR = 1.21 per 5 points, 95% CI 1.05-1.47) experienced earlier DU occurrences, which were delayed by vasodilator therapy (HR = 0.17, 95% CI 0.09-0.32). Patients with shorter durations between first and second DU episodes, particularly with a second episode within 2 years of the first, experienced a higher yearly incidence of DU episodes (0.85 +/- 0.57 and 0.48 +/- 0.26, respectively, if less or more than 2 yrs; p = 0.04). Throughout the duration of followup, the incidence of finger amputation was 1.2% per patient-year in patients with DU history.


CONCLUSIONS
Patients who are young at first sign of SSc, with high Rodnan scores and without vasodilator therapy, are at high risk of developing DU. Development of DU typically occurred within 5 years of the first non-Raynaud clinical symptom of SSc in the majority of patients.
Hachulla
Eric
E
Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille, Lille, France. ehachulla@chru-lille.fr
Clerson
Pierre
P
Launay
David
D
Lambert
Marc
M
Morell-Dubois
Sandrine
S
Queyrel
Viviane
V
Hatron
Pierre-Yves
PY
D000328:Adult; D005260:Female; D005385:Fingers / Q000098:blood supply*; D006801:Humans; D007511:Ischemia; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D012883:Skin Ulcer / Q000209:etiology* / Q000503:physiopathology