Clinical features of scleroderma patients with or without prior or current ischemic digital ulcers: post-hoc analysis of a nationwide multicenter cohort (ItinérAIR-Sclérodermie).
OBJECTIVE
Digital ulcers are the most frequent vascular manifestations of systemic sclerosis (SSc). Clinical features of patients with prior or current digital ulcers have not been extensively described. This cross-sectional analysis of a large multicenter cohort compared the characteristics of SSc patients with prior or current digital ulcers with those never affected.


METHODS
Patients with prior/current digital ulcers or never affected were identified in the cohort of SSc patients enrolled in the French ItinérAIR-Sclérodermie registry. Rodnan skin scores, pulmonary function test results, and clinical and immunological data were analyzed to identify digital ulcerassociated clinical features.


RESULTS
Of 599 SSc patients, 317 had prior or current digital ulcers. These patients were more frequently male, with impaired diffusing capacity for carbon monoxide (DLCO), and higher Rodnan skin scores than patients never affected by digital ulcers. In a multivariate analysis, male gender, early onset of SSc, increased duration of SSc, high Rodnan skin score, and presence of anti-topoisomerase I antibodies (anti-topo I) were associated with prior or current digital ulcers. Comparison of patients with current digital ulcers versus patients never affected indicated that affected patients had increased duration of SSc, impaired DLCO, increased Rodnan score, and younger age at onset of SSc.


CONCLUSIONS
Male patients with early onset SSc, more severe skin fibrosis, impaired DLCO, and anti-topo I were most likely to exhibit prior or current digital ulcers. Confirmation of these results in a prospective longitudinal study may enable identification of patients at greatest risk of developing digital ulcers, facilitating management of this disabling complication.
Tiev
Kiet Phong
KP
Service de Médecine Interne, Hôpital Saint Antoine, Paris, France.
Diot
Elisabeth
E
Clerson
Pierre
P
Dupuis-Siméon
Frédérique
F
Hachulla
Eric
E
Hatron
Pierre-Yves
PY
Constans
Joël
J
Cirstéa
Daniéla
D
Farge-Bancel
Dominique
D
Carpentier
Patrick H
PH
D000328:Adult; D000367:Age Factors; D000368:Aged; D000906:Antibodies / Q000097:blood; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D005260:Female; D005385:Fingers / Q000098:blood supply*; D005602:France; D006801:Humans; D007511:Ischemia / Q000150:complications*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D015995:Prevalence; D011446:Prospective Studies; D012008:Recurrence; D012042:Registries; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology*; D012737:Sex Factors; D012883:Skin Ulcer / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology*
D000906:Antibodies; D004264:DNA Topoisomerases, Type I