Background In systemic sclerosis (SSc), digital ulcers (DU) are debilitating and sometimes recurrent complications. Treatment of DU is not dissociable from that of Raynaud’s phenomenon (RF), both express the same microvascular disfunction at different stages.
Objectives Our goal is clinical characterization of a population of SSc patients with DU, its treatment, complications and outcome.
Methods Retrospective analysis of 46 consecutive SSc patients between 1999-2012, meeting ACR or Le Roy classification criteria, from our department in a general hospital serving approximately 200.000 inhabitants. Clinical data were obtained from patient files.
Results DU were detected in 10 patients: three males and 7 females, with a mean age of 60,2 years (range 48-76 years). Nine patients had limited type SSc (two had digital calcinosis) and one had diffuse type scleroderma. Anticentromere autoantibodies (ACA) were positive in 8 patients, anti-topoisomerase antibodies were present in one patient. Antiphospholipid antibodies were determined in six patients and were positive in one. All 10 patients were treated with calcium channel blockers (CCB) for RF. Healing of DU was made with intravenous prostanoid in 9 patients, four patients required repeated treatment with prostanoid for DU healing, one of these has a very slowly healing ulcer and positive antiphospholipid autoantibodies (APLA). After DU healing, there was persistent RF in three patients, two of them required repeated prostanoid infusions. Nine patients were later treated with bosentan, one of which experienced recurrence of DU. Alteration of liver function tests (LFT) was present in three patients: persistent in one patient with a history of alcohol intake, so bosentam was stopped; two were previously exposed to methotrexate, and LFT improved after folinic acid theraphy. Antiaggregant therapy was prescribed in nine patients and oral anticoagulants in two patients. Two patients had digital loss and two other patients healed with scars. Patients with digital loss were late presenters. The only patient that required hospitalization presented at diagnosis with pulmonary arterial hypertension (PAH), DU and digital loss; PAH normalized after treatment with bosentan. Two patients are dead: one died with massive digestive bleeding; the other died of liver failure, he had a history of alcohol intake. One patient is lost to follow-up. Three patients are still professionally active.
Conclusions DU are serious manifestations of vasculopathy, a sign of target-organ damage; they are associated with increased morbidity (hand disability, reduced quality of life) and mortality. Treatment of DU comprises detection of complications, promotion of healing and preventing recurrence. Patients with SSc and DU have a worse prognosis, but there is sufficient evidence to support recommendations on standard of care and the most effective drugs available. Intravenous prostanoids and CCB have the highest level of evidence. Bosentan is recommended to prevent recurrence of DU, improving outcome. In this small series, patients with worse outcome were older, had longer follow-up and late diagnosis, already with organ damage. Younger and more recent patients have better results, probably mirroring improvement in quality of care.
Disclosure of Interest None Declared
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