Digital ulcers in a cohort of 333 scleroderma patients

Abstract

Digital ulcers (DU) at the hands are one of the more frequent and severe complications in systemic sclerosis. Data on their prevalence and distribution in the different subsets of disease are variable in the literature. We studied the frequency of DU in a cohort of 333 scleroderma patients followed in the last 10 years in our Unit. DU have been recorded in 133 patients (39,9%), more frequently in males, in patients with cutaneous diffuse form of disease and in patients with anti-Scl70 ANA specificity. Complications of DU have been observed in 12,3% of cases. Surgery of the hands has been required in 8,7% of patients. The more effective treatment of DU are i.v. prostanoids, performed usually in day hospital, with high costs for the National Health Service. Recently the efficacy of bosentan, an oral receptor antagonist of endothelin, has been demonstrated, thus opening new perspectives in the treatment of DU in systemic sclerosis

Prediction risk chart for scleroderma digital ulcers: A composite predictive model based on capillaroscopic, demographic and clinico-serological parameters

“…Males presented an higher risk to develop DU than female (odds ratio: 12.9; Table 3); this data reflect previous observations about an overall worse prognosis of the disease in males [8,9] and, in particular, the association between male gender and DU was already suggested by other Authors [21][22][23].…”

“…DU affect about 50% of the patients, recognizing a multifactorial and only partially known pathogenesis; in addition data present in literature about risk factors are often discordant [1,5,7,10,11,13,14,[16][17][18][21][22][23]. In this context, a generalization in estimating the risk to develop DU is reasonable if prevention is the goal.…”

“…Their management, needing combined systemic and local treatments, is very challenging [13,19], even because of the frequent infectious complications [10] and the absence of validated treatment guidelines. Moreover, risk factors for the scleroderma DU are not well defined and data from literature are discordant [1,5,10,11,[21][22][23], thus the possibility to predict their appearance remains scarce. Furthermore, the pathogenesis of scleroderma DU is multifactorial; besides microvascular alterations it includes both predisposing and triggering factors [13,19].…”

Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors

“…A univariate analysis on 333 patients showed a higher prevalence of DU in patients with SSc who were male, and had diffuse skin sclerosis and anti‐Scl70 antibodies. A multivariate analysis was not performed 5 . Thus, these studies confirmed some of the risk factors we identified by univariate analysis.…”

Capillaroscopic skin ulcer risk index: A new prognostic tool for digital skin ulcer development in systemic sclerosis patients

“…Because only 50–60% of patients with SSc experience a digital skin ulcer in their clinical history (1, 18), the advantage of this approach is questionable. On the other hand, a wait‐and‐see strategy exposes the patients to potential severe complications that require hospitalization and long‐term treatments (49, 50). In the Randomized Placebo‐Controlled Study on the Prevention of Ischemic Digital Ulcers Secondary to Systemic Sclerosis 1 (42), the patient's clinical history of multiple digital ulcers represents the most helpful predictor and indicator of preventive therapy.…”