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The aim of the present study was to retrospectively evaluate the risk factors and response to therapy of ischemic digital ulcers in a cohort of 75 patients affected by systemic sclerosis cyclically treated for long time with intravenous Iloprost for peripheral vascular involvement (mean: 50,41 ± 38,43 months).
75 SSc patients were enrolled. Data were collected by chart review and by phone or direct interview. Patients underwent a thorough physical examination with Adson test and questionnaires for quality of life, hand function, ulcer count and routine work up at the end of follow-up. The incidence of severe vascular manifestations during the follow-up was also assessed. Statistical analysis was performed by Wilcoxon signed rank test and descriptive statistics, using Stat View software. Patients underwent Iloprost treatment at regular intervals (mean number of cicles/months of follow up: 0,46±0,2).
55/75 (73%) patients had a history of ischemic digital ulcers. A non significant reduction of the number of ulcers was observed at the end of the treatment. Skin ulcers completely healed in 47/55 of the patients (85%). Pain and Raynaud’s visual analogue scale and global health assessement significantly improved. 39/55 patients (70%) relapsed after a mean of 24 months. Relapse rate was significantly correlated with the diffuse subset and therapy (combination therapy, higher number of cycles of Iloprost). A positive Adson test correlated with a delay in ulcer healing. Higher number of ulcers at the end of follow-up was correlated with a worse hand function. Patients with an increase of NT-pro BNP had a higher number of ulcers than patients whose values were within the normal range. The variation of NT-pro BNP during the follow-up, was correlated to the number of ulcers at the end of follow-up.
The annual incidence of pulmonary arterial hypertension (PAH) was 2.42 per 100 patient-year, the rate of gangrene was 4% and no cases of scleroderma renal crisis were recorded.
These data suggest that our patients treated with Iloprost have a higher vascular burden than the rest of SSc population as mirrored by a higher rate of digital ulcer history, gangrene and PAH annual incidence. Peripheral vascular involvement severity seem to be paralleled by escalation of treatment. NT proBNP is substantially increased in patients with digital ulcers and the variation of this marker during follow-up correlates with the load of ulcers. This finding suggests that such patients, even though asymptomatic, might be at risk of myocardial dysfunction, and thus warrant further investigation.