The aim of the present study was to retrospectively evaluate response to therapy in 73 patients affected by systemic sclerosis (SSc) who underwent long-term cyclic treatment with intravenous iloprost for peripheral vascular involvement (average duration of treatment 54.12±41.04 months). Seventy-three SSc patients were enrolled. Data were collected by reviewing clinical records and by phone or direct interview. Patients underwent a thorough physical examination at the end of follow up. The incidence of severe vascular manifestations was also assessed. Statistical analysis was performed by Wilcoxon's signed rank test and descriptive statistics using Statview software. In this study cohort, 55 of 73 (75.2%) patients had a history of ischemic digital ulcers (DUs); 28 patients (38.4%) had active DUs at the beginning of treatment. Skin ulcers healed completely in 25 of 28 patients (89.3%) at the end of the first treatment. However, 40 of 55 patients (72.6%) relapsed after an average of 24 months. There was a significant correlation between relapse rate and/or number of ulcers and clinical factors (diffuse subset, changes in results of Allen's test, NT-pro BNP levels). The annual incidence of pulmonary arterial hypertension (PAH) was 2.34 (95%CI: 0.94-4.83) per 100 person years, the rate of gangrene was 2.7%, and no cases of scleroderma renal crisis were recorded. The incidence of PAH and of digital gangrene was higher than that observed in unselected SSc case series. These data suggest that our patients treated with iloprost have a higher vascular involvement than large case series of unselected SSc patients. A number of clinical factors are correlated to the severity of vascular involvement and could have an impact on the response to therapy. The clinical significance of these findings requires clarification and further investigation is needed.

Background Scleroderma Spectrum Diseases (SSDs) are characterized by alterations of the microarchitecture of small arteries. Recently, gadolinium enhanced MRA has been used as a safe and accurate technique for the evaluation of vascular pathologies of the small arteries of the hands1. Objectives the aims of the present study were to: 1. evaluate the feasibility of unenhanced MRA in healthy controls (HC) and in SSDs. 2. outline differences between HC and SSDs 3. assess the capability of unenhanced MRA to measure the severity of vascular damage in SSDs. Methods MRA was performed both in HS and in consecutive SSDs patients: both hands were assessed with a 3 tesla system (Discovery MR750, GE) using 3D delta flow MRA. All of the patients performed a standardized clinical-instrumental assessment to evaluate organ involvement as well as capillaroscopy, with classification of the pattern according to Cutolo et al. Results 10 HC and 7 consecutive SSDs were enrolled in the period from September to December 2011. 3 patients were classified, according to ACR criteria, as limited cutaneous SSc (Lc-SSc), 2 as diffuse cutaneous SSc (Dc-Ssc), 1 patient fulfilled very early diagnosis of systemic sclerosis (VEDOSS) criteria and 1 was classified as mixed connective tissue disease (MCTD). All of the patients were female with a mean age at the evaluation of 46 years [30-71], mean disease duration 5.9 years [1-9]. Five patients had a history of digital ulcers (DUs), 2 had active DUs, all had esophageal involvement. Five patients were affected by interstitial lung disease and no case of pulmonary arterial hypertension was recorded. Table 1 summarizes the clinical data of the patients. Whereas in HC proper digital arteries (PDAs) were depictet in 100% of the cases (fully depicted in 7/10, partially depicted in 3/10), in SSDs these vessels were depicted in only 57% of the cases (fully: 3 partially: 1). In SSDs patients, abnormalities of MRA findings were correlated to Allen test alteration, moreover, 3/5 of the patients with a history of DUs had major alteration of proper digital arteries. SubsetAllen testDUs historyActive DUsCapillary densityCapillaroscopic patternDetectable PDAs artery Lc-SScNormalNo02,875LateThin PDAs MCTDNormalYes13,75LateNormal Dc-SScAbnormalYes04,5ActiveNot depictable Lc-SScNormalYes03LateNormal Lc-SScAbnormalYes04ActiveNot depictable VEDOSSNormalNo06EarlyNormal Dc-SSCAbnormalYes12,93LateNot depictable Conclusions vascular involvement is important in the pathogenesis of digital skin ulcers in SSDs. Unhenanced MRA seems to be feasible and able to improve the assessment of vascular involvement in these disorders. However, the unenhanced MRA technique needs to be validated on larger number of patients in order to establish if it can measure the severity of vascular involvement and predict the development of digital ulcers and if it could replace contrast-enhanced MRA. References Allanore Y, Seror R et al. Hand vascular involvement assessed by magnetic resonance angiography in Systemic Sclerosis. Arthritis & R...