OBJECTIVES: To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in systemic sclerosis (SSc). METHODS: The EUSTAR database was first searched. A casecontrol study of a patient subset was then performed to further identify independent factors associated with LV dysfunction by simple and multiple regression. RESULTS: Of 7073 patients, 383 (5.4%) had an LV ejection fraction (EF) of <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension were associated with LV dysfunction. In the second phase, 129 patients with SSc with LVEF <55% were compared with 256 patients with SSc with normal LVEF. Male sex (OR 3.48; 95% CI 1.74 to 6.98), age (OR 1.03; 95% CI 1.01 to 1.06), digital ulcerations (OR 1.91; 95% CI 1.05 to 3.50), myositis (OR 2.88; 95% CI 1.15 to 7.19) and use of calcium channel blockers (OR 0.41; 95% CI 0.22 to 0.74) were independent factors associated with LV dysfunction. CONCLUSION: The prevalence of LV dysfunction in SSc is 5.4%. Age, male gender, digital ulcerations, myositis and lung involvement are independently associated with an increased prevalence of LV dysfunction. Conversely, the use of calcium channel blockers may be protective. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of systemic sclerosis patientsAllanore Y (1), Meune C (2), Vonk MC (3), Airo P (4), Hachulla E (5), Caramaschi P (6), Riemekasten G (7), Cozzi F(8), Beretta L (9), Derk CT (10) AbstractStudy objectives and methods To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in SSc, we first queried the EUSTAR database. In a second phase, we performed a case-control study of a patient subset, to further identify independent factors associated with LV dysfunction by simple and multiple regression. Results Among 7,073 patients, 383 (5.4%) had a LV ejection fraction (EF) <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension (PAH) were associated with LV dysfunction. In a second phase, 129 SSc patients with LVEF <55% were compared with 256 SSc patients with normal LVEF. Male sex (OR 3.48; 95% CI1.74-6.98), age (OR 1.03; 95% CI 1.01-1.06), digital ulcerations (OR 1.91; 95% CI 1.05-3.50), myositis (OR 2.88; 95% CI 1.15-7.19), and calcium channel blockers (CCB) use (OR 0.41; 95% CI 0.22-0.74) were independent factors associated with LV dysfunction.

Scleroderma is a disorder involving oral and facial tissues, with skin hardening, thin lips, deep wrinkles, xerostomia, tongue rigidity, and microstomia. The aim of this study was to investigate the prevalence of oral manifestations and temporomandibular disorders (TMD) in Systemic Sclerosis (SSc) patients compared with healthy people. Eighty patients (6 men, 74 women) fulfilling ACR/EULAR SSc Criteria were enrolled. A randomly selected group of 80 patients, matched by sex and age served as control group. The examination for TMD signs and symptoms was based on the standardized Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD) through a questionnaire and clinical examination. SSc patients complained more frequently (78.8%) of oral symptoms (Xerostomia, dysgeusia, dysphagia and stomatodynia) than controls (28.7%) (χ2 = 40.23 p = 0.001). TMD symptoms (muscle pain on chewing, difficulty in mouth opening, headaches) were complained by 92.5% of SSc patients and by 76.2% of controls (χ2 = 8.012 p = 0.005). At the clinical examination, 85% of SSc patients showed restricted opening versus 20.0% of controls (χ2 = 67.77 p = 0.001), 81.2% of SSc showed reduced right lateral excursion versus 50% of controls (χ2 = 17.316 p = 0.001); 73.8% of SSc showed limited left lateral excursion versus 53.8% of controls (χ2 = 6.924 p = 0.009); and 73.8% of SSc had narrow protrusion versus 56.2% of controls (χ2 = 5.385 p = 0.02).

In real-world settings, TNF inhibitors showed a high rate of drug survival at 4 years. Further, the need for glucocorticoids for controlling active PsA was lowered with time.

Microcirculatory blood flow increases following 3 days of iloprost infusion but fades shortly after treatment. Although iloprost is effective in reducing the severity of RP in SSc, the most suitable regimen and timing to obtain longer lasting vasodilatory benefits remain to be established.

BackgroundInterstitial lung disease (ILD) is a typical clinical manifestation in Systemic Sclerosis (SSc). The high resolution chest computed tomography (HRCT) is the gold standard to evaluate and grade ILD. The presence of fibrotic tissue in the lung, or other structures beside the air, allows obtaining specific sonography images (such as ultrasound lung comet, higher pleural line thickness, irregular pleural margins and subpleural cysts) by chest ultrasound. In previous studies it was demonstrated that the ultrasound comets number increases in SSc patients.ObjectivesWe aimed at correlating the specific lung sonography signs with ground glass and honeycombing pattern detected by chest HRCT.MethodsA total of 60 SSc outpatients (54 female, mean age 56,2 ± 13,8 ys and disease duration of 9,57±8,7 ys), who fulfilled ACR/EULAR 2013 SSc classification criteria, were recruited. All patients underwent chest HRCT and US examination. The US examination was performed with 7,5 Mhz probe and conducted with patients in sitting position from paraspinal line to anterior axillary line, for each intercostals space. The presence of typical ultrasound signs as lung comets (pathological if >35), higher pleural line thickness (pathological if >2mm), irregular pleural margins, subpleural cysts and pleural effusion was detected. The sensitivity, specificity and accuracy of US patterns (compared to chest HRTC) were evaluated by ROC analysis. Statistic significance was set at p<0.05. All results are expressed as mean ± 1 standard deviation.Results23 patients had the CT honeycombing pattern, of which 92% had the US irregular pleural margins, 70% had US higher pleural line thickness and sub-pleural cysts, just 54% had US lung comets. 16 patients had the CT ground glass pattern, of these in 94% the US irregular pleural margins, in 87% US lung comets, in 60% US higher pleural line thickness were found. As regard the CT ground glass pattern the US sign with the highest specificity (91%), sensibility (85,7%) and accuracy (88%) was the lung comets; while regarding the CT honeycombing pattern the US sign with the highest specificity (92%), sensibility (69,6%) and accuracy (82%).ConclusionsThe lung US is a good diagnostic technique for its repeatability, low cost and risklessness. Although HRCT remains the best imaging technique to assess the ILD, in SSC the lung US could be a useful tool to detect the presence or the evolution of ILD and to improve the timing of HRTC without exposing the patients to high radiation doses over time.Disclosure of InterestNone declared