Introduction: Erectile dysfunction (ED) is common in men with systemic sclerosis (SSc) but the demographics, risk factors and treatment coverage for ED are not well known. Method: This study was carried out prospectively in the multinational EULAR Scleroderma Trial and Research database by amending the electronic data-entry system with the International Index of Erectile Function-5 and items related to ED risk factors and treatment. Centres participating in this EULAR Scleroderma Trial and Research substudy were asked to recruit patients consecutively. Results: Of the 130 men studied, only 23 (17.7%) had a normal International Index of Erectile Function-5 score. Thirty-eight per cent of all participants had severe ED (International Index of Erectile Function-5 score ≤ 7). Men with ED were significantly older than subjects without ED (54.8 years vs. 43.3 years, P < 0.001) and more frequently had simultaneous non-SSc-related risk factors such as alcohol consumption. In 82% of SSc patients, the onset of ED was after the manifestation of the first non-Raynaud's symptom (median delay 4.1 years). ED was associated with severe cutaneous, muscular or renal involvement of SSc, elevated pulmonary pressures and restrictive lung disease. ED was treated in only 27.8% of men. The most common treatment was sildenafil, whose efficacy is not established in ED of SSc patients. Conclusions: Severe ED is a common and early problem in men with SSc. Physicians should address modifiable risk factors actively. More research into the pathophysiology, longitudinal development, treatment and psychosocial impact of ED is needed.

Twice-daily dosing of proton pump inhibitor (PPI), the standard therapy for gastro-oesophageal reflux disease (GERD), is an effective therapy for GERD in SSc. The aim of this study was to compare the efficacy of omeprazole in combination with domperidone vs in combination with algycon in reducing the severity and frequency of reflux symptoms of PPI partial response (PPI-PR) GERD in SSc. Adult SSc patients having PPI-PR GERD were randomly assigned to receive domperidone plus algycon placebo or algycon plus domperidone placebo in a 1:1 ratio plus omeprazole for 4 weeks. The assessment included severity of symptom grading by visual analogue scale, frequency of symptoms by frequency scale for symptoms of GERD and quality of life (QoL) by EuroQol five-dimensions questionnaire scoring. One hundred and forty-eight SSc-GERD patients were enrolled, of whom 88 had PPI-PR. Eighty cases were randomized for either domperidone (n = 38) or algycon (n = 37) therapy. The majority in both groups had the diffuse SSc subset. At the end of the study, no significant difference in symptom grading was found between groups. After treatment and compared with baseline, the severity of symptoms, frequency scale for symptoms of GERD and QoL significantly improved in both groups. Five (13.2%) and 8 (21.6%) respective cases in the domperidone and algycon groups did not respond. The prevalence of PPI-PR GERD is common. Domperidone and algycon are equally effective treatments in combination with omeprazole. However, ∼17% of patients were non-responsive, so the effectiveness of domperidone, algycon and PPI combination therapy should be further investigated. https://clinicaltrials.gov (NCT01878526).

Objective. To determine the prevalence of asymptomatic cardiac involvement and its correlation with non-cardiac manifestation in Thai SSc patients.

Proton pump inhibitor (PPI) twice daily dosing is a standard therapy for gastroesophageal reflux disease (GERD) in systemic sclerosis (SSc) but there is no data on its response rate or the predictors of PPI-partial response GERD. Aims were to determine the prevalence of PPI-partial response GERD in SSc and to define its predictors. A prospective study was conducted in SSc patients with GERD. The patients were treated with omeprazole 20 mg bid for 4 weeks. The severity of symptom-grading by visual analogue scale (VAS) and frequency of symptoms by frequency scale for symptoms of GERD (FSSG) were assessed at baseline and 4 weeks after treatment. PPI-partial response GERD was defined as less than 50% improvement in the VAS for severity of symptom as well as acid reflux score by FSSG after treatment. According to the sample size calculation, 243 SSc-GERD patients were enrolled; of whom 166 (68.3%) had the diffuse cutaneous SSc. PPI-partial response GERD was found in 131 SSc patients (prevalence 53.9%; 95%CI 47.4–60.3). The multivariate analysis revealed that esophageal dysphagia was an only predictor the PPI-partial response GERD (OR 1.82; 95%CI 1.01–3.29) while neither SSc subset nor severity of skin tightness were significantly associated with PPI-partial response GERD. Half of the SSc patients were PPI-partial response GERD. Esophageal dysphagia was the only predictor of PPI-partial response GERD in SSc patients. Screening for dysphagia before starting GERD treatment is helpful for assessment the risk of PPI refractoriness GERD in SSc patients.