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Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach.

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Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. New insights into the recognition, classification and management of systemic sclerosis-associated pulmonary hypertension. Association between initial oral therapy and outcomes in systemic sclerosis-related pulmonary arterial hypertension. March;68(3):740–748.

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Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Infusion of iloprost, a prostacyclin analogue, for treatment of sildenafil pfizer 100g Raynaud’s phenomenon in systemic sclerosis. Iloprost treatment in patients with Raynaud’s phenomenon secondary to systemic sclerosis and the quality of life: a new therapeutic protocol. Open label study of escalating doses of oral treprostinil diethanolamine in patients with systemic sclerosis and digital ischemia: pharmacokinetics and correlation with digital perfusion.

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3. Conclusion

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A randomized double blind placebo controlled trial to compare the efficacy of initial combination therapy vs monotherapy for pulmonary arterial hypertension in systemic sclerosis. Criteria for diagnosis of exercise pulmonary hypertension. Exercise pulmonary sildenafil gel haemo-dynamics predict outcome in patients with systemic sclerosis.

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Abnormal pulmonary artery systolic pressure response after exercise in systemic sclerosis patients: A PRISMA-compliant meta-analysis.

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Effects of 1-year sildenafil 50mg tablet treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. [DOI] [PMC free article] [PubMed] [Google Scholar] 40.Volkmann ER, Tashkin DP, Sim M, et al. Cyclophosphamide for systemic sclerosis-related interstitial lung disease: a comparison of scleroderma lung study I and II. [DOI] [PMC free article] [PubMed] [Google Scholar] 41.Volkmann ER, Tashkin D. Roth M, LeClair H, et al.

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Mycophenolate mofetil and cyclophosphamide improve health-related quality of life in patients with systemic sclerosis who participated in SLS II. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Efficacy and safety of tocilizumab for the treatment of systemic sclerosis: results from a phase 3 randomized controlled trial. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. [DOI] [PMC free article] [PubMed] [Google Scholar] 45.Saunders P, Tsipouri V, Keir GJ, et al. Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.

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Rituximab versus cyclophosphamide for the treatment of connective tissue disease-associated interstitial lung disease (RECITAL): study protocol for a randomised controlled trial. [DOI] [PMC free article] [PubMed] [Google Scholar] 47.Nihtyanova SI, Brough GM, Black CM, et al. Clinical burden of digital vasculopathy in limited and diffuse cutaneous systemic sclerosis. On-demand sildenafil as a treatment for Raynaud phenomenon: a series of n-of-1 trials. Evaluation of the effect of sildenafil on the microvascular blood flow in patients with systemic sclerosis: a randomised, double-blind, placebo-controlled study.

Areas covered:

Sep-Oct;35(Suppl 106(4)):151–158. Efficacy of sildenafil on ischaemic digital ulcer healing in systemic sclerosis: the placebo-controlled SEDUCE study. Randomized placebo-controlled crossover trial of tadalafil in Raynaud’s phenomenon secondary to systemic sclerosis. Efficacy of tadalafil in secondary Raynaud’s phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Intravenous iloprost treatment of Raynaud’s phenomenon and ischemic ulcers secondary to systemic sclerosis. Adenosine deaminase increases release of excitatory amino acids through a mechanism independent of adenosine depletion. Probiotics for the treatment of systemic sclerosis-associated gastrointestinal bloating/distention.

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Intravenous cyclophosphamide vs rituximab for the treatment of early diffuse scleroderma lung disease: open label, randomized, controlled trial. A multicenter, open-label, comparative study of B-cell depletion therapy with Rituximab for systemic sclerosis-associated interstitial lung disease. Rituximab in diffuse cutaneous systemic sclerosis: an open-label clinical and histopatho-logical study. Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group. Combination therapy with rituximab and mycophenolate mofetil in systemic sclerosis.

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Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group. Severe restrictive lung disease in systemic sclerosis. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. October;69(10):1809–1815. Effect of probiotics on gastrointestinal symptoms and immune parameters in systemic sclerosis: a randomized placebo-controlled trial. Effectiveness of saccharomyces boulardii and metronidazole for small intestinal bacterial overgrowth in systemic sclerosis. The 5-HT1A receptor agonist buspirone improves esophageal motor function and symptoms in systemic sclerosis: a 4-week, open-label trial.

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Treprostinil hydrogel iontophoresis in systemic sclerosis-related digital skin ulcers: a safety study. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. [DOI] [PMC free article] [PubMed] [Google Scholar] 64.Khanna D, Denton CP, Merkel PA, et al. Effect of macitentan on the development of new ischemic digital ulcers in patients with systemic sclerosis: DUAL-1 and DUAL-2 randomized clinical trials. A systematic review and meta-analysis of the effects of topical nitrates in the treatment of primary and secondary Raynaud’s phenomenon. [DOI] [PMC free article] [PubMed] [Google Scholar] 89.Bharadwaj S, Tandon P, Gohel T, et al. Gastrointestinal manifestations, malnutrition, and role of enteral and parenteral nutrition in patients with scleroderma. Screening and therapy for malnutrition and related gastro-intestinal disorders in systemic sclerosis: recommendations of a North American expert panel.

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