Charan Vegivinti MD Charan Vegivinti MD
Charan Vegivinti MD
Internal Medicine Resident
Charan Vegivinti MD
Internal Medicine Resident

[DOI] [PMC free article] [PubMed] [Google Scholar] 38.Khanna D, Yan X, Tashkin DP, et al. Impact of oral cyclophosphamide on health-related quality of life in patients with active scleroderma lung disease: results from the scleroderma lung study. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease.

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[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. Mycophenolate mofetil and cyclophosphamide improve health-related quality of life in patients with systemic sclerosis who participated in SLS II.

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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. [DOI] [PubMed] [Google Scholar] 35.Volkmann ER, Tashkin DP, Kuwana M, et al. Progression of Interstitial Lung Disease in Systemic Sclerosis: the Importance of Pneumoproteins Krebs von den Lungen 6 and CCL18. December;71(12):2059–2067. 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.

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[DOI] [PMC free article] [PubMed] [Google Scholar] 45.Saunders P, Tsipouri V, Keir GJ, et al.

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Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being. Scleroderma trials study G. The health assessment questionnaire (HAQ) is strongly predictive of good outcome in early diffuse scleroderma: results from an analysis of two randomized controlled trials in early diffuse scleroderma. Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis. [DOI] [PMC free article] [PubMed] [Google Scholar] 12.Tashkin DP, Roth MD, Clements PJ, et al.

Nicht-medizinische Verwendung

Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. [DOI] [PMC free article] [PubMed] [Google Scholar]; • This landmark RCT demonstrated that MMF was not only as effective as CYC at improving lung function and radiographic fibrosis in ILD, but was safer and better tolerated than CYC. Efficacy of mycophenolate mofetil and oral cyclophosphamide on skin thickness: post hoc analyses from two randomized placebo-controlled trials. [DOI] [PMC free article] [PubMed] [Google Scholar] 14.Volkmann ER, Tashkin DP, Li N, et al. Mycophenolate mofetil versus placebo for systemic sclerosis-related interstitial lung disease: an analysis of scleroderma lung studies I and II. 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.

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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. Sep-Oct;35(Suppl 106(4)):151–158.

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Efficacy of sildenafil on sildenafil 25 mg tablets 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. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis.

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[DOI] [PMC free article] [PubMed] [Google Scholar] 36.Fischer A, Strek ME, Cottin V, et al. Proceedings of the American College of Rheumatology/Association of pfizer sildenafil 50 Physicians of Great Britain and Ireland connective tissue disease-associated interstitial lung disease summit: a multidisciplinary approach to address challenges and opportunities. February;71(2):182–195. [DOI] [PubMed] [Google Scholar] 37.Goldin JG, Kim GHJ, Tseng CH, et al. Longitudinal changes in quantitative interstitial lung disease on computed tomography after immunosuppression in the scleroderma lung study II. A multicenter, placebo-controlled, double-blind study.

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Infusion of iloprost, a prostacyclin analogue, for treatment of Raynaud’s phenomenon in systemic sclerosis.

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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. 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. 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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Papers of special note have been highlighted as either of interest (•) or of considerable interest (••) to readers. Treatment algorithms for systemic sclerosis according to experts. Emerging targets of disease-modifying therapy for systemic sclerosis. [DOI] [PMC free article] [PubMed] [Google Scholar] 4.Peoples C, Medsger TA Jr., Lucas M, et al. Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes.

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[DOI] [PMC free article] [PubMed] [Google Scholar] February;34(1):1–15;v. [DOI] [PubMed] [Google Scholar] 6.Sierra-Sepulveda A, Esquinca-Gonzalez A, Benavides-Suarez SA, et al. Systemic sclerosis pathogenesis and emerging therapies, beyond the fibroblast. [DOI] [PMC free article] [PubMed] [Google Scholar] Management of systemic sclerosis-related skin disease: a review of existing and experimental therapeutic approaches. 8.Medsger TA Jr. [DOI] [PMC free article] [PubMed] [Google Scholar] 59.Shah AA, Schiopu E, Chatterjee S, et al. The recurrence of digital ulcers in patients with systemic sclerosis after discontinuation of oral treprostinil. Vascular biomarkers and digital ulcerations in systemic sclerosis: results from a randomized controlled trial of oral treprostinil (DISTOL-1).

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[DOI] [PMC free article] [PubMed] [Google Scholar] 15.Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. [DOI] [PubMed] [Google Scholar]; • This was the first large, RCT to evaluate the safety and efficacy of HSCT in SSc and demonstrated improved long-term mortality in dsSSc patients randomized to HSCT compared with CYC. Myeloablative autologous stem-cell transplantation for severe scleroderma. [DOI] [PMC free article] [PubMed] [Google Scholar]; • This was the second large, RCT to evaluate the safety and efficacy of HSCT in SSc and also demonstrated improved long-term mortality in dsSSc patients randomized to HSCT compared with CYC.

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Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). [DOI] [PMC free article] [PubMed] [Google Scholar] 19.Khanna sildenafil citrate drug D, Spino C, Johnson S, et al. Abatacept in early diffuse cutaneous systemic sclerosis: results of a phase II investigator-initiated, multicenter, double-blind, randomized, placebo-controlled trial. [DOI] [PMC free article] [PubMed] [Google Scholar] 20.Gonzalez EG, Selvi E, Balistreri E, et al. Synthetic cannabinoid ajulemic acid exerts potent antifibrotic effects in experimental models of systemic sclerosis. [DOI] [PMC free article] [PubMed] [Google Scholar] 61.Gaillard-Bigot F, Roustit M, Blaise S, et al.

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Safety and efficacy of lenabasum (JBT-101) in diffuse cutaneous systemic sclerosis subjects treated for one year in an open-label extension of trial JBT101-SSC-001. The American college of rheumatology provisional composite response index for clinical trials in early diffuse cutaneous systemic sclerosis. [DOI] [PMC free article] [PubMed] [Google Scholar] 23.R Dn S, Bloom B, White B, et al. Baseline subject demographics and disease characteristics in a phase 3 study of safety and efficacy of lenabasum in diffuse cutaneous systemic sclerosis. An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS trial.

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Nintedanib for systemic sclerosis-associated interstitial lung disease. [DOI] [PubMed] [Google Scholar]; • This was the largest RCT ever conducted in SSc and demonstrated that treatment with nintedanib slows the rate of decline of lung function in patients with SSc-ILD compared with placebo. 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. Treprostinil iontophoresis improves digital blood flow during local cooling in systemic sclerosis. Treprostinil hydrogel iontophoresis in systemic sclerosis-related digital skin ulcers: a safety study.

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