The effects of oral treatment for systemic sclerosis related pulmonary arterial hypertension: A systematic review and meta-analysis

References

• Geyer M, Muller-Ladner U. The pathogenesis of systemic sclerosis revisited. Clinic Rev Allerg Immunol. 2011;40(2):92–103.
   PubMed Web of Science ®Google Scholar
• Nikpour M, Stevens WM, Herrick AL, Proudman SM. Epidemiology of systemic sclerosis. Best Pract Res Clin Rheumatol. 2010;24(6):857–69.
   PubMed Web of Science ®Google Scholar
• Hudson M, Thombs BD, Steele R, Panopalis P, Newton E, Baron M, et al. Quality of life in patients with systemic sclerosis compared to the general population and patients with other chronic conditions. J Rheumatol. 2009;36(4):768–72.
   PubMed Web of Science ®Google Scholar
• Mok CC, Kwok CL, Ho LY, Chan PT, Yip SF. Life expectancy, standardized mortality ratios, and causes of death in six rheumatic diseases in Hong Kong, China. Arthritis Rheum. 2011;63(5):1182–9.
   PubMed Web of Science ®Google Scholar
• Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005;52(12):3792–800.
   PubMed Web of Science ®Google Scholar
• Mukerjee D, St George D, Coleiro B, Knight C, Denton C, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62(11):1088–93.
   PubMed Web of Science ®Google Scholar
• Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000;132(6):425–34.
   PubMed Web of Science ®Google Scholar
• Barnes H, Yeoh HL, Fothergill T, Burns A, Humbert M, Williams T. Prostacyclin for pulmonary arterial hypertension. Cochrane Database Syst Rev. 2019;5:CD012785.
   PubMedGoogle Scholar
• Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353:2148–57.
   PubMed Web of Science ®Google Scholar
• Liu C, Chen J, Gao Y, Deng B, Liu K. Endothelin receptor antagonists for pulmonary arterial hypertension. Cochrane Database Syst Rev. 2013;3:CD004434.
   Google Scholar
• Ahmadi-Simab K, Hellmich B, Gross WL. Bosentan for severe pulmonary arterial hypertension related to systemic sclerosis with interstitial lung disease. Eur J Clin Invest. 2006;36(s3):44–8.
   PubMedGoogle Scholar
• Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet. 2001;358(9288):1119–23.
   PubMed Web of Science ®Google Scholar
• Denton CP, Humbert M, Rubin L, Black CM. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis. 2006;65(10):1336–40.
   PubMed Web of Science ®Google Scholar
• Seibold JR, Denton CP, Furst DE, Guillevin L, Rubin LJ, Wells A, et al. Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis. Arthritis Rheum. 2010;62(7):2101–8.
   PubMed Web of Science ®Google Scholar
• Rubin LJ, Badesch DB, Barst RJ, Galiè N, Black CM, Keogh A, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002;346(12):896–903.
   PubMed Web of Science ®Google Scholar
• Coghlan JG, Galiè N, Barberà JA, Frost AE, Ghofrani H-A, Hoeper MM, et al. Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial. Ann Rheum Dis. 2017;76(7):1219–27.
   PubMed Web of Science ®Google Scholar
• Kumar U, Sankalp G, Sreenivas V, Kaur S, Misra D. Prospective, open-label, uncontrolled pilot study to study safety and efficacy of sildenafil in systemic sclerosis-related pulmonary artery hypertension and cutaneous vascular complications. Rheumatol Int. 2013;33(4):1047–52.
   PubMed Web of Science ®Google Scholar
• Oudiz RJ, Schilz RJ, Barst RJ, Galié N, Rich S, Rubin LJ, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest. 2004;126(2):420–7.
   PubMed Web of Science ®Google Scholar
• Shirai Y, Yasuoka H, Takeuchi T, Satoh T, Kuwana M. Intravenous epoprostenol treatment of patients with connective tissue disease and pulmonary arterial hypertension at a single center. Mod Rheumatol. 2013;23(6):1211–20.
   PubMed Web of Science ®Google Scholar
• Zeng X, Zhang Y, Kwong JSW, Zhang C, Li S, Sun F, et al. The methodological quality assessment tools for preclinical and clinical studies, systematic review and meta-analysis, and clinical practice guideline: a systematic review. J Evid Based Med. 2015;8(1):2–10.
   PubMedGoogle Scholar
• Moher D, Liberati A, Tetzlaff J, Altman DG, PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Ann Intern Med. 2009;151(4):264–9.
   PubMed Web of Science ®Google Scholar
• Cozzi F, Montisci R, Marotta H, Bobbo F, Durigon N, Ruscazio M, et al. Bosentan therapy of pulmonary arterial hypertension in connective tissue diseases. Eur J Clin Invest. 2006;36(s3):49–53.
   PubMedGoogle Scholar
• Girgis RE, Mathai SC, Krishnan JA, Wigley FM, Hassoun PM. Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. J Heart Lung Transplant. 2005;24(10):1626–31.
   PubMed Web of Science ®Google Scholar
• Kovacs G, Maier R, Aberer E, Brodmann M, Graninger W, Kqiku X, et al. Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure. Arthritis Rheum. 2012;64(4):1257–62.
   PubMed Web of Science ®Google Scholar
• Launay D, Sitbon O, Le Pavec J, Savale L, Tcherakian C, Yaici A, et al. Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil. Rheumatology. 2010;49(3):490–500.
   PubMed Web of Science ®Google Scholar
• Murdaca G, Lantieri F, Puppo F, Bezante GP, Balbi M. Beneficial effects of long-term treatment with bosentan on the development of pulmonary arterial hypertension in patients with systemic sclerosis. J Int Med Res. 2016;44(1_suppl):85–9.
   PubMedGoogle Scholar
• Romaniello A, Gigante A, Di Paolo M, Tallerini G, Barbano B, Amoroso D, et al. Bosentan for digital ulcers prevention does not worsen cardiopulmonary exercise test parameters in SSc patients with interstitial lung disease. Int J Cardiol. 2016;223:113–5.
   PubMed Web of Science ®Google Scholar
• Saggar R, Khanna D, Shapiro S, Furst DE, Maranian P, Clements P, et al. Brief report: effect of ambrisentan treatment on exercise-induced pulmonary hypertension in systemic sclerosis: a prospective single-center, open-label pilot study. Arthritis Rheum. 2012;64(12):4072–7.
   PubMedGoogle Scholar
• Caravita S, Wu SC, Secchi MB, Dadone V, Bencini C, Pierini S, et al. Long-term effects of intermittent Iloprost infusion on pulmonary arterial pressure in connective tissue disease. Eur J Intern Med. 2011;22(5):518–21.
   PubMed Web of Science ®Google Scholar
• de la Mata J, Gomez-Sanchez MA, Aranzana M, Gomez-Reino JJ. Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases. Arthritis Rheum. 1994;37(10):1528–33.
   PubMedGoogle Scholar
• Klings ES, Hill NS, Ieong MH, Simms RW, Korn JH, Farber HW, et al. Systemic sclerosis-associated pulmonary hypertension: short- and long-term effects of epoprostenol (prostacyclin). Arthritis Rheum. 1999;42(12):2638–45.
   PubMedGoogle Scholar
• Sun Y-J, Xiong C-M, Shan G-L, Gu Q, Zeng W-J, Lu X-L, et al. Inhaled low-dose iloprost for pulmonary hypertension: a prospective, multicenter, open-label study. Clin Cardiol. 2012;35(6):365–70.
   PubMed Web of Science ®Google Scholar
• Hassoun PM, Zamanian RT, Damico R, Lechtzin N, Khair R, Kolb TM, et al. Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2015;192(9):1102–10.
   PubMed Web of Science ®Google Scholar
• Mercurio V, Mukherjee M, Tedford RJ, Zamanian RT, Khair RM, Sato T, et al. Improvement in right ventricular strain with ambrisentan and tadalafil upfront therapy in scleroderma-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2018;197(3):388–91.
   PubMed Web of Science ®Google Scholar
• Sato T, Ambale-Venkatesh B, Lima JAC, Zimmerman SL, Tedford RJ, Fujii T, et al. The impact of ambrisentan and tadalafil upfront combination therapy on cardiac function in scleroderma associated pulmonary arterial hypertension patients: cardiac magnetic resonance feature tracking study. Pulm Circ. 2018;8(1):204589321774830.
   Web of Science ®Google Scholar
• Lammi MR, Mathai SC, Saketkoo LA, Domsic RT, Bojanowski C, Furst DE, et al. Association between initial oral therapy and outcomes in systemic sclerosis-related pulmonary arterial hypertension. Arthritis Rheumatol. 2016;68(3):740–8.
   PubMed Web of Science ®Google Scholar
• Mathai SC, Girgis RE, Fisher MR, Champion HC, Housten-Harris T, Zaiman A, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007;29(3):469–75.
   PubMed Web of Science ®Google Scholar
• Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart. 2006;92(7):926–32.
   PubMed Web of Science ®Google Scholar
• Sanchez O, Sitbon O, Jais X, Simonneau G, Humbert M. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest. 2006;130(1):182–9.
   PubMed Web of Science ®Google Scholar
• Page MJ, Moher D. Evaluations of the uptake and impact of the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) Statement and extensions: a scoping review. Syst Rev. 2017;6(1):263.
   PubMedGoogle Scholar
• MacGregor AJ, Canavan R, Knight C, Denton CP. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology. 2001;40(4):453–9.
   PubMed Web of Science ®Google Scholar
• Distler O, Behrens F, Huscher D, Foeldvari I, Zink A, Nash P, et al. Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis. Rheumatology. 2006;45(12):1455–7.
   PubMed Web of Science ®Google Scholar
• Avouac J, Wipff J, Kahan A, Allanore Y. Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta-analysis of randomised controlled trials. Ann Rheum Dis. 2008;67(6):808–14.
   PubMed Web of Science ®Google Scholar
• Kedzierski RM, Yanagisawa M. Endothelin system: the double-edged sword in health and disease. Annu Rev Pharmacol Toxicol. 2001;41(1):851–76.
   PubMedGoogle Scholar
• Kahan A, Allanore Y. Primary myocardial involvement in systemic sclerosis. Rheumatology. 2006;45(suppl_4):iv14–7.
   PubMedGoogle Scholar
• Villalba WO, Sampaio-Barros PD, Pereira MC, Cerqueira EMFP, Leme CA, Marques-Neto JF, et al. Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Chest. 2007;131(1):217–22.
   PubMed Web of Science ®Google Scholar
• Avouac J, Guerini H, Wipff J, Assous N, Chevrot A, Kahan A, et al. Radiological hand involvement in systemic sclerosis. Ann Rheum Dis. 2006;65(8):1088–92.
   PubMed Web of Science ®Google Scholar
• Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004;351(14):1425–36.
   PubMed Web of Science ®Google Scholar
• Johnson SR, Brode SK, Mielniczuk LM, Granton JT. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med. 2012;106(5):730–9.
   PubMed Web of Science ®Google Scholar
• Jacobs W, Boonstra A, Marcus JT, Postmus PE, Vonk-Noordegraaf A. Addition of prostanoids in pulmonary hypertension deteriorating on oral therapy. J Heart Lung Transplant. 2009;28(3):280–4.
   PubMed Web of Science ®Google Scholar
• Bhatia S, Frantz RP, Severson CJ, Durst LA, McGoon MD. Immediate and long-term hemodynamic and clinical effects of sildenafil in patients with pulmonary arterial hypertension receiving vasodilator therapy. Mayo Clin Proc. 2003;78(10):1207–13.
   PubMed Web of Science ®Google Scholar
• Hoeper MM, Faulenbach C, Golpon H. Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension. Eur Respir J. 2004;24(6):1007–10.
   PubMed Web of Science ®Google Scholar
• Paul GA, Gibbs JS, Boobis AR, Abbas A, Wilkins MR. Bosentan decreases the plasma concentration of sildenafil when coprescribed in pulmonary hypertension. Br J Clin Pharmacol. 2005;60(1):107–12.
   PubMed Web of Science ®Google Scholar