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Prospective, open-label, uncontrolled pilot study to study safety and efficacy of sildenafil in systemic sclerosis–related pulmonary artery hypertension and cutaneous vascular complications

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An Erratum to this article was published on 25 September 2012

Abstract

Pulmonary artery hypertension (PAH) remains the leading cause of morbidity and mortality in systemic sclerosis, while Raynaud’s phenomenon and digital ulcers significantly add to the morbidity in systemic sclerosis (SSc). This study was undertaken to evaluate the role of sildenafil in PAH, Raynaud’s phenomenon, and digital ulcers in systemic sclerosis patients. A prospective, open-label, uncontrolled pilot study was done at a tertiary care centre in India to study the safety and efficacy of oral sildenafil in PAH, Raynaud’s phenomenon, digital infarcts, and ulcers in SSc. Seventeen patients fulfilling ACR classification criteria for scleroderma and having PAH were recruited. Six-minute walk test, WHO class of dyspnoea, severity of Raynaud’s phenomenon, and 2D ECHO were performed in all the study subjects at baseline and at 3 months post-treatment. All patients were treated with oral sildenafil 25 mg three times a day for a period of 3 months. The pre- and post-treatment values of mean pulmonary artery pressure (PAP), 6-min walk test, WHO class of dyspnoea, and severity of Raynaud’s phenomenon were compared to look for any significant change. Sixteen patients who completed 3-month follow-up had shown statistically significant improvement in 6-min walk test, WHO class of dyspnoea, severity of Raynaud’s phenomenon, and mPAP. Also, there was no occurrence of new digital infarcts or ulcers, and existing ulcers showed signs of healing. Sildenafil is highly efficacious cheaper and safe alternative to other available therapies for SSc-associated PAH, Raynaud’s phenomenon, and digital infarcts/ulcers.

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References

  1. Denton CP, Black CM, Abraham DJ (2009) Mechanism and consequence of fibrosis in systemic sclerosis. Nat Clin Pract Rheumatol 2:134–144

    Article  Google Scholar 

  2. Trad S, Amoura Z, Beigelman C et al (2006) Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis independent of interstitial lung disease. Arthritis Rheum 54(1):184–191

    Article  PubMed  Google Scholar 

  3. Magliano M, Isenberg DA, Hillson J (2002) Pulmonary hypertension in autoimmune rheumatic diseases: where are we now? Arthritis Rheum 46(8):1997–2009

    Article  PubMed  CAS  Google Scholar 

  4. Kawut SM, Taichman DB, Archer-Chicko CL (2003) Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 123:344–350

    Article  PubMed  Google Scholar 

  5. Watanabe H, Ohashi K, Takeuchi K et al (2002) Sildenafil for primary and secondary pulmonary hypertension. Clin Pharmacol Ther 71(5):398–402

    Article  PubMed  Google Scholar 

  6. Galiè N, Ghofrani HA, Torbicki A et al (2005) Sildenafil use in pulmonary arterial hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353(20):2148–2157

    Article  PubMed  Google Scholar 

  7. Wilkins MR, Paul GA, Strange JW et al (2005) Sildenafil versus endothelin receptor antagonist for pulmonary hypertension (SERAPH) study. Am J Respir Crit Care Med 171(11):1292–1297

    Article  PubMed  Google Scholar 

  8. Lewis GD, Shah R, Shahzad K et al (2007) Sildenafil improves exercise capacity and quality of life in patients with systolic heart failure and secondary pulmonary hypertension. Circulation 116(14):1555–1562

    Article  PubMed  CAS  Google Scholar 

  9. Badesch DB, Hill NS, Burgess G, SUPER Study Group et al (2007) Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 34(12):2417–2422

    PubMed  Google Scholar 

  10. Xu XQ, Jing ZC, Zhang JH et al (2009) The efficacy and safety of sildenafil in Chinese patients with pulmonary arterial hypertension. Hypertens Res 32(10):911–915

    Article  PubMed  CAS  Google Scholar 

  11. Samarzija M, ZuljeviÄ E, JakopoviÄ M et al (2009) One year efficacy and safety of oral sildenafil treatment in severe pulmonary hypertension. Coll Antropol 33(3):799–803

    PubMed  CAS  Google Scholar 

  12. Levien TL (2006) Phosphodiesterase inhibitors in Raynaud’s phenomenon. Ann Pharmacother 40(7–8):1388–1393

    Article  PubMed  CAS  Google Scholar 

  13. Gemne G (1997) Diagnostics of hand-arm system disorders in workers who use vibrating tools. Occup Environ Med 54(2):90–95

    Article  PubMed  CAS  Google Scholar 

  14. Sastry BK, Narasimhan C, Reddy NK et al (2002) Study of clinical efficacy of sildenafil in patients with primary pulmonary hypertension. Indian Heart J 54:410–414

    PubMed  CAS  Google Scholar 

  15. Singh TP, Rohit M, Grover A (2006) A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J 151(4):851–855

    Article  PubMed  Google Scholar 

  16. Garg N, Sharma MK, Sinha N (2007) Role of oral sildenafil in severe pulmonary arterial hypertension: clinical efficacy and dose response relationship. Int J Cardiol 120(3):306–313

    Article  PubMed  Google Scholar 

  17. Kuang TG, Wang J, Zhai ZG (2007) Efficacy and safety of sildenafil therapy in pulmonary artery hypertension: a meta-analysis. Zhonghua Yi Xue Za Zhi 87(15):1021–1024

    PubMed  Google Scholar 

  18. Miyamoto S, Nagaya N, Satoh T et al (2000) Clinical correlates and prognostic significance of six minutes’ walk test in patients with primary pulmonary hypertension. Am J Respir Crit Care Med 161:487–492

    PubMed  CAS  Google Scholar 

  19. Fries R, Shariat K, von Wilmowsky H et al (2005) Sildenafil in the treatment of Raynaud's phenomenon resistant to vasodilatory therapy. Circulation 112:2980–2985

    PubMed  CAS  Google Scholar 

  20. Friedrichson E, Rehberger P, Fuhrmann JT et al (2008) Fast and efficient healing of scleroderma-associated acral ulcers with sildenafil. Hautarzt 59(3):230–232

    Article  PubMed  CAS  Google Scholar 

  21. Jackson G, Benjamin N, Jackson N et al (1999) Effect of sildenafil citrate on human hemodynamics. Am J Cardiol 83(5A):13C–20C

    Article  PubMed  CAS  Google Scholar 

  22. Watanabe H, Ohashi K, Takeuchi K et al (2002) Sildenafil for primary and secondary pulmonary hypertension. Clin Pharmacol Ther 71:398–402

    Article  PubMed  Google Scholar 

  23. Rosenkranz S, Diet F, Karasch T et al (2003) Sildenafil improved pulmonary hypertension and peripheral blood flow in a patient with scleroderma-associated lung fibrosis and the raynaud phenomenon. Ann Intern Med 134(10):871–872

    Google Scholar 

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Ethics approval was taken from Institute Human Ethics Committee.

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Authors and Affiliations

  1. Clinical Immunology and Rheumatology Division, Department of Medicine, All India Institute of Medical Sciences, New Delhi, 110029, India

    Uma Kumar, Sankalp S. Gokhle, Satbir Kaur & Durgaprasanna Misra

  2. Department of Biostatistics, All India Institute of Medical Sciences, New Delhi, 110029, India

    V. Sreenivas

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  1. Uma Kumar
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  2. Sankalp S. Gokhle
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  3. V. Sreenivas
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  4. Satbir Kaur
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Correspondence to Uma Kumar.

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Kumar, U., Gokhle, S.S., Sreenivas, V. et al. 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 33, 1047–1052 (2013). https://doi.org/10.1007/s00296-012-2466-5

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  • DOI: https://doi.org/10.1007/s00296-012-2466-5

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