Klin Farmakol Farm. 2012;26(4):190-193

Bosentan in treating vascular complications of systemic sclerosis

David Suchý1, Pavel Jeremiáš1, Milan Hromádka2
1 Oddělení klinické farmakologie FN v Plzni
2 Kardiologické oddělení FN v Plzni

Systemic sclerosis (SSc) is an autoimmune disease of unknown etiology characterized by vasculopathy and fibrosis. Digital ischemia

presented as Raynaud’s phenomenon (RP) or digital ulcers (DUs) is the hallmark of SSc-related vasculopathy. DUs are a frequent complication

and are associated with significant morbidity. This paper briefly summarizes the current approach to the treatment of DUs and,

in addition, presents a case report of a young female patient suffering from secondary RP with DUs, treated with bosentan.

Keywords: systemic sclerosis, Raynaud’s phenomenon, digital ulcers, treatment, bosentan

Published: December 1, 2012  Show citation

ACS AIP APA ASA Harvard Chicago Chicago Notes IEEE ISO690 MLA NLM Turabian Vancouver
Suchý D, Jeremiáš P, Hromádka M. Bosentan in treating vascular complications of systemic sclerosis. Klin Farmakol Farm. 2012;26(4):190-193.
Download citation

References

  1. Bečvář R. Současný pohled na diagnostiku a léčbu systémové sklerodermie a Sjögrenova syndromu. Med. praxi 2008; 5(3): 109-112.
  2. Bečvář R. Terapie systémové sklerodermie. In: Pavelka K, a spol. (Ed.), Farmakoterapie revmatických onemocnění. 1. vydání, Praha: Galén, 2005: 257-269.
  3. Bečvář R. Raynaudův fenomén u systémových chorob pojiva. Postgraduální Medicína.
  4. Steen V, Denton CP, Pope JE, Mattucci-Cerinic M. Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology. 2009; 48: 19-24. Go to original source... Go to PubMed...
  5. Schiopu E, Impens AJ, Phillips K. Digital ischemia in scleroderma spectrum of disease Ann Rheum Dis. 2012; 71(7): 134-1142.
  6. Tiev KP, Diot E, Clerson P, et al. Clinical features of scleroderma patients with or without prior or current ischemic digital ulcers: post-hoc analysis of a nationwide multicenter cohort (ItinerAIR-Sclerodermie) The Journal of Rheumatology 2009; 36: 1470-1476. Go to original source... Go to PubMed...
  7. Hachulla E, Clerson P, Launay D, et al. Natural history of ischemic digital ulcers in systemic sclerosis: single-center retrospective longitudinal study. J Rheumatol.2007; 34(12): 2423-2430. Go to PubMed...
  8. Flavahan NA, Flavahan S, Mitra S, et al. The vasculopathy of Raynauds phenomenon and scleroderma. Rheum Dis Clin North Am, 2003; 29: 275-291. Go to original source... Go to PubMed...
  9. Rodnan GP, Myerowitz RL, Justh GO. Morphologic changes in the digital arteries of patients with progressive systemic sclerosis (scleroderma) and Raynaud's phenomenon. Medicine 1980; 59: 393-408. Go to original source... Go to PubMed...
  10. Choi JJ, Min DJ, ChoML, et al. Elevated vascular endothelial growth factor in systemic sclerosis. J Rheumatol 2003; 30: 1529-1533. Go to PubMed...
  11. Biondi ML, Marasini B, Bassani C, Agostoni A. Increased plasma endothelin levels in patients with Raynaud'sphenomenon. The New England Journal of Medicine 1991; 324: 1139-1140. Go to original source... Go to PubMed...
  12. Sunderkotter C, Herrgott I. Comparison of patients with and without digital ulcers in systemic sclerosis: detection of possible risk factors. British Journal of Dermatology. 2009; 160(4): 835-843. Go to original source... Go to PubMed...
  13. Sebastiani A, Manfredi M, Colaci R, Damico V. Malagoli, D. Giuggioli, and C. Ferri. Capillaroscopic skin ulcer risk index: a new prognostic tool for digital skin ulcer development in systemic sclerosis patients, " Arthritis Care and Research.2009; 61(5): 688-694. Go to original source... Go to PubMed...
  14. Kowal-Bielecka O, Landewe R, Avouac J. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR) Ann Rheum Dis 2009; 68: 620-628. Go to original source... Go to PubMed...
  15. Gliddon AE, Dor´e CJ, Black CM, et al. Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, doubleblind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril. Arthritis Rheum. 2007; 56(11): 3837-3846. Go to original source... Go to PubMed...
  16. Dziadzio M, Denton CP, Smith R, et al. Losartan therapy for Raynaud's phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallelgroup, controlled trial, Arthritis Rheum. 1999; 42(12): 2646-2655. Go to original source...
  17. Thompson AE. Shea B, Welch V, Fenlon D, Pope JE. Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis. Arthritis Rheum.2001; 44: 1841-1847. Go to original source...
  18. Rademaker M, Cooke DE, Almond NE, et al. Comparisonof intravenous infusions of iloprost and oral nifedipine intreatment of Raynaud's phenomenon in patients with systemic sclerosis: a double blind randomised study. British Medical Journal.1989; 298: 561-564. Go to original source... Go to PubMed...
  19. Wigley FM, Wise RA, Seibold JR, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multi-center, placebo-controlled, double-blind study. Ann Intern Med 1994; 120: 199-206. Go to original source... Go to PubMed...
  20. Fries R, Shariat K, vonWilmowsky H, Bohm M. Sildenafilin the treatment of Raynaud's phenomenon resistant to vasodilatory therapy. Circulation. 2005; 112: 2980-2985. Go to original source... Go to PubMed...
  21. Brueckner CS, Becker MO, Kroencke T, et al. Effect of sildenafil on digital ulcers in systemic sclerosis: analysis from a single centre pilot study. Ann Rheum Dis 2010; 69: 1475-1478. Go to original source... Go to PubMed...
  22. Korn JH, Mayes M, Matucci-Cerinic M, et al. Digital ulcers in systemic sclerosis - prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004; 50: 3985-3993. Go to original source... Go to PubMed...
  23. Black C, et al. Bosentan reduces the number of new digital ulcers in patients with systemic sclerosis: open lable extension to a double-blind trial. Annals of Rheumatic Diseases 2006; 65(Suppl 2): 384.
  24. Matuci Cerrinic, M, Denton CP, Furst DE, et al.Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double blind, placebo controlled trial. Ann Rheum Dis. 2011; 70: 32-38. Go to original source... Go to PubMed...
  25. Kuwana M, Kaburaki J, Okazaki Y, et al. Increase in circulating endothelial precursors by atorvastatin in patients with systemic sclerosis. Arthritis Rheumatism 2006; 54: 1946-1951. Go to original source... Go to PubMed...
  26. Abou-Raya A, Abou-Raya S, Helmii M. Statins: potentially useful in therapy of systemic sclerosis-related Raynaud's phenomenon and digital ulcers J Rheumatol. 2008; 35: 1801-1808. Go to PubMed...
  27. Sambo P, Amico D, Giacomelli R, et al. Intravenous N-acetylcysteine for treatment of Raynaud's phenomenon secondary to systemic sclerosis: a pilot study. The Journal of Rheumatology. 2001; 28(10): 2257-2262. Go to PubMed...
  28. Rosato E, Borghese F, Pisarri S, Salsano F. The treatment with N-acetylcysteine of Raynaud's phenomenon and ischemic ulcers therapy in sclerodermic patients: a prospective observational study of 50 patients. Clinical Rheumatolog, 2009; 28(12): 1379-1384. Go to original source... Go to PubMed...
  29. Skácelová S, Bečvář R, Štork J. Účinnost bosentanu v prevenci vzniku nových digitálních ulcerací u nemocných se systémovou sklerodermií (kazuistika). Čes. Revmatol; 20(2): 74-81.
  30. Humbert M, Cabane J. Successful treatment of systemic sclerosis digital ulcers and pulmonary arterial hypertension with endothelin receptor antagonist bosentan. Rheumatology 2003; 42: 191-193. Go to original source... Go to PubMed...
  31. García de la Pe?a-Lefebvre P, Rodríguez Rubio S, Valero Expósito M, et al. Long-term experience of bosentan for treating ulcers and healed ulcers in systemic sclerosis patients. Rheumatology (Oxford) 2008; 47: 464-466. Go to original source... Go to PubMed...




Clinical Pharmacology and Pharmacy

Madam, Sir,
please be aware that the website on which you intend to enter, not the general public because it contains technical information about medicines, including advertisements relating to medicinal products. This information and communication professionals are solely under §2 of the Act n.40/1995 Coll. Is active persons authorized to prescribe or supply (hereinafter expert).
Take note that if you are not an expert, you run the risk of danger to their health or the health of other persons, if you the obtained information improperly understood or interpreted, and especially advertising which may be part of this site, or whether you used it for self-diagnosis or medical treatment, whether in relation to each other in person or in relation to others.

I declare:

  1. that I have met the above instruction
  2. I'm an expert within the meaning of the Act n.40/1995 Coll. the regulation of advertising, as amended, and I am aware of the risks that would be a person other than the expert input to these sites exhibited


No

Yes

If your statement is not true, please be aware
that brings the risk of danger to their health or the health of others.