Skip to main content
Top
Published in: Modern Rheumatology 5/2011

01-10-2011 | Case Report

Significant attenuation of macrovascular involvement by bosentan in a patient with diffuse cutaneous systemic sclerosis with multiple digital ulcers and gangrene

Authors: Yohei Ichimura, Yoshihide Asano, Masaru Hatano, Zenshiro Tamaki, Tomonori Takekoshi, Asako Kogure, Manabu Tomita, Tomohiko Kawashima, Miki Miyazaki, Takashi Taniguchi, Takehiro Takahashi, Hiroshi Mitsui, Makoto Sugaya, Atsushi Yao, Koichiro Kinugawa, Shinichi Sato

Published in: Modern Rheumatology | Issue 5/2011

Login to get access

Abstract

Systemic sclerosis (SSc) is characterized by vascular injuries, and bosentan has recently been proved to be efficacious for the prevention of new digital ulcers in SSc. We herein report a case of SSc in a patient with refractory digital ulcers and gangrene treated with bosentan. Stenosis of the ulnar artery, evaluated by magnetic resonance angiography, was attenuated by the bosentan treatment, suggesting that bosentan exerts a reverse remodeling effect against the pathological organic changes of arteries in SSc.
Literature
1.
go back to reference Abraham DJ, Krieg T, Distler J, Distler O. Overview of pathogenesis of systemic sclerosis. Rheumatology (Oxford) 2009;48(Suppl 3):iii3–7. Abraham DJ, Krieg T, Distler J, Distler O. Overview of pathogenesis of systemic sclerosis. Rheumatology (Oxford) 2009;48(Suppl 3):iii3–7.
2.
go back to reference Hasegawa M, Nagai Y, Tamura A, Ishikawa O. Arteriographic evaluation of vascular changes of the extremities in patients with systemic sclerosis. Br J Dermatol. 2006;155:1159–64.PubMedCrossRef Hasegawa M, Nagai Y, Tamura A, Ishikawa O. Arteriographic evaluation of vascular changes of the extremities in patients with systemic sclerosis. Br J Dermatol. 2006;155:1159–64.PubMedCrossRef
3.
go back to reference Stafford L, Englert H, Gover J, Bertouch J. Distribution of macrovascular disease in scleroderma. Ann Rheum Dis. 1998;57:476–9.PubMedCrossRef Stafford L, Englert H, Gover J, Bertouch J. Distribution of macrovascular disease in scleroderma. Ann Rheum Dis. 1998;57:476–9.PubMedCrossRef
4.
go back to reference Youssef P, Englert H, Bertouch J. Large vessel occlusive disease associated with CREST syndrome and scleroderma. Ann Rheum Dis. 1993;52:464–6.PubMedCrossRef Youssef P, Englert H, Bertouch J. Large vessel occlusive disease associated with CREST syndrome and scleroderma. Ann Rheum Dis. 1993;52:464–6.PubMedCrossRef
5.
go back to reference Korn JH, Mayes M, Matucci Cerinic M, Rainisio M, Pope J, Hachulla E, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004;50:3985–93.PubMedCrossRef Korn JH, Mayes M, Matucci Cerinic M, Rainisio M, Pope J, Hachulla E, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004;50:3985–93.PubMedCrossRef
6.
go back to reference Kowal-Bielecka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68:620–8.PubMedCrossRef Kowal-Bielecka O, Landewe R, Avouac J, Chwiesko S, Miniati I, Czirjak L, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68:620–8.PubMedCrossRef
7.
go back to reference Norton WL, Nardo JM. Vascular disease in progressive systemic sclerosis (scleroderma). Ann Intern Med. 1970;73:317–24.PubMed Norton WL, Nardo JM. Vascular disease in progressive systemic sclerosis (scleroderma). Ann Intern Med. 1970;73:317–24.PubMed
8.
go back to reference Campbell PM, LeRoy EC. Pathogenesis of systemic sclerosis: a vascular hypothesis. Semin Arthritis Rheum. 1975;4:351–68.PubMedCrossRef Campbell PM, LeRoy EC. Pathogenesis of systemic sclerosis: a vascular hypothesis. Semin Arthritis Rheum. 1975;4:351–68.PubMedCrossRef
9.
go back to reference D’Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969;46:428–40.PubMedCrossRef D’Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med. 1969;46:428–40.PubMedCrossRef
10.
go back to reference 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(Suppl 3):44–8.PubMedCrossRef 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(Suppl 3):44–8.PubMedCrossRef
11.
go back to reference 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(Suppl 3):49–53.PubMedCrossRef 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(Suppl 3):49–53.PubMedCrossRef
12.
go back to reference Denton CP, Pope JE, Peter HH, Gabrielli A, Boonstra A, van den Hoogen FH, et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis. 2008;67:1222–8.PubMedCrossRef Denton CP, Pope JE, Peter HH, Gabrielli A, Boonstra A, van den Hoogen FH, et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis. 2008;67:1222–8.PubMedCrossRef
13.
go back to reference McLaughlin VV. Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest. 2006;36(Suppl 3):10–5.PubMedCrossRef McLaughlin VV. Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest. 2006;36(Suppl 3):10–5.PubMedCrossRef
14.
go back to reference Cambrey AD, Harrison NK, Dawes KE, Southcott AM, Black CM, du Bois RM, et al. Increased levels of endothelin-1 in bronchoalveolar lavage fluid from patients with systemic sclerosis contribute to fibroblast mitogenic activity in vitro. Am J Respir Cell Mol Biol. 1994;11:439–45.PubMed Cambrey AD, Harrison NK, Dawes KE, Southcott AM, Black CM, du Bois RM, et al. Increased levels of endothelin-1 in bronchoalveolar lavage fluid from patients with systemic sclerosis contribute to fibroblast mitogenic activity in vitro. Am J Respir Cell Mol Biol. 1994;11:439–45.PubMed
15.
go back to reference Yang Z, Krasnici N, Luscher TF. Endothelin-1 potentiates human smooth muscle cell growth to PDGF: effects of ETA and ETB receptor blockade. Circulation. 1999;100:5–8.PubMed Yang Z, Krasnici N, Luscher TF. Endothelin-1 potentiates human smooth muscle cell growth to PDGF: effects of ETA and ETB receptor blockade. Circulation. 1999;100:5–8.PubMed
16.
go back to reference Shi-Wen X, Chen Y, Denton CP, Eastwood M, Renzoni EA, Bou-Gharios G, et al. Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts. Mol Biol Cell. 2004;15:2707–19.PubMedCrossRef Shi-Wen X, Chen Y, Denton CP, Eastwood M, Renzoni EA, Bou-Gharios G, et al. Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts. Mol Biol Cell. 2004;15:2707–19.PubMedCrossRef
17.
go back to reference Iannone F, Riccardi MT, Guiducci S, Bizzoca R, Cinelli M, Matucci-Cerinic M, et al. Bosentan regulates the expression of adhesion molecules on circulating T cells and serum soluble adhesion molecules in systemic sclerosis-associated pulmonary arterial hypertension. Ann Rheum Dis. 2008;67:1121–6.PubMedCrossRef Iannone F, Riccardi MT, Guiducci S, Bizzoca R, Cinelli M, Matucci-Cerinic M, et al. Bosentan regulates the expression of adhesion molecules on circulating T cells and serum soluble adhesion molecules in systemic sclerosis-associated pulmonary arterial hypertension. Ann Rheum Dis. 2008;67:1121–6.PubMedCrossRef
18.
go back to reference Abraham D. Role of endothelin in lung fibrosis. Eur Respir Rev. 2008;17:145–50.CrossRef Abraham D. Role of endothelin in lung fibrosis. Eur Respir Rev. 2008;17:145–50.CrossRef
19.
go back to reference Vancheeswaran R, Magoulas T, Efrat G, Wheeler-Jones C, Olsen I, Penny R, et al. Circulating endothelin-1 levels in systemic sclerosis subsets––a marker of fibrosis or vascular dysfunction? J Rheumatol. 1994;21:1838–44.PubMed Vancheeswaran R, Magoulas T, Efrat G, Wheeler-Jones C, Olsen I, Penny R, et al. Circulating endothelin-1 levels in systemic sclerosis subsets––a marker of fibrosis or vascular dysfunction? J Rheumatol. 1994;21:1838–44.PubMed
20.
go back to reference Yamane K, Miyauchi T, Suzuki N, Yuhara T, Akama T, Suzuki H, et al. Significance of plasma endothelin-1 levels in patients with systemic sclerosis. J Rheumatol. 1992;19:1566–71.PubMed Yamane K, Miyauchi T, Suzuki N, Yuhara T, Akama T, Suzuki H, et al. Significance of plasma endothelin-1 levels in patients with systemic sclerosis. J Rheumatol. 1992;19:1566–71.PubMed
Metadata
Title
Significant attenuation of macrovascular involvement by bosentan in a patient with diffuse cutaneous systemic sclerosis with multiple digital ulcers and gangrene
Authors
Yohei Ichimura
Yoshihide Asano
Masaru Hatano
Zenshiro Tamaki
Tomonori Takekoshi
Asako Kogure
Manabu Tomita
Tomohiko Kawashima
Miki Miyazaki
Takashi Taniguchi
Takehiro Takahashi
Hiroshi Mitsui
Makoto Sugaya
Atsushi Yao
Koichiro Kinugawa
Shinichi Sato
Publication date
01-10-2011
Publisher
Springer Japan
Published in
Modern Rheumatology / Issue 5/2011
Print ISSN: 1439-7595
Electronic ISSN: 1439-7609
DOI
https://doi.org/10.1007/s10165-011-0436-5

Other articles of this Issue 5/2011

Modern Rheumatology 5/2011 Go to the issue
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine