{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T19:13:01Z","timestamp":1774120381032,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"s3","license":[{"start":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T00:00:00Z","timestamp":1154476800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2006,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background\u2002<\/jats:bold> The oral dual endothelin (ET) antagonist bosentan has been established as a cornerstone in the treatment of pulmonary arterial hypertension (PAH). ET is believed to be a key pathogenic mediator in systemic sclerosis (scleroderma, SSc), causing fibrotic, hypertrophic and inflammatory processes. PAH is one of the resulting deleterious effects in approximately 15% of SSc patients.<\/jats:p><jats:p><jats:bold>Material and methods\u2002<\/jats:bold> This was an open\u2010label prospective observational study of 8 patients aged 34\u221273\u00a0years with symptomatic, severe PAH related to SSc (WHO class III or IV) and mostly, lung fibrosis. PAH diagnosis was ascertained with echocardiography or right heart catheterization. Patients were treated on top of diuretics and anticoagulants with bosentan 62\u00b75\u00a0mg twice daily for 4\u00a0weeks followed by a maintenance dose of 125\u00a0mg twice daily.<\/jats:p><jats:p><jats:bold>Results\u2002<\/jats:bold> The mean 6\u2010minute walk distance (data available in 7 patients) increased from 71\u00b79 (\u00b1\u00a054\u00b77) m at baseline to 191\u00b79 (\u00b1\u00a0104\u00b76) m after 3\u00a0months (<jats:italic>P\u00a0=<\/jats:italic>\u00a00\u00b7012) and to 202\u00b76 (\u00b1\u00a0108\u00b71) m after 6\u00a0months (<jats:italic>P\u00a0=<\/jats:italic>\u00a00\u00b7011), respectively. Six of 8 patients improved in the 6\u2010minute walk test, and these 6 patients also improved in World Health Organization functional class. Two patients did not sufficiently respond to bosentan therapy; one of them died. The tolerability of bosentan was good, and there were no discontinuations. Elevations of hepatic aminotransferases above 3 times the upper limit of normal were not recorded.<\/jats:p><jats:p><jats:bold>Conclusion\u2002<\/jats:bold> Bosentan treatment was well tolerated in this cohort of SSc patients with interstitial lung disease and was effective for treatment of severe PAH in the majority of patients.<\/jats:p>","DOI":"10.1111\/j.1365-2362.2006.01695.x","type":"journal-article","created":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T23:14:53Z","timestamp":1154560493000},"page":"44-48","source":"Crossref","is-referenced-by-count":26,"title":["Bosentan for severe pulmonary arterial hypertension related to systemic sclerosis with interstitial lung disease"],"prefix":"10.1111","volume":"36","author":[{"given":"K.","family":"Ahmadi\u2010Simab","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Hellmich","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W. 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