{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T04:06:38Z","timestamp":1772165198277,"version":"3.50.1"},"reference-count":22,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,8,1]],"date-time":"2017-08-01T00:00:00Z","timestamp":1501545600000},"content-version":"vor","delay-in-days":31,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2017,7,1]],"date-time":"2017-07-01T00:00:00Z","timestamp":1498867200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/100004326","name":"Bayer","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004326","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:p>Patients with pulmonary arterial hypertension associated with connective tissue disease (PAH\u2010PAH\u2010CTD) such as systemic sclerosis (SSc) have a poorer response to treatment and increased mortality compared with patients with idiopathic PAH. Current treatment options for PAH\u2010CTD include prostanoids, phosphodiesterase type\u20105\u00a0inhibitors (PDE\u20105i), endothelin receptor antagonists, and the soluble guanylate cyclase stimulator riociguat. In this case series, we describe three patients with PAH\u2010CTD related to limited scleroderma who were switched from a PDE\u20105i to riociguat due to insufficient clinical response. The switch to riociguat was associated with an improvement in respiratory and hemodynamic parameters and a favorable tolerability profile. These cases demonstrate that switching to riociguat is a therapeutic option in patients with PAH\u2010CTD who have not achieved a satisfactory clinical response to a PDE\u20105i.<\/jats:p>","DOI":"10.1177\/2045893217721694","type":"journal-article","created":{"date-parts":[[2017,7,17]],"date-time":"2017-07-17T10:11:59Z","timestamp":1500286319000},"page":"741-746","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":13,"title":["Replacing a phosphodiesterase\u20105\u00a0inhibitor with riociguat in patients with connective tissue disease\u2010associated pulmonary arterial hypertension: a case series"],"prefix":"10.1002","volume":"7","author":[{"given":"Amresh","family":"Raina","sequence":"first","affiliation":[{"name":"Cardiovascular Institute Allegheny General Hospital Allegheny Health Network Pittsburgh PA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raymond 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