Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

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dc.contributor.authorMittoo, Shikha
dc.contributor.authorJacob, Thomas
dc.contributor.authorCraig, Andrea
dc.contributor.authorBshouty, Zoheir
dc.date.accessioned2016-06-07T21:17:30Z
dc.date.available2016-06-07T21:17:30Z
dc.date.issued2010-1-1
dc.date.updated2016-06-07T06:55:59Z
dc.description.abstractBACKGROUND: Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.OBJECTIVE: To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.METHODS: A retrospective evaluation of 13 CTD-PH-ILD patients who were treated with bosentan, sildenafil or bosentan plus sildenafil, was conducted. Immunosuppressants were prescribed as indicated. Patients underwent pulmonary function testing and assessment of 6 min walk distance at the time of treatment initiation and during follow-up. Patients were followed until time of death, lung transplantation or the end of the study. Kaplan-Meier estimates of survival were calculated and log-rank testing was used to analyze survival differences according to CTD subtype.RESULTS: Thirteen patients (seven with systemic sclerosis [SSc], four with overlap syndrome, and two with rheumatoid arthritis) were followed for a mean (± SD) duration of 33.8±21.7 months. The survival estimate at a median duration of 34 months was 85%; two patients with SSc died. Mortality rates were greater among patients with SSc versus other CTD subtypes (P=0.04). No changes from baseline to follow-up in mean forced vital capacity or exercise capacity, and no treatment-related toxicity, were observed.CONCLUSION: Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.
dc.description.versionPeer Reviewed
dc.identifier.citationShikha Mittoo, Thomas Jacob, Andrea Craig, and Zoheir Bshouty, “Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease,” Canadian Respiratory Journal, vol. 17, no. 6, pp. 282-286, 2010. doi:10.1155/2010/686098
dc.identifier.urihttp://dx.doi.org/10.1155/2010/686098
dc.identifier.urihttp://hdl.handle.net/1993/31370
dc.language.rfc3066en
dc.rightsopen accessen_US
dc.rights.holderCopyright © 2010 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
dc.titleTreatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease
dc.typeJournal Article
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