| The pulmonary arterial hypertension quality enhancement research initiative: comparison of patients with idiopathic PAH to patients with systemic sclerosis-associated PAH. | |
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MedLine Citation:
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PMID: 21998119 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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OBJECTIVE: The objective of this report is to compare baseline, management and survival characteristics in idiopathic pulmonary arterial hypertension (IPAH) with systemic sclerosis-associated pulmonary arterial hypertension (SSc-APAH) using data from the prospectively enrolled PAH Quality Enhancement Research Initiative.METHODS: Between August 2005 and July 2007, patients with IPAH and SSc-APAH were enrolled across 60 US sites and followed up for 3 years. Data on diagnostic tests, clinical variables, pulmonary arterial hypertension (PAH) medication and outcomes were recorded.RESULTS: With some exceptions, baseline clinical and laboratory characteristics were similar between the 279 patients with IPAH and the 228 with SSc-APAH. Patients with SSc-APAH were older at the time of PAH diagnosis, were more likely to be female and were antinuclear antibody positive. Patients with SSc-APAH had poorer spirometric results. During the 3-year follow-up, both groups were managed with prostacyclin and prostacyclin analogue treatment, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors (PDE5i) singly or in combination. At 3 years, patients with SSc-APAH were more likely to be treated with PDE5i alone or with an endothelin receptor antagonist. Patients with SSc-APAH had a significantly lower survival rate compared to patients with IPAH (60% vs 77%, p<0.0001).CONCLUSIONS: The cohort with SSc-APAH was older, was more severely ill, was more likely to be female, was managed with PDE5i and had reduced 3-year survival compared with the cohort with IPAH. |
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Authors:
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Philip J Clements; Mary Tan; Vallerie V McLaughlin; Ronald J Oudiz; Victor F Tapson; Richard N Channick; Lewis J Rubin; Anatoly Langer; |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-10-13 |
Journal Detail:
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Title: Annals of the rheumatic diseases Volume: - ISSN: 1468-2060 ISO Abbreviation: - Publication Date: 2011 Oct |
Date Detail:
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Created Date: 2011-10-14 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0372355 Medline TA: Ann Rheum Dis Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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1Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California, USA. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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