| Clinical characteristics and survival in systemic sclerosis-related PH associated With ILD. | |
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MedLine Citation:
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PMID: 21474572 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Abstract: BACKGROUND: Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) (PH-ILD) is usually associated with a poor prognosis. However, data are either lacking or scarce on prognosis factors in this condition. The objectives of the study were to compare the survival of patients with PH-ILD or pulmonary arterial hypertension (PAH) and to determine whether the severity of PH has prognostic value in SSc-associated PH-ILD. METHODS: Consecutive patients with SSc and PH-ILD (n = 47) or PAH (n = 50) confirmed by right heart catheterization were included in a cross-sectional analysis. PH was classified as mild (mPAP ≤ 35 mm Hg) or moderate to severe (mPAP > 35 mm Hg). RESULTS: As compared with patients with PAH, subjects with PH-ILD were younger, were more frequently males with an history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies and a lower FVC/DLCO ratio. They had a worse prognosis than patients with PAH (3-year survival of 47% vs 71%, respectively, P = .07). Patients with mild PH-ILD had similar poor outcomes when compared with those with moderate to severe PH-ILD. Pericardial effusion (hazard ratio (HR): 2.44, P = .04) and lower DLCO (HR: 0.96, P = .01) were the only independent factors predictive of a poor survival in the PH-ILD group. CONCLUSIONS: SSc patients with PH-ILD had a different phenotype and a worse prognosis than those with SSc and PAH. Lower DLCO and presence of pericardial effusion were predictive of a poor outcome in PH-ILD, whereas mPAP seemed to have no prognostic significance. |
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Authors:
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David Launay; Marc Humbert; Alice Berezne; Vincent Cottin; Yannick Allanore; Louis-Jean Couderc; Olivier Bletry; Azzedine Yaici; Pierre-Yves Hatron; Luc Mouthon; Jérôme Le Pavec; Pierre Clerson; Eric Hachulla |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-4-7 |
Journal Detail:
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Title: Chest Volume: - ISSN: 1931-3543 ISO Abbreviation: - Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-4-8 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0231335 Medline TA: Chest Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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1Université Lille 2; Service de Médecine Interne. Centre de référence de la sclérodermie. Hôpital Claude-Huriez. CHRU Lille, Lille, France. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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