Document Detail

Systemic sclerosis related pulmonary hypertension associated with interstitial lung disease: Impact of pulmonary arterial hypertension therapies.
MedLine Citation:
PMID:  21538327     Owner:  NLM     Status:  Publisher    
OBJECTIVE.: Precapillary pulmonary hypertension (PH) is an important cause of mortality in systemic sclerosis (SSc) where it can be isolated (pulmonary arterial hypertension [PAH]) or associated with interstitial lung disease (ILD). Importantly, outcomes and efficacy of PAH therapies in patients with PH complicating SSc-related ILD (PH-ILD) remain unknown. This study was undertaken to evaluate our experience with PH-ILD and the efficacy and safety of PAH therapies in this patient cohort. METHODS.: A retrospective analysis of consecutive SSc patients with PH-ILD confirmed by right heart catheterization from two large referral centers who received targeted PAH therapies was conducted. World Health Organization (WHO) functional class, 6-minute walk test (6-minWT), and hemodynamics were assessed at baseline and after 7.7 ± 6.2 months of PAH treatment. Kaplan Meier and Cox proportional hazards models were used to analyze survival and identify prognostic factors. RESULTS.: Seventy patients were included in the study. No significant changes were observed in functional class, 6-minWT or hemodynamic measurements after therapy. The 1-, 2- and 3-year survival estimates were 71%, 39% and 21%, respectively. In the multivariate model, worsening oxygenation during follow-up and reduced renal function were the only significant risk factors for mortality. CONCLUSION.: This series represents the largest to date describing the impact of PAH therapies in SSc related PH-ILD. In this cohort, PAH therapies were associated with no clear benefits. Deterioration in oxygenation was an important determinant of long-term survival. Prospective clinical trials focusing on this group of patients are warranted.
Jérôme Le Pavec; Reda E Girgis; Noah Lechtzin; Stephen C Mathai; David Launay; Laura K Hummers; Ari Zaiman; Olivier Sitbon; Gérald Simonneau; Marc Humbert; Paul M Hassoun
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-5-2
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  -     ISSN:  1529-0131     ISO Abbreviation:  -     Publication Date:  2011 May 
Date Detail:
Created Date:  2011-5-3     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370605     Medline TA:  Arthritis Rheum     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 by the American College of Rheumatology.
Divisions of Pulmonary and Critical Care Medicine, Baltimore, Johns Hopkins University, School of Medicine, Maryland, USA; Université Paris-Sud, Faculté de Médecine, Kremlin-Bicêtre; AP-HP, Centre National de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine,Béclère, Clamart; INSERM U999, Hypertension Artérielle Pulmonaire:Physiopathologie et Innovation Thérapeutique, Centre Chirurgical, Marie-Lannelongue, Le Plessis-Robinson.
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