Document Detail


Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary pressures.
MedLine Citation:
PMID:  22127844     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
OBJECTIVE: Borderline pulmonary arterial hypertension (PAH), characterised by a marked exercise-induced increase in pulmonary arterial pressure with normal resting values may precede manifest PAH in systemic sclerosis. We hypothesised that PAH treatment is safe in these patients and might attenuate hemodynamic progression. METHODS: Systemic sclerosis patients with borderline PAH underwent a baseline right heart catheterisation, a control after a 12-month observation and a second control after 6 months bosentan therapy. As primary end point the changes of mean pulmonary arterial pressure at 50 Watts during the observation vs. therapy were compared. RESULTS: Ten patients completed the study. Mean pulmonary arterial pressure at rest, 50 Watt, and maximal exercise increased significantly during the observation period (+2.5±3.0mmHg; p=0.03, +4.0±2.9mmHg; p=0.002, and +6.8±4.1mmHg; p<0.005) and tended to decrease during the treatment period (-2.5±3.9mmHg; p=0.07, -1.5±4.5mmHg; p=0.32, and -1.8±7.0mmHg; p=0.43). The changes during observation vs. therapy period were significantly different (p=0.03, p=0.01 (primary end point), and p=0.02, respectively). The changes in resting pulmonary vascular resistance were also significantly different (+8±25 vs. -45±22dyn*s*cm(-5) ; p<0.001). The changes in resting pulmonary arterial wedge pressure (+2.6±2.5mmHg (p<0.01) vs. -0.6±3.8mmHg (NS)) were not significant (p=0.12), despite the significant increase during observation period. No relevant adverse effects were reported. CONCLUSION: In systemic sclerosis patients with borderline pulmonary hemodynamics, resting and exercise pulmonary arterial pressure may significantly increase within one year of observation. Bosentan might be safe and effective to attenuate these changes. Randomised controlled trials are warranted to confirm these explorative findings of a hypothesis generating study.
Authors:
Gabor Kovacs; Robert Maier; Elisabeth Aberer; Marianne Brodmann; Winfried Graninger; Xhylsime Kqiku; Stefan Scheidl; Natascha Tröster; Christian Hesse; Lewis Rubin; Horst Olschewski
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-11-29
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  -     ISSN:  1529-0131     ISO Abbreviation:  -     Publication Date:  2011 Nov 
Date Detail:
Created Date:  2011-11-30     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.
Affiliation:
Medical University of Graz, Division of Pulmonology, Graz, Austria. gabor.kovacs@klinikum-graz.at.
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