Document Detail

Outcome measures in pulmonary arterial hypertension associated with systemic sclerosis.
MedLine Citation:
PMID:  18784140     Owner:  NLM     Status:  MEDLINE    
SSc is complicated in approximately 10% of the patients by pulmonary arterial hypertension (PAH), a rare dyspnoea-fatigue syndrome caused by an increase in pulmonary vascular resistance. The prognosis of SSc-PAH is particularly poor, with estimated survival rates of approximately 50% at 2 yrs without pulmonary circulation-targeted therapies. Prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors have been shown to be efficacious in PAH, with persistent long-term benefit and approximate doubling of survival rate, and these encouraging results appear transposable to the SSc-PAH subcategory. However, PAH as well as SSc-PAH remain incurable, with insufficient functional improvement in many patients. More progress is needed, and this will require more effective drugs and adapted outcome measures.
O Kowal-Bielecka; M Delcroix; A Vonk-Noordegraaf; M M Hoeper; R Naeije
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  47 Suppl 5     ISSN:  1462-0332     ISO Abbreviation:  Rheumatology (Oxford)     Publication Date:  2008 Oct 
Date Detail:
Created Date:  2008-09-11     Completed Date:  2009-05-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  England    
Other Details:
Languages:  eng     Pagination:  v39-41     Citation Subset:  AIM; IM    
Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland.
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MeSH Terms
Endpoint Determination
Exercise Test
Hypertension, Pulmonary / complications*,  drug therapy,  physiopathology
Scleroderma, Systemic / complications*,  drug therapy,  physiopathology
Treatment Outcome

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