Document Detail

Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis.
MedLine Citation:
PMID:  19487219     Owner:  NLM     Status:  MEDLINE    
Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of CTDs. In patients with SSc, PAH has a dramatic impact on prognosis and survival and is the single most common cause of disease-related death.Yearly echocardiographic screening for PAH is recommended in patients with SSc. If suspected, confirmation of PAH diagnosis by right heart catheterization is necessary. Treatment goals for patients with PAH associated with SSc (PAH-SSc) aim to slow disease progression and improve quality of life. Some measures used to gauge the effect of treatment in patients with PAH-SSc remain to be fully validated; the 6-min walk distance, for example, is a simple and reproducible means of assessing exercise capacity, but there exists a need to understand what constitutes a clinically relevant change in this specific patient population. Currently, pharmacological intervention in PAH-SSc may target one or more of three pathophysiological pathways in PAH. The prostacyclin analogue epoprostenol has been shown to improve exercise capacity and haemodynamics in PAH-SSc patients and similar data are available from smaller studies on trepostinil and iloprost. The dual endothelin receptor antagonist bosentan has been shown to improve exercise capacity and haemodynamics in PAH-SSc, and similar data have been obtained in small numbers of patients treated with the endothelin receptor A antagonists sitaxsentan and ambrisentan. Impaired production of nitric oxide may be addressed by inhibiting phosphodiesterase type-5 with sildenafil or possibly tadalafil. Combinations of multiple targeted therapies may be beneficial to this patient population.
V McLaughlin; M Humbert; G Coghlan; P Nash; V Steen
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  48 Suppl 3     ISSN:  1462-0332     ISO Abbreviation:  Rheumatology (Oxford)     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2009-06-02     Completed Date:  2009-07-06     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  England    
Other Details:
Languages:  eng     Pagination:  iii25-31     Citation Subset:  AIM; IM    
Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA.
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MeSH Terms
Antihypertensive Agents / therapeutic use
Cardiac Catheterization
Epoprostenol / therapeutic use
Exercise Tolerance
Hypertension, Pulmonary / diagnosis,  drug therapy,  etiology*
Piperazines / therapeutic use
Purines / therapeutic use
Scleroderma, Systemic / complications*,  drug therapy,  physiopathology
Sulfonamides / therapeutic use
Sulfones / therapeutic use
Reg. No./Substance:
0/Antihypertensive Agents; 0/Piperazines; 0/Purines; 0/Sulfonamides; 0/Sulfones; 35121-78-9/Epoprostenol; 3M7OB98Y7H/sildenafil; Q326023R30/bosentan

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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