Document Detail

Pulmonary hypertension in sarcoidosis: A review.
MedLine Citation:
PMID:  20920145     Owner:  NLM     Status:  In-Data-Review    
Pulmonary hypertension (PH) is a well-recognized complication of sarcoidosis. Patients with sarcoidosis-associated PH (SAPH) have poorer functional status and greater supplemental oxygen requirements than sarcoidosis patients without PH, and are more likely to be listed for lung transplantation. PH is an independent risk factor for mortality in sarcoidosis patients awaiting lung transplantation. The pathophysiology of SAPH is complex, with multiple mechanisms contributing to pathogenesis, including the fibrous destruction of the pulmonary vascular bed, extrinsic compression of the central pulmonary vessels and an intrinsic vasculopathy. Recognition of SAPH may be delayed as it can be masked by the clinical picture of underlying pulmonary sarcoidosis, and right heart catheter remains the gold-standard for diagnosis. Management of SAPH is based on reversal of resting hypoxaemia, treatment of comorbidities and treatment of the underlying sarcoidosis. The use of corticosteroids in SAPH is controversial. Specific PH therapy is not routinely recommended in SAPH as there are no successful placebo-controlled trials, although there is limited data to suggest that endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be useful.
Tamera J Corte; Athol U Wells; Andrew G Nicholson; David M Hansell; Stephen J Wort
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Respirology (Carlton, Vic.)     Volume:  16     ISSN:  1440-1843     ISO Abbreviation:  Respirology     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-04-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9616368     Medline TA:  Respirology     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  69-77     Citation Subset:  IM    
Copyright Information:
© 2010 The Authors. Respirology © 2010 Asian Pacific Society of Respirology.
Royal Brompton Hospital and National Heart and Lung Institute Imperial College, London, UK.
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