Document Detail


Sarcoidosis-associated pulmonary hypertension: assessment and management.
MedLine Citation:
PMID:  20665399     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms. Diverse processes may coexist in the pathogenesis of SAPH, and there is an overlap with mechanisms of pulmonary arterial hypertension (PAH). This has encouraged the study of PAH-specific therapeutic agents in the treatment of SAPH. In small series, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have been shown to improve hemodynamics, functional status, and outcomes. This article reviews the most recent data available in the epidemiology, pathophysiology, diagnosis, and treatment of SAPH.
Authors:
Veronica Palmero; Roxana Sulica
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Publication Detail:
Type:  Journal Article; Review     Date:  2010-07-27
Journal Detail:
Title:  Seminars in respiratory and critical care medicine     Volume:  31     ISSN:  1098-9048     ISO Abbreviation:  Semin Respir Crit Care Med     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-28     Completed Date:  2010-11-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9431858     Medline TA:  Semin Respir Crit Care Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  494-500     Citation Subset:  IM    
Copyright Information:
Copyright Thieme Medical Publishers.
Affiliation:
Division of Pulmonary and Critical Care Medicine, Beth Israel Medical Center, New York, NY 10003, USA.
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MeSH Terms
Descriptor/Qualifier:
Cyclic Nucleotide Phosphodiesterases, Type 5 / antagonists & inhibitors
Humans
Hypertension, Pulmonary / diagnosis,  drug therapy*,  etiology
Phosphodiesterase Inhibitors / therapeutic use
Prognosis
Prostaglandins I / therapeutic use
Pulmonary Fibrosis / diagnosis,  drug therapy,  etiology
Receptors, Endothelin / antagonists & inhibitors
Sarcoidosis / complications*,  diagnosis,  drug therapy
Chemical
Reg. No./Substance:
0/Phosphodiesterase Inhibitors; 0/Prostaglandins I; 0/Receptors, Endothelin; EC 3.1.4.35/Cyclic Nucleotide Phosphodiesterases, Type 5

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