Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2006-9-18
pubmed:abstractText
Survival rates in pulmonary arterial hypertension (PAH) associated with rheumatic diseases, in particular connective tissue diseases such as systemic sclerosis, are even lower than in idiopathic PAH. These low survival rates highlight the need for early diagnosis and treatment in these patients. Transthoracic Doppler-echocardiography is most often used for diagnostic screening of patients at risk. Other screening tests are serum pro-brain-natriuretic peptide (pro-BNP) and diffusion capacity for carbon monoxide (DLCO), which appear to be changed early in the course of the PAH associated with connective tissue diseases. The diagnosis needs to be confirmed by right heart catheterization, which is recommended in all patients with suspected PAH. Besides the conventional background therapy, a number of specific therapies have been evaluated in randomized controlled trials in the recent years. These therapies include prostacyclins and prostacyclin analogues, endothelin-receptor antagonists and phosphodiesterase-5 inhibitors. Response to treatment can be measured by exercise capacity (e.g. 6 min walk distance) and pro-BNP, although certain aspects of validation for these outcome measures are lacking in PAH associated with connective tissue diseases.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
1462-0332
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
45 Suppl 4
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
iv22-5
pubmed:meshHeading
pubmed:year
2006
pubmed:articleTitle
Pulmonary arterial hypertension and rheumatic diseases--from diagnosis to treatment.
pubmed:affiliation
Department of Rheumatology, University Hospital Zurich, Gloriastrasse 25, 8091 Zurich, Switzerland. Oliver.Distler@usz.ch
pubmed:publicationType
Journal Article, Review