Document Detail

Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma.
MedLine Citation:
PMID:  16465657     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To determine risk factors for developing pulmonary hypertension (PH) in patients with scleroderma (SSc, systemic sclerosis). METHODS: We used a cohort of 1136 SSc patients using severe PH as the primary outcome in a natural history study. RESULTS: Among 361 individuals with no initial echocardiographic PH, 92 (26.0%) developed mild-moderate PH and 48 (13.6%) severe PH. Patients developing severe PH had lower initial DLCO (48.8% of predicted) than those who did not develop PH (56.8% of predicted). Patients with mild-moderate PH had a 17% probability of progressing to severe PH, and 15.6% probability of regressing to no PH. Individuals with limited disease, mild-moderate PH, and age > or= 47 years at diagnosis had a 27.3% probability of developing severe PH, compared to 8.5% in individuals with diffuse disease, no evidence of PH, and age < 47 years at diagnosis. Longitudinal regression models estimated that individuals with limited disease, mild-moderate PH, and DLCO < 50% predicted had an age-adjusted odds ratio of 8.6 of developing severe PH within 2 years compared to individuals without these risk factors. CONCLUSION: Development of severe PH is uncommon in certain subgroups of SSc patients. Risk factors for progression of PH include older age, limited skin disease, and elevated pulmonary artery pressures at the time of initial evaluation.
Betty Chang; Lionel Schachna; Barbara White; Fredrick M Wigley; Robert A Wise
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of rheumatology     Volume:  33     ISSN:  0315-162X     ISO Abbreviation:  J. Rheumatol.     Publication Date:  2006 Feb 
Date Detail:
Created Date:  2006-02-08     Completed Date:  2006-05-04     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7501984     Medline TA:  J Rheumatol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  269-74     Citation Subset:  IM    
Division of Pulmonary and Critical Care Medicine, University of New Mexico, Albuquerque, 87131, USA.
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MeSH Terms
Cohort Studies
Disease Progression
Hospitals, University
Hypertension, Pulmonary / epidemiology,  etiology*,  physiopathology
Maryland / epidemiology
Middle Aged
Respiratory Function Tests
Risk Factors
Scleroderma, Systemic / complications*,  epidemiology,  physiopathology
Comment In:
J Rheumatol. 2006 Feb;33(2):204-6   [PMID:  16465647 ]

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

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