Prognostic factors for survival in scleroderma associated pulmonary arterial hypertension.
J Rheumatol
; 35(8): 1584-90, 2008 Aug.
Article
in En
| MEDLINE
| ID: mdl-18597400
ABSTRACT
OBJECTIVE:
Identification of prognostic factors for survival in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is necessary for appropriate monitoring, interventions, and timely referral for lung transplantation. Our objectives were (1) to identify factors associated with survival in SSc-PAH and (2) to evaluate the methodologic quality of prognostic studies against current standards.METHODS:
A systematic review was performed to identify studies evaluating factors associated with survival in SSc-PAH. The methodologic quality of each study was evaluated using a methodologic quality index.RESULTS:
HLA-DRw6 (RR 54.52, p = 0.01), HLA-DRw52 (RR not reported, p = 0.02), initial systolic pulmonary artery pressure (sPAP) > 60 mmHg (HR 3.60, 95% CI 1.42, 9.15), elevated mean right atrial pressure (mRAP) (HR 20.7, p = 0.0001), and shorter time between SSc onset and observed PAH (5.24 vs 9.93 yrs, p < 0.01) were associated with decreased survival. Age > 50 years (HR 2.34, 95% CI 0.54, 10.2), male sex (HR 2.02, 95% CI 0.65, 6.20), limited subtype (HR 2.37, 95% CI 0.68, 8.20), pulmonary fibrosis [Kaplan-Meier (KM) curves, p = 0.3], change in pulmonary vascular resistance (KM curves, p = 0.8), anti-centromere (HR 1.67, 95% CI 0.66, 4.26) and anti-ScL-70 (HR 0.28, 95% CI 0.03, 1.99) antibodies were not definitively associated with survival. Attributes of participants, prognostic factors, and outcome measures were well reported. Study attrition, confounding, and analysis were not well reported.CONCLUSION:
HLA-DRw52 and -DRw6, initial sPAP > 60 mmHg, mRAP, and shorter time between SSc onset and observed PAH were associated with decreased survival; however, methodologic quality of study reporting was variable. Prognostic factor research is needed using current methodologic standards.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Scleroderma, Systemic
/
Hypertension, Pulmonary
Type of study:
Prognostic_studies
/
Risk_factors_studies
/
Systematic_reviews
Limits:
Female
/
Humans
/
Male
Language:
En
Journal:
J Rheumatol
Year:
2008
Document type:
Article
Affiliation country:
Canada