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Prognostic Factors for Survival in Scleroderma Associated Pulmonary Arterial Hypertension
SINDHU R. JOHNSON, JOHN R. SWINTON, and JOHN T. GRANTON
ABSTRACT. 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. (First Release July 1 2008; J Rheumatol 2008;35:1584–90) Key Indexing Terms:
PROGNOSIS From the Divisions of Rheumatology and Respirology and the Pulmonary Hypertension Programme, University Health Network, University of Toronto, Toronto, ON, Canada. Dr. Sindhu Johnson is supported by the Abbott Scholar Award for Rheumatology Research and Canadian Arthritis Network Fellowship. S.R. Johnson, MD, FRCP, Division of Rheumatology and Pulmonary Hypertension Programme; J.R. Swinton, MD, FRCP, Division of Respirology and Pulmonary Hypertension Programme; J.T. Granton, MD, FRCP, Division of Rheumatology and Pulmonary Hypertension Programme, University Health Network and Pulmonary Hypertension Programme, University of Toronto. Address reprint requests to Dr. J. Granton, Pulmonary Hypertension Programme, 11 NCSB-1170, Toronto General Hospital, 200 Elizabeth Street, Toronto, ON, Canada, M5G 2C4. E-mail: John.Granton@uhn.on.ca Accepted for publication March 26, 2008. |
