Chest
Clinical InvestigationsPULMONARY HYPERTENSIONAge and Risk of Pulmonary Arterial Hypertension in Scleroderma*
Section snippets
Study Sample and Measurements
A total of 899 patients with scleroderma attended The Johns Hopkins and University of Maryland Scleroderma Center over a 4.5-year period between January 1997 and June 2001 (Fig 1). Within this cohort, 849 patients (94.4%) satisfied the American College of Rheumatology (ACR) criteria for scleroderma,30 or had at least three features of the CREST syndrome; 50 patients (5.6%) had systemic sclerosis sine scleroderma. The diagnosis of scleroderma was established by two of the authors (Dr. Wigley or
Results
Table 1 summarizes the demographic, clinical, and serologic features of the study population: 592 women (83.5%), 130 African Americans (18.3%), and 354 former or current smokers (49.9%). In terms of scleroderma subtype, 405 patient had limited (57.1%), 271 patients had diffuse (38.2%), and 33 patients had systemic sclerosis sine scleroderma (4.7%). ACA was identified in 130 patients (19.0%), whereas 89 patients had antitopoisomerase I antibody (13.0%). Mean age at disease onset was 41.8 ± 14.6
Discussion
In this large scleroderma cohort, we identified increasing age at disease onset as a risk factor for PAH. There was an approximately 50% increase in risk of PAH for every 10 years of age at onset of scleroderma after adjustment for demographic and clinical parameters. The oldest patients (≥ 60 years) had substantially higher risk of PAH, twice as high as those with earlier-onset disease. Furthermore, the association between increasing age and PAH remained evident when the outcome was restricted
ACKNOWLEDGMENT
We thank Christy Boling, Arash Banuwal, and Maria Fiesta for managing The Johns Hopkins and University of Maryland Scleroderma Center Database; and Gwen Leatherman, RN, and Jane Hofherr, RN, for their dedication to our Scleroderma Center and patients.
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This study was supported by a Maryland Chapter Arthritis Foundation Institutional Grant, the Scleroderma Research Foundation, and the Jack Alfano, Joachim & Nancy Bechtle and Don & Nancy Powell Research Funds.
Dr. Schachna was supported by an Arthritis Foundation Postdoctoral Fellowship Award.
Dr. Gelber was supported by an Arthritis Foundation Arthritis Investigator Award and Maryland Chapter Grant.