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Bosentan in Pulmonary Arterial Hypertension Secondary to Scleroderma
AMIT JOGLEKAR, FAUSAN S. TSAI, DEBORAH A. McCLOSKEY, JULIANNE E. WILSON, JAMES R. SEIBOLD, and DAVID J. RILEY
ABSTRACT. Methods. We retrospectively reviewed 23 SSc-PAH patients with PAH at baseline [PA systolic pressure (PASP) ≥ 45 mm Hg by echocardiogram or mean PA pressure > 25 mm Hg at rest by cardiac catheterization], World Health Organization (WHO) functional classes II–IV, and with data available for 18 months. Bosentan dose was 62.5 mg twice daily for 1 month then 125 mg twice daily. Outcomes were WHO functional class, PASP, and pulmonary function tests (PFT) at 3-month intervals for 18 months. Results. WHO class at baseline 3.1 ± 0.1 (mean ± SE); 3 months, 2.5 ± 0.2*; 6 months, 2.4 ± 0.2*; 9 months, 2.5 ± 0.2* (*p < 0.02 vs baseline, n = 21 to 23), indicating clinical improvement at 9 months. After 9 months, results were not significant versus baseline. Reduction in WHO class by at least one rank was 57% at 3 months; none worsened. After 9 months, WHO class tended to worsen compared to baseline. Baseline PASP was 54 ± 2 mm Hg (n = 23) and did not change significantly with therapy. Restriction (total lung capacity 76% ± 4% of predicted) and reduced diffusing capacity (39% ± 3% of predicted) were unchanged during therapy. Abnormal transaminases in 2 patients (9%) necessitated discontinuing drug in both. Conclusion. Bosentan is clinically beneficial in patients with SSc-PAH including patients with restrictive lung disease, but pulmonary hemodynamics and PFT results remained stable during treatment. (J Rheumatol 2006;33:61-8) Key Indexing Terms:
PULMONARY HYPERTENSION
From the Division of Pulmonary and Critical Care Medicine and the University of Medicine and Dentistry of New Jersey (UMDNJ) Scleroderma Program, UMDNJ–Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA. Supported by the F.M. Kirby Foundation, Scleroderma Research Foundation (New Jersey), Foundation of UMDNJ–Scleroderma Research Fund, and Barbara Cornwall Wallace Respiratory Research Fund. A. Joglekar, MD, Fellow; D.J. Riley, MD, Professor, Pulmonary and Critical Care Division; F.S. Tsai, BS, Medical Student; D.A. McCloskey, BSN, Nurse Care Coordinator; J.E. Wilson, BSN, Staff Nurse, Scleroderma Program; J.R. Seibold, MD, Professor of Medicine, Director, Scleroderma Program (currently, Director, University of Michigan Scleroderma Program, Ann Arbor, Michigan). Address reprint requests to Dr. D.J. Riley, Department of Medicine, UMDNJ-Robert Wood Johnson Medical School, One Robert Wood Johnson Place, New Brunswick, NJ 08903-0019. E-mail: riley@umdnj.edu Accepted for publication August 30, 2005. |
