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21 March 2000 | Volume 132 Issue 6 | Pages 425-434
Background: Pulmonary hypertension is a progressive and often fatal complication of the scleroderma spectrum of disease for which no treatment has been proven effective in a randomized trial.
Objective: To determine the effect of epoprostenol on pulmonary hypertension secondary to the scleroderma spectrum of disease.
Design: Randomized, open-label, controlled trial.
Setting: 17 pulmonary hypertension referral centers.
Patients: 111 patients with moderate to severe pulmonary hypertension.
Intervention: Epoprostenol plus conventional therapy or conventional therapy alone.
Measurements: The primary outcome measure was exercise capacity. Other measures were cardiopulmonary hemodynamics, signs and symptoms of pulmonary hypertension and scleroderma, and survival.
Results: Exercise capacity improved with epoprostenol (median distance walked in 6 minutes, 316 m at 12 weeks compared with 270 m at baseline) but decreased with conventional therapy (192 m at 12 weeks compared with 240 m at baseline). The difference between treatment groups in the median distance walked at week 12 was 108 m (95% CI, 55.2 m to 180.0 m) (P < 0.001). Hemodynamics improved at 12 weeks with epoprostenol. The changes in mean pulmonary artery pressure for the epoprostenol and conventional therapy groups were–5.0 and 0.9 mm Hg, respectively (difference, –6.0 mm Hg [CI, –9.0 to –3.0 mm Hg], and the mean changes in pulmonary vascular resistance were –4.6 and 0.9 mm Hg/L per minute, respectively (difference, –5.5 mm Hg/L per minute [CI, –7.3 to –3.7 mm Hg/L per minute]. Twenty-one patients treated with epoprostenol and no patients receiving conventional therapy showed improved New York Heart Association functional class. Borg Dyspnea Scores and Dyspnea-Fatigue Ratings improved in the epoprostenol group. Trends toward greater improvement in severity of the Raynaud phenomenon and fewer new digital ulcers were seen in the epoprostenol group. Four patients in the epoprostenol group and five in the conventional therapy group died (P value not significant). Side effects of epoprostenol therapy included jaw pain, nausea, and anorexia. Adverse events related to the epoprostenol delivery system included sepsis, cellulitis, hemorrhage, and pneumothorax (4% incidence for each condition).
Conclusions: Continuous epoprostenol therapy improves exercise capacity and cardiopulmonary hemodynamics in patients with pulmonary hypertension due to the scleroderma spectrum of disease.
Author and Article Information
From University of Colorado, Denver, Colorado; Duke University, Durham, North Carolina; Mayo Clinic, Rochester, Minnesota; University of California, Los Angeles, Los Angeles, California; University of Maryland and Johns Hopkins University, Baltimore, Maryland; Rush-Presbyterian Medical Center, Chicago, Illinois; Columbia-Presbyterian Medical Center, New York, New York; Glaxo Wellcome, Inc., Research Triangle Park, North Carolina; Vanderbilt University, Nashville, Tennessee; University of Pittsburgh, Pittsburgh, Pennsylvania; Baylor College of Medicine, Houston, Texas; Wayne State University, Detroit, Michigan; University of Alabama, Birmingham, Alabama; University of Washington, Seattle, Washington; LDS Hospital, Salt Lake City, Utah; Brown University, Providence, Rhode Island; McGill University, Montreal, Quebec, Canada; The Cleveland Clinic, Cleveland, Ohio; and Harbor-UCLA Medical Center, Torrance, California.
Presented at the 71st Scientific Sessions of the American Heart Association, 11 November 1998, Dallas, Texas, and published in abstract form (Circulation. 1998; Suppl 1:I614). The rheumatologic outcomes of the study were presented at the 62nd Annual Scientific Meeting of the American College of Rheumatology, 10 November 1998, San Diego, California, and were published in abstract form (Arthritis Rheum. 1998; 41[Suppl]:A1243).
Acknowledgments: The authors thank the members of the Data Safety Monitoring Board, study coordinators, study monitors, and pharmacists who participated in this trial.
Grant Support: By Glaxo Wellcome, Inc. Drs. Badesch, Tapson, McGoon, Brundage, and Rubin served on the Steering Committee for the Development of Epoprostenol in Secondary Pulmonary Hypertension and were paid consultants to Glaxo Wellcome, Inc. Dr. Wigley was also a paid consultant to Glaxo Wellcome, Inc.
Requests for Single Reprints: David B. Badesch, MD, University of Colorado Health Sciences Center, Division of Pulmonology, C272, 4200 East Ninth Avenue, Denver, CO 80262; e-mail, David.Badesch{at}UCHSC.edu.
Requests To Purchase Bulk Reprints (minimum, 100 copies): the Reprints Coordinator; phone, 215-351-2657; e-mail, reprints{at}mail.acponline.org.
Current Author Addresses: Dr. Badesch: Division of Pulmonology, University of Colorado Health Sciences Center, C272, 4200 East Ninth Avenue, Denver, CO 80262.
Dr. Tapson: Division of Pulmonary Medicine, Duke University Medical Center, DUMN Box 31175, Durham, NC 27710.
Dr. McGoon: Division of Cardiovascular Diseases, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.
Dr. Brundage: Bend Memorial Clinic, 1501 NE Medical Center Drive, Bend, OR 97701.
Dr. Rubin: University of California, San Diego, School of Medicine, 200 West Arbor Drive, Westwing Room 135, San Diego, CA 92103.
Dr. Wigley: Division of Rheumatology, Johns Hopkins University, 720 Rutland Avenue, Room 1059 South, Baltimore, MD 21205.
Dr. Rich: Section of Cardiology, Rush-Presbyterian-St. Luke's Medical Center, 1725 West Harrison Street, Suite 945, Chicago, IL 60612.
Dr. Barst: Babies Hospital, Columbia-Presbyterian Medical Center, Pediatric-Cardiology Center BH-2 North, Room 262, 3959 Broadway, New York, NY 10032.
Dr. Barrett, Mr. Kral, Ms. Jöbsis, Mr. Decker, and Dr. Knobil: Glaxo Wellcome, Inc., 5 Moore Drive, Research Triangle Park, NC 27709.
Drs. Loyd and Robbins: Pulmonary Medicine, Vanderbilt University Medical Center, 1161 21st Avenue South, Room T-1217 MCN, Nashville, TN 37232-2650.
Dr. Murali: Transplantation Cardiology, University of Pittsburgh Medical Center, 556 Scaife Hall, 200 Lothrop Street, Pittsburgh, PA 15213.
Dr. Frost: Pulmonary Section, Lung Transplant Program, Baylor College of Medicine, 6550 Fannin Smith Tower, Suite 1236, Houston, TX 77030.
Dr. Girgis: Division of Pulmonary and Critical Care Medicine, Wayne State University School of Medicine, Harper Hospital, 3-Hudson, 3990 John R, Detroit, MI 48201.
Dr. Bourge: Division of Cardiovascular Diseases, University of Alabama, 1900 University Boulevard, 321-M Tinsley Harrison Tower, Birmingham, AL 35294.
Dr. Ralph: Pulmonary and Critical Care Medicine, University of Washington Medical Center, Box 356522, Seattle, WA 98195-6522.
Dr. Elliott: Pulmonary Division, LDS Hospital, Ninth Avenue and C Street, Salt Lake City, UT 84143.
Dr. Hill: Pulmonary Division, Rhode Island Hospital, 593 Eddy Street, Providence, RI 02903.
Dr. Langleben: Jewish General Hospital, Room E-258, 3755 chemin Côte Ste-Catherine, Montreal, Quebec H3T 1E2, Canada.
Dr. Schilz: Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195.
Dr. McLaughlin: Section of Cardiology, Rush Presbyterian-St. Luke's Medical Center, 1725 West Harrison Street, Suite 020, Chicago, IL 60612.
Dr. Groves: Division of Cardiology, B132, University of Colorado Health Sciences Center, 4300 East Ninth Avenue, Denver, CO 80262.
Dr. Shapiro: Division of Cardiology, Harbor-UCLA Medical Center, 1000 West Carson Street, Torrance, CA 90509.
Dr. Medsger: University of Pittsburgh, 502 Kaufmann Building, 3471 Fifth Avenue, Pittsburgh, PA 15213.
Dr. Gaine: Division of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, 10 South Pine Street, Room 800, Baltimore, MD 21201.
Dr. Horn: Cardiology Division, Department of Medicine, Columbia-Presbyterian Medical Center, 630 West 168th Street, Box 93, New York, NY 10032.
Author Contributions: Conception and design: D.B. Badesch, V.F. Tapson, M.D. McGoon, B.H. Brundage, L.J. Rubin, F.M. Wigley, S. Rich, R.J. Barst, P.S. Barrett, K.M. Kral, M.M. Jöbsis, R.C. Bourge, C.G. Elliott, B.M. Groves.
Analysis and interpretation of the data: D.B. Badesch, V.F. Tapson, M.D. McGoon, B.H. Brundage, L.J. Rubin, F.M. Wigley, S. Rich, R.J. Barst, P.S. Barrett, K.M. Kral, M.M. Jöbsis, R.C. Bourge, D. Langleben, J.C. Decker, K. Knobil.
Drafting of the article: D.B. Badesch, V.F. Tapson, M.D. McGoon, B.H. Brundage, F.M. Wigley, K.M. Kral, M.M. Jöbsis, D. Langleben, T.A. Medsger, J.C. Decker, K. Knobil.
Critical revision of the article for important intellectual content: D.B. Badesch, V.F. Tapson, M.D. McGoon, B.H. Brundage, F.M. Wigley, S. Rich, R.J. Barst, P.S. Barrett, K.M. Kral, M.M. Jöbsis, J.E. Loyd, S. Murali, A.E. Frost, D. Ralph, C.G. Elliott, D. Langleben, T.A. Medsger, J.C. Decker, K. Knobil.
Final approval of the article: D.B. Badesch, V.F. Tapson, M.D. McGoon, B.H. Brundage, F.M. Wigley, S. Rich, R.J. Barst, K.M. Kral, M.M. Jöbsis, J.E. Loyd, S. Murali, A.E. Frost, R.E. Girgis, R.C. Bourge, D. Ralph, C.G. Elliott, D. Langleben, J.C. Decker, K. Knobil.
Provision of study materials or patients: D.B. Badesch, V.F. Tapson, M.D. McGoon, B.H. Brundage, L.J. Rubin, S. Rich, F.M. Wigley, R.J. Barst, J.E. Loyd, S. Murali, A.E. Frost, R.E. Girgis, R.C. Bourge, D. Ralph, C.G. Elliott, N.S. Hill, D. Langleben, R.J. Schilz, V.V. McLaughlin, I.M. Robbins, B.M. Groves, S. Shapiro, T.A. Medsger, S.P. Gaines, E. Horn.
Statistical expertise: K.M. Kral, J.C. Decker.
Obtaining of funding: D.B. Badesch, M.M. Jöbsis.
Administrative, technical, or logistic support: M.M. Jöbsis, T.A. Medsger.
Collection and assembly of data: M.D. McGoon, K.M. Kral, M.M. Jöbsis, J.E. Loyd, R.E. Girgis, C.G. Elliott, I.M. Robbins, J.C. Decker. ARTICLE
Continuous Intravenous Epoprostenol for Pulmonary Hypertension Due to the Scleroderma Spectrum of Disease
A Randomized, Controlled Trial
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