David B. Badesch, MD; Victor F. Tapson, MD; Michael D. McGoon, MD; Bruce H. Brundage, MD; Lewis J. Rubin, MD; Fredrick M. Wigley, MD; Stuart Rich, MD; Robyn J. Barst, MD; Pamela S. Barrett, PharmD; Kenneth M. Kral, MS; Maria M. Jöbsis, BA; James E. Loyd, MD; Srinivas Murali, MD; Adaani Frost, MD; Reda Girgis, MB, BCh; Robert C. Bourge, MD; David D. Ralph, MD; C. Gregory Elliott, MD; Nicholas S. Hill, MD; David Langleben, MD; Robert J. Schilz, DO, PhD; Vallerie V. McLaughlin, MD; Ivan M. Robbins, MD; Bertron M. Groves, MD; Shelley Shapiro, MD, PhD; Thomas A. Medsger, MD; Sean P. Gaine, MB, BCh; Evelyn Horn, MD; James C. Decker, MS; Katharine Knobil, MD
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@UCHSC.edu.
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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.
Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous Intravenous Epoprostenol for Pulmonary Hypertension Due to the Scleroderma Spectrum of Disease: A Randomized, Controlled Trial. Ann Intern Med. 2000;132:425-434. doi: 10.7326/0003-4819-132-6-200003210-00002
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Published: Ann Intern Med. 2000;132(6):425-434.
Pulmonary hypertension is characterized by progressive elevation of pulmonary artery pressure and vascular resistance, often leading to right ventricular failure and death (1-3). Continuous intravenous infusion of epoprostenol improves prognosis and symptoms in patients with primary (idiopathic) pulmonary hypertension (4-8). Randomized, controlled clinical trials of epoprostenol for secondary pulmonary hypertension have not been conducted.
Pulmonary hypertension frequently complicates the scleroderma spectrum of disease, which includes diffuse scleroderma, limited scleroderma (the CREST syndrome [calcinosis cutis, the Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangectasia]), and the overlap syndrome. These multisystem diseases are characterized by connective tissue and vascular abnormalities; vascular lesions are prominent in all affected tissues (9). Pulmonary hypertension occurs in up to 33% of patients with diffuse scleroderma and 10% to 50% of those with the CREST syndrome (10, 11), in which it is one of the leading causes of death (12, 13). Pulmonary hypertension in the scleroderma spectrum of disease may be associated with interstitial pulmonary fibrosis or may consist of a direct involvement of small and medium-sized pulmonary arteries and arterioles with smooth-muscle hyperplasia, medial hypertrophy, and intimal proliferation (10, 13, 14). Principal involvement of the pulmonary vasculature is more common in the CREST syndrome, whereas patients with pulmonary hypertension and diffuse scleroderma more often have interstitial lung disease (13).
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Cardiology, Nephrology, Pulmonary/Critical Care, Rheumatology, Hypertension.
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