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Hypothyroidism and Primary Pulmonary Hypertension: An Autoimmune Pathogenetic Link?

David B. Badesch, MD; Kristine M. Wynne, RN, BSN; Scott Bonvallet, MD; Norbert F. Voelkel, MD; Chester Ridgway, MD; and Bertron M. Groves, MD
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University of Colorado Health Sciences Center, Denver, Colorado. Requests for Reprints: David B. Badesch, MD, Division of Pulmonary Sciences and Critical Care Medicine, Box B-133, University of Colorado Health Sciences Center, 4200 East Ninth Avenue, Denver, CO 80262. Acknowledgments: The authors thank Rubin Tudor, MD for help with interpreting the pathologic findings and John T. Reeves, MD for help with the manuscript. Grant Support: By PHS research grant M501RR00051. Dr. Badesch is supported by a Pfizer Scholar's Award in Cardiovascular Medicine, NIH Clinical Investigator Award HL 02408-04, and the American Heart Association of Colorado. Drs. Badesch and Groves are supported by the Burroughs Wellcome Company for the prostacyclin study. Dr. Voelkel is supported by NIH HL 43180-01A3. Dr. Ridgeway is supported by NIH DK 36843, NIH CA 47411, NIH CA 46934, and NIH RR 00051.

Copyright 2004 by the American College of Physicians

Ann Intern Med. 1993;119(1):44-46. doi:10.7326/0003-4819-119-1-199307010-00008
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Primary pulmonary hypertension (PPH), an often fatal disease of unknown cause that primarily affects young women, is a diagnosis of exclusion. The relative roles of genetic predisposition, autoimmunity, viral infection, hormonal influences, and environmental and drug exposures are not known. Among seven patients enrolled by our center in a multicenter trial of continuous prostacyclin in severe PPH, four of the five women had hypothyroidism. Although a causal relationship cannot be established based on this small case series, the possibility of an autoimmune pathogenetic link between PPH and hypothyroidism should be investigated further.





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