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Refractory pulmonary hypertension in a lupus patient with occult pulmonary vasculitis.
MedLine Citation:
PMID:  17041468     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Primary pulmonary hypertension is a disease that has become increasingly recognized in lupus patients. Pathologic findings from lupus patients usually do not differ from those who have idiopathic pulmonary hypertension. In recent years, intravenous vasodilator therapy has improved morbidity and mortality in patients with primary pulmonary hypertension. In this case report, we describe a young woman with severe pulmonary hypertension refractory to aggressive parenteral vasodilator treatment. Steroid treatment was initiated after a tentative diagnosis of lupus was made on the basis of the presence of Raynaud's phenomenon, proteinuria, pericarditis with tamponade, and a positive anti-Ro antibody. Despite treatment with vasodilators and steroids, the patient's condition rapidly deteriorated and she died. Unexpectedly, active pulmonary arteritis was demonstrated at autopsy. This case suggests that immunosuppressive therapy should be considered in lupus patients with severe pulmonary hypertension who are refractory to aggressive vasodilator therapy and high-dose parenteral steroids.
Authors:
Kathleen Srock; Leslie Dubin Kerr; Michael Poon; John T Fallon
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases     Volume:  9     ISSN:  1076-1608     ISO Abbreviation:  J Clin Rheumatol     Publication Date:  2003 Aug 
Date Detail:
Created Date:  2006-10-16     Completed Date:  2007-07-05     Revised Date:  2008-04-10    
Medline Journal Info:
Nlm Unique ID:  9518034     Medline TA:  J Clin Rheumatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  263-6     Citation Subset:  -    
Affiliation:
Departments of Medicine, Mount Sinai School of Medicine, New York, New York, USA.
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