 |
|
|
 |
Case Report
Pulmonary Veno-Occlusive Disease and Scleroderma Associated Pulmonary Hypertension
SINDHU R. JOHNSON, DEMETRIS PATSIOS, DAVID M. HWANG, and JOHN T. GRANTON ABSTRACT. Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). We describe a series of 4 patients with systemic sclerosis (SSc), concomitant PH, and biopsy-proven/presumed PVOD. We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect. (First Release Sept 15 2006; J Rheumatol 2006;33:2347-50) Key Indexing Terms:
PULMONARY VENO-OCCLUSIVE DISEASE From the Division of Rheumatology, the Departments of Pathology and Medical Imaging, and the Pulmonary Hypertension Program, Division of Respirology, University Health Network, University of Toronto, Toronto, Ontario, Canada. Dr. Johnson is the recipient of a Research Fellowship award from the Canadian Institutes of Health Research, the Scleroderma Society of Ontario, and The Arthritis Society. S.R. Johnson, MD, FRCPC, Division of Rheumatology; D. Patsios, BM, BCH, FRCR(UK), Medical Imaging Department; D.M. Hwang, MD, PhD, FRCPC, Department of Pathology; J.T. Granton, MD, FRCPC, Pulmonary Hypertension Program, Division of Respirology, University Health Network, University of Toronto. Address reprint requests to Dr. J. Granton, Pulmonary Hypertension Program, 11 NCSB-1170, Toronto General Hospital, 200 Elizabeth Street, Toronto, ON M5G 2C4, Canada. E-mail: John.Granton@uhn.on.ca Accepted for publication July 4, 2006.
|
