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American Journal of Medical Case Reports. 2016, 4(5), 170-172
DOI: 10.12691/AJMCR-4-5-7
Case Report

Pulmonary Hypertension in a Patient with Non-cirrhotic Portal Hypertension and Scleroderma Sine Scleroderma: A Case Report

Ghassan Kamel1, Joseph Espiritu1, Adrian M. Di Bisceglie2, Guilan Chen3, Reema Syed4 and Ravi Nayak1,

1Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Saint Louis University School of Medicine, St Louis, MO, United States

2Department of Internal Medicine, Saint Louis University School of Medicine, St Louis, MO, United States

3Department of Pathology, Saint Louis University School of Medicine, St Louis, MO, United States

4Division of Rheumatology, Department of Internal Medicine, Saint Louis University School of Medicine, St Louis, MO, United States

Pub. Date: May 25, 2016
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Cite this paper

Ghassan Kamel, Joseph Espiritu, Adrian M. Di Bisceglie, Guilan Chen, Reema Syed and Ravi Nayak. Pulmonary Hypertension in a Patient with Non-cirrhotic Portal Hypertension and Scleroderma Sine Scleroderma: A Case Report. American Journal of Medical Case Reports. 2016; 4(5):170-172. doi: 10.12691/AJMCR-4-5-7

Abstract

Porto-pulmonary hypertension is a known complication of liver cirrhosis but its association with non-cirrhotic portal hypertension patients is rare. We report a case of pulmonary hypertension in a patient with non-cirrhotic portal hypertension and scleroderma sine scleroderma. The two latter conditions have been shown to be independently associated with pulmonary arterial hypertension. DLCO is expected to decrease in scleroderma patients due to pulmonary vascular disease, which will result in an increased FVC/DLCO ratio. The low FVC/DLCO ratio in our patient suggests that pulmonary arterial hypertension was more likely to have been due to non-cirrhotic portal hypertension than scleroderma sine scleroderma.

Keywords

scleroderma, pulmonary hypertension, non-cirrhotic portal hypertension

Copyright

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

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