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
American Journal of Medical Case Reports.
2016,
Vol. 4 No. 5, 170-172
DOI: 10.12691/ajmcr-4-5-7
Copyright © 2016 Science and Education PublishingCite this paper: Ghassan Kamel, Joseph Espiritu, Adrian M. Di Bisceglie, Guilan Chen, Reema Syed, 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.
Correspondence to: Ravi Nayak, Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Saint Louis University School of Medicine, St Louis, MO, United States. Email:
nayakrp@slu.eduAbstract
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.
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