American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: http://www.sciepub.com/journal/ajmcr Editor-in-chief: Samy, I. McFarlane
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American Journal of Medical Case Reports. 2016, 4(5), 170-172
DOI: 10.12691/ajmcr-4-5-7
Open AccessCase 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

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

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/

References:

[1]  De BK, Pal A, Santra A, Das TK, Biswas P, Agarwal PK, Mazumder DN. Primary pulmonary hypertension in non-cirrhotic portal fibrosis. Indian J Gastroenterol. 1997 Jul;16(3):85-7.
 
[2]  Savale L, O'Callaghan DS, Magnier R, Le Pavec J, Hervé P, Jaïs X, Seferian A, Humbert M, Simonneau G, Sitbon O. Current management approaches to portopulmonary hypertension. Int J Clin Pract Suppl. 2011 Jan;(169):11-8.
 
[3]  Scourfield A, Waters L, Holmes P, Panos G, Randell P, Jackson A, Mandalia S, Gazzard B, Nelson M. Non-cirrhotic portal hypertension in HIV-infected individuals. Int J STD AIDS. 2011 Jun;22(6):324-8.
 
[4]  Babbs C, Warnes TW, Haboubi NY. Non-cirrhotic portal hypertension with hypoxaemia. Gut. 1988 Jan;29(1):129-31.
 
[5]  Sweeney EC. Non-cirrhotic portal hypertension in Felty's syndrome. Ir J Med Sci. 1975 Apr;144(4):172-4.
 
[6]  Leung VK, Loke TK, Luk IS, Ng WL, Chau TN, Law ST, Chan JC. Nodular regenerative hyperplasia of the liver associated with systemic lupus erythematosus: three cases. Hong Kong Med J. 2009 Apr;15(2):139-42.
 
[7]  Sharma P, Kumar A, Mehta V, Sharma BC, Sarin SK. Systemic and pulmonary hemodynamics in patients with non-cirrhotic portal fibrosis (NCPF) is similar to compensated cirrhosis. Hepatol Int. 2007 Mar;1(1):275-80.
 
[8]  Steen VD. The lung in systemic sclerosis. J Clin Rheumatol. 2005 Feb;11(1):40-6.
 
[9]  Toya S, Tzelepis G. The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications. Rheumatol Int (2009) 29:861-868.
 
[10]  Park YW, Woo H, Jeong YY, Lee JH, Park JJ, Lee SS. Association of nodular regenerative hyperplasia of the liver with porto-pulmonary hypertension in a patient with systemic lupus erythematosus. Lupus. 2006;15(10):686-8.
 
[11]  Riviere E, Vergniol J, Reffet A, Lippa N, Le Bail B, de Ledinghen V. Gastric variceal bleeding uncovering a rare association of CREST syndrome, primary biliary cirrhosis, nodular regenerative hyperplasia and pulmonary hypertension. Eur J Gastroenterol Hepatol. 2010 Sep;22(9):1145-8.
 
[12]  Fischer A, Bull TM, Steen VD. Practical approach to screening for scleroderma-associated pulmonary arterial hypertension. Arthritis Care Res (Hoboken). 2012 Mar;64(3):303-10.