Abstract
Abstract:Hepatopulmonary syndrome (HSP) is characterized by the triad of advanced liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. Most cases of HSP are associated with cirrhotic portal hypertension; however, it has also been reported in acute liver failure patients. An estimated prevalence of HPS in patients with chronic liver disease is around 5–32%. Sarcoidosis is a granulomatous disorder with noncaseating granulomas involving the lungs in 90% of cases. Sarcoidosis can involve the liver but in only about 1% of cases does it lead to cirrhosis and portal hypertension. In this review, we report a rare case of liver cirrhosis due to sarcoidosis with associated hepatopulmonary syndrome and discuss this in detail.
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References
References1. Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome—a liver-induced lung vascular disorder. N Engl J Med 2008;358:2378–2387.Rodríguez-RoisinR]]KrowkaMJHepatopulmonary syndrome—a liver-induced lung vascular disorder.N Engl J Med20083582378-23872. Fuhrmann V, Madl C, Mueller C, et al. Hepatopulmonary syndrome in patients with hypoxic hepatitis. Gastroenterology 2006;131:69–75.FuhrmannV]]MadlC]]MuellerC&etal;Hepatopulmonary syndrome in patients with hypoxic hepatitis.Gastroenterology200613169-753. Schenk P, Fuhrmann V, Madl C, et al. Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences. Gut 2002;51:853–859.SchenkP]]FuhrmannV]]MadlC&etal;Hepatopulmonary syndrome: prevalence and predictive value of various cut offs for arterial oxygenation and their clinical consequences.Gut200251853-8594. Judson MA. Sarcoidosis: clinical presentation, diagnosis, and approach to treatment. Am J Med Sci 2008;335:26–33.JudsonMASarcoidosis: clinical presentation, diagnosis, and approach to treatment.Am J Med Sci200833526-335. Blich M, Edoute Y. Clinical manifestations of sarcoid liver disease. J Gastroenterol Hepatol 2004;19:732–737.BlichM]]EdouteYClinical manifestations of sarcoid liver disease.J Gastroenterol Hepatol200419732-7376. Porter GH. Hepatic sarcoidosis. A cause of portal hypertension and liver failure; review. Arch Intern Med 1961;108:483–495.PorterGHHepatic sarcoidosis. A cause of portal hypertension and liver failure; review.Arch Intern Med1961108483-4957. Tekeste H, Latour F, Levitt RE. Portal hypertension complicating sarcoid liver disease: case report and review of the literature. Am J Gastroenterol 1984;79:389–396.TekesteH]]LatourF]]LevittREPortal hypertension complicating sarcoid liver disease: case report and review of the literature.Am J Gastroenterol198479389-3968. Ganguli SN, Steinhardt MI, Kundu S. Hepatopulmonary syndrome associated with sarcoidosis of the liver: case report. Can Assoc Radiol J 1998;49:411–414.GanguliSN]]SteinhardtMI]]KunduSHepatopulmonary syndrome associated with sarcoidosis of the liver: case report.Can Assoc Radiol J199849411-4149. Gupta S, Faughnan ME, Prud'homme GJ, et al. Sarcoidosis complicated by cirrhosis and hepatopulmonary syndrome. Can Respir J 2008;15:124–126.GuptaS]]FaughnanME]]Prud'hommeGJ&etal;Sarcoidosis complicated by cirrhosis and hepatopulmonary syndrome.Can Respir J200815124-12610. Lange PA, Stoller JK. The hepatopulmonary syndrome. Ann Intern Med 1995;122:521–529.LangePA]]StollerJKThe hepatopulmonary syndrome.Ann Intern Med1995122521-52911. Irani SK, Dobbins WO III. Hepatic granulomas: review of 73 patients from one hospital and survey of the literature. J Clin Gastroenterol 1979;1:131–143.IraniSK]]DobbinsWOIIIHepatic granulomas: review of 73 patients from one hospital and survey of the literature.J Clin Gastroenterol19791131-14312. Israel HL, Margolis ML, Rose LJ. Hepatic granulomatosis and sarcoidosis. Further observations. Dig Dis Sci 1984;29:353–356.IsraelHL]]MargolisML]]RoseLJHepatic granulomatosis and sarcoidosis. Further observations.Dig Dis Sci198429353-35613. Vatti R, Sharma OP. Course of asymptomatic liver involvement in sarcoidosis: role of therapy in selected cases. Sarcoidosis Vasc Diffuse Lung Dis 1997;14:73–76.VattiR]]SharmaOPCourse of asymptomatic liver involvement in sarcoidosis: role of therapy in selected cases.Sarcoidosis Vasc Diffuse Lung Dis19971473-7614. Bass NM, Burroughs AK, Scheuer PJ, et al. Chronic intrahepatic cholestasis due to sarcoidosis. Gut 1982;23:417–421.BassNM]]BurroughsAK]]ScheuerPJ&etal;Chronic intrahepatic cholestasis due to sarcoidosis.Gut198223417-42115. Rudzki C, Ishak KG, Zimmerman HJ. Chronic intrahepatic cholestasis of sarcoidosis. Am J Med 1975;59:373–387.RudzkiC]]IshakKG]]ZimmermanHJChronic intrahepatic cholestasis of sarcoidosis.Am J Med197559373-38716. Pereira-Lima J, Schaffner F. Chronic cholestasis in hepatic sarcoidosis with clinical features resembling primary biliary cirrhosis. Report of two cases. Am J Med 1987;83:144–148.Pereira-LimaJ]]SchaffnerFChronic cholestasis in hepatic sarcoidosis with clinical features resembling primary biliary cirrhosis. Report of two cases.Am J Med198783144-14817. Alam I, Levenson SD, Ferrell LD, Bass NM. Diffuse intrahepatic biliary strictures in sarcoidosis resembling sclerosing cholangitis. Case report and review of the literature. Dig Dis Sci 1997;42:1295–1301.AlamI]]LevensonSD]]FerrellLD]]BassNMDiffuse intrahepatic biliary strictures in sarcoidosis resembling sclerosing cholangitis. Case report and review of the literature.Dig Dis Sci1997421295-130118. Swanson KL, Wiesner RH, Krowka MJ. Natural history of hepatopulmonary syndrome: impact of liver transplantation. Hepatology 2005;41:1122–1129.SwansonKL]]WiesnerRH]]KrowkaMJNatural history of hepatopulmonary syndrome: impact of liver transplantation.Hepatology2005411122-112919. Lange PA, Stoller JK. The hepatopulmonary syndrome. Effect of liver transplantation. Clin Chest Med 1996;17:115–123.LangePA]]StollerJKThe hepatopulmonary syndrome. Effect of liver transplantation.Clin Chest Med199617115-12320. Krowka MJ, Cortese DA. Severe hypoxemia associated with liver disease: Mayo Clinic experience and the experimental use of almitrine bismesylate. Mayo Clin Proc 1987;62:164–173.KrowkaMJ]]CorteseDASevere hypoxemia associated with liver disease: Mayo Clinic experience and the experimental use of almitrine bismesylate.Mayo Clin Proc198762164-17321. Rodríguez-Roisin R, Agustí AG, Roca J. The hepatopulmonary syndrome: new name, old complexities. Thorax 1992;47:897–902.Rodríguez-RoisinR]]AgustíAG]]RocaJThe hepatopulmonary syndrome: new name, old complexities.Thorax199247897-902
