Journal of Yeungnam Medical Science

영남대 의대 학술지 편집위원회 (Yeungnam University College of Medicine, Yeungnam University Institute Medical Science)
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Primary hepatic sarcoidosis presenting with cholestatic liver disease and mimicking primary biliary cholangitis: a case report

  • Park, Young Joo (Department of Internal Medicine, Pusan National University Hospital) ;
  • Woo, Hyun Young (Department of Internal Medicine, Pusan National University Hospital) ;
  • Kim, Moon Bum (Department of Dermatology, Pusan National University Hospital) ;
  • Ahn, Jihyun (Department of Pathology, Pusan National University Hospital) ;
  • Heo, Jeong (Department of Internal Medicine, Pusan National University Hospital)
  • 투고 : 2021.05.20
  • 심사 : 2021.06.28
  • 발행 : 2022.07.31
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초록

Sarcoidosis often involves the liver. However, primary hepatic sarcoidosis confined to the liver without evidence of systemic involvement is rare. We report the case of a 37-year-old man with hepatic sarcoidosis who initially presented with elevated liver enzymes and suspicious cirrhotic nodules on computed tomography. The patient had cirrhosis but did not have portal hypertension. Based on the initial histopathologic finding of chronic granulomatous inflammation and the common clinical characteristics of sarcoidosis, he was initially diagnosed with primary biliary cholangitis, and his daily dosage of ursodeoxycholic acid was increased to 900 mg. After 14 months of treatment, his total serum bilirubin concentration was 10.9 mg/dL (upper normal limit, 1.2 mg/dL). Additionally, a transjugular liver biopsy revealed multiple noncaseating granulomas. He was diagnosed with primary hepatic sarcoidosis involving the lungs, heart, spleen, kidneys, and skin. Treatment with methylprednisolone was initiated. Two weeks later, he was started on azathioprine, and the dose of steroid was simultaneously reduced. These findings indicate the importance of including hepatic sarcoidosis as a possible diagnosis in patients with elevated liver enzymes or cryptogenic cirrhosis.

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과제정보

This research was supported by a clinical research grant from Pusan National University Hospital in 2019.

참고문헌

  1. Ebert EC, Kierson M, Hagspiel KD. Gastrointestinal and hepatic manifestations of sarcoidosis. Am J Gastroenterol 2008;103:3184-92. https://doi.org/10.1111/j.1572-0241.2008.02202.x
  2. Mueller S, Boehme MW, Hofmann WJ, Stremmel W. Extrapulmonary sarcoidosis primarily diagnosed in the liver. Scand J Gastroenterol 2000;35:1003-8. https://doi.org/10.1080/003655200750023110
  3. Judson MA. Hepatic, splenic, and gastrointestinal involvement with sarcoidosis. Semin Respir Crit Care Med 2002;23:529-41. https://doi.org/10.1055/s-2002-36517
  4. Ishak KG. Sarcoidosis of the liver and bile ducts. Mayo Clin Proc 1998;73:467-72. https://doi.org/10.1016/S0025-6196(11)63733-6
  5. Blich M, Edoute Y. Clinical manifestations of sarcoid liver disease. J Gastroenterol Hepatol 2004;19:732-7. https://doi.org/10.1111/j.1440-1746.2003.03335.x
  6. Cremers J, Drent M, Driessen A, Nieman F, Wijnen P, Baughman R, et al. Liver-test abnormalities in sarcoidosis. Eur J Gastroenterol Hepatol 2012;24:17-24. https://doi.org/10.1097/MEG.0b013e32834c7b71
  7. Kennedy PT, Zakaria N, Modawi SB, Papadopoulou AM, Murray-Lyon I, du Bois RM, et al. Natural history of hepatic sarcoidosis and its response to treatment. Eur J Gastroenterol Hepatol 2006;18:721-6. https://doi.org/10.1097/01.meg.0000223911.85739.38
  8. Maddrey WC, Johns CJ, Boitnott JK, Iber FL. Sarcoidosis and chronic hepatic disease: a clinical and pathologic study of 20 patients. Medicine (Baltimore) 1970;49:375-95. https://doi.org/10.1097/00005792-197009000-00002
  9. Ungprasert P, Crowson CS, Simonetto DA, Matteson EL. Clinical characteristics and outcome of hepatic sarcoidosis: a population-based study 1976-2013. Am J Gastroenterol 2017;112:1556-63. https://doi.org/10.1038/ajg.2017.231
  10. Bihari C, Rastogi A, Kumar N, Rajesh S, Sarin SK. Hepatic sarcoidosis: clinico-pathological characterization of symptomatic cases. Acta Gastroenterol Belg 2015;78:306-13.
  11. Devaney K, Goodman ZD, Epstein MS, Zimmerman HJ, Ishak KG. Hepatic sarcoidosis. Clinicopathologic features in 100 patients. Am J Surg Pathol 1993;17:1272-80. https://doi.org/10.1097/00000478-199312000-00009
  12. Karagiannidis A, Karavalaki M, Koulaouzidis A. Hepatic sarcoidosis. Ann Hepatol 2006;5:251-6. https://doi.org/10.1016/S1665-2681(19)31983-0
  13. Giovinale M, Fonnesu C, Soriano A, Cerquaglia C, Curigliano V, Verrecchia E, et al. Atypical sarcoidosis: case reports and review of the literature. Eur Rev Med Pharmacol Sci 2009;13(Suppl 1):37-44.
  14. Flamm SL. Granulomatous liver disease. Clin Liver Dis 2012;16:387-96. https://doi.org/10.1016/j.cld.2012.03.013
  15. Syed U, Alkhawam H, Bakhit M, Companioni RA, Walfish A. Hepatic sarcoidosis: pathogenesis, clinical context, and treatment options. Scand J Gastroenterol 2016;51:1025-30. https://doi.org/10.1080/00365521.2016.1177856
  16. Tadros M, Forouhar F, Wu GY. Hepatic Sarcoidosis. J Clin Transl Hepatol 2013;1:87-93.
  17. Modaresi Esfeh J, Culver D, Plesec T, John B. Clinical presentation and protocol for management of hepatic sarcoidosis. Expert Rev Gastroenterol Hepatol 2015;9:349-58. https://doi.org/10.1586/17474124.2015.958468
  18. Bunting PS, Szalai JP, Katic M. Diagnostic aspects of angiotensin converting enzyme in pulmonary sarcoidosis. Clin Biochem 1987;20:213-9. https://doi.org/10.1016/S0009-9120(87)80123-6
  19. Kessler A, Mitchell DG, Israel HL, Goldberg BB. Hepatic and splenic sarcoidosis: ultrasound and MR imaging. Abdom Imaging 1993;18:159-63. https://doi.org/10.1007/BF00198055
  20. Cremers JP, Drent M, Baughman RP, Wijnen PA, Koek GH. Therapeutic approach of hepatic sarcoidosis. Curr Opin Pulm Med 2012;18:472-82. https://doi.org/10.1097/MCP.0b013e3283541626