Isolated hepatic sarcoidosis

Ivana Jovičić; Dušan Dj. Popović; Ljubiša Tončev; Žikica Jovičić; Violeta Vučinić; Nada Kovačević; Srdjan Djuranović; Ivan Boričić; Marjan Micev; Milan Špuran; Tomica Milosavljević

doi:10.2298/2797

Ivana Jovičić Clinic for Gastroenterology and Hepatology, Clinical Center of Serbia, Belgrade, Serbia
Dušan Dj. Popović Clinic for Gastroenterology and Hepatology, Clinical Center of Serbia, Belgrade, Serbia
Ljubiša Tončev Clinic for Gastroenterology and Hepatology, Clinical Center of Serbia, Belgrade, Serbia
Žikica Jovičić Clinic for Alergology and Immunology, Clinical Center of Serbia, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Violeta Vučinić Institute of Pathology, Faculty of Medicine, University of Belgrade, Belgrade Serbia
Nada Kovačević Clinic for Gastroenterology and Hepatology, Clinical Center of Serbia, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Srdjan Djuranović Clinic for Gastroenterology and Hepatology, Clinical Center of Serbia, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Ivan Boričić Institute of Pathology, Faculty of Medicine, University of Belgrade, Belgrade Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Marjan Micev Department for Histopathology of Clinic for Abdominal Surgery, Clinical Center of Serbia, Belgrade, Serbia
Milan Špuran Clinic for Gastroenterology and Hepatology, Clinical Center of Serbia, Belgrade, Serbia
Tomica Milosavljević Clinic for Gastroenterology and Hepatology, Clinical Center of Serbia, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia

DOI: https://doi.org/10.2298/2797

Keywords: sarcoidosis, liver cirrhosis, splenomegaly, ascites, diagnosis, histological techniques, treatment outcome,

Abstract

Introduction. Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Although hepatic granulomas occur in 50–65% of patients with systemic sarcoidosis, isolated liver sarcoidosis is rare. Clinical presentation varies from asymptomatic to manifest. The diagnosis is based on a characteristic histopathological finding of liver biopsy. Case report. We reported a 69-year old man was admitted due to abdominal swelling and abdominal pain. Laboratory studies detected: cholestasis, pancytopenia and elevaton of angiotensin-converting enzyme. Abdominal imaging techniques showed liver cirrhosis, splenomegaly and ascites. The diagnosis of the hepatic sarcoidosis was confirmed by histopathological examination of liver biopsy. The patient was treated with corticosteroids. After 18 months the patient was without any subjective symptoms, and with biochemical and clinical improvement. Conclusion. Isolated hepatic sarcoidosis should be considered in the differential diagnosis of asymptomatic or simptomatic patients with hepatosplenomegaly and changes in liver functional tests. Only the timely diagnosis and proper treatment can lead to subjective and objective improvement of patients.

References

Hunninghake GW, Costabel U, Ando M, Baughman R, Cordier JF, du Bois R, et al. Statement on sarcoidosis. Joint Statement of the Am Thor Soc (ATS), the Eur Resp Soc (ERS) and the World Assoc of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, Feb 1999. Am J Respir Crit Care Med 1999; 160(2): 736−55.

Baughman RP, Teirstein AS, Judson MA, Rossman MD, Jeager H Jr, Bresnitz EA, et al. Case Control Etiologic Study of Sarcoidosis (ACCESS) research group. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001; 164(10 Pt 1): 1885−9.

Neville E, Walker AN, James DG. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. Q J Med 1983; 52(208): 525−33.

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.

Dourakis SP, Cokkinos DD, Soultati AS, Alexopoulou A, Nezi V, Archimandritis AJ. A case of liver sarcoidosis mimicking cirrho-sis. Clin Imaging 2007; 31(1): 47−9.

Suzuki K, Morise Z, Furuta S, Tanahashi Y, Takeura C, Kagawa T, et al. Hepatic Sarcoidosis Mimicking Hilar Cholangiocarcino-ma: Case Report and Review of the Literature. Case Rep Ga-stroenterol 2011; 5(1): 152−8.

Škodric-Trifunović V, Vučinić V, Čolović RB, Videnović-Ivanov J, Žugić V, Stojšić J. Liver and splenic sarcoidosis: diagnostic pro-cedures. Med Pregl 2004; 57(9−10): 462−6. (Serbian)

Kennedy PTF, 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(7): 721−6.

Mueller S, Boehme MW, Hofmann WJ, Stremmel W. Extrapulmo-nary sarcoidosis primarily diagnosed in the liver. Scand J Ga-stroenterol 2000; 35(9): 1003−8.

Amarapurkar DN, Patel ND, Amarapurkar AD. Hepatic sarcoi-dosis. Indian J Gastroenterol 2003; 22(3): 98−100.

Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med 1997; 336(17): 1224−34.

Wainwright H. Hepatic granulomas. Eur J Gastroenterol Hepa-tol 2007; 19(2): 93−5.

Kahi CJ, Saxena R, Temkit M, Canlas K, Roberts S, Knox K, et al. Hepatobiliary disease in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2006; 23(2): 117−23.

Costabel U. Sarcoidosis: clinical update. Eur Respir J Suppl 2001; 32: 56s−68s.

Hercules HD, Bethlem NM. Value of liver biopsy in sarcoidosis. Arch Pathol Lab Med 1984; 108(10): 831−4.

Vanatta JM, Modanlou KA, Dean AG, Nezakatgoo N, Campos L, Nair S, et al. Outcomes of orthotopic liver transplantation for hepatic sarcoidosis: an analysis of the United Network for Or-gan Sharing/Organ Procurement and Transplantation Net-work data files for a comparative study with cholestatic liver diseases. Liver Transpl 2011; 17(9): 1027−34.

Ebert EC, Kierson M, Hagspiel KD. Gastrointestinal and hepatic manifestations of sarcoidosis. Am J Gastroenterol 2008; 103(12): 3184−92.

Karagiannidis A, Karavalaki M, Koulaouzidis A. Hepatic sarcoido-sis. Ann Hepatol 2006; 5(4): 251−6.

Rezeig MA, Fashir BM. Biliary tract obstruction due to sarcoi-dosis: a case report. Am J Gastroenterol 1997; 92(3): 527−8.

Pungpapong S, Steers JL, Wallace MB, Krishna M, Keaveny AP. He-patobiliary sarcoidosis mimicking Klatskin's cholangiocarci-noma. Gastrointest Endosc 2006; 64(1): 124−5.

Juntermanns B, Kaiser GM, Reis H, Saner FH, Radunz S, Vernadakis S, et al. Klatskin-mimicking lesions: still a diagnostical and therapeutical dilemma. Hepatogastroenterology 2011; 58(106): 265−9.

Cremers J, Drent M, Driessen A, Nieman F, Wijnen P, Baughman R, et al. Liver-test abnormalities in sarcoidosis. Eur J Gastroente-rol Hepatol 2012; 24(1): 17−24.

Kessler A, Mitchell DG, Israel HL, Goldberg BB. Hepatic and splenic sarcoidosis: ultrasound and MR imaging. Abdom Imaging 1993; 18(2): 159−63.

Turhan N, Kurt M, Ozderin YO, Kurt OK. Hepatic granulomas: a clinicopathologic analysis of 86 cases. Pathol Res Pract 2011; 207(6): 359−65.

Kleiner DE. Granulomas in the liver. Semin Diagn Pathol 2006; 23(3−4): 161−9.

Nakanuma Y, Ohta G. Quantitation of hepatic granulomas and epithelioid cells in primary biliary cirrhosis. Hepatology 1983; 3(3): 423−7.

Gaya DR, Thorburn D, Oien KA, Morris AJ, Stanley AJ. Hepatic granulomas: a 10 year single centre experience. J Clin Pathol 2003; 56(11): 850−3.

Geramizadeh B, Jahangiri R, Moradi E. Causes of hepatic granu-loma: a 12-year single center experience from southern Iran. Arch Iran Med 2011; 14(4): 288−9.

Judson MA. Hepatic, splenic, and gastrointestinal involvement with sarcoidosis. Semin Respir Crit Care Med 2002; 23(6): 529−41.

Ayyala US, Padilla ML. Diagnosis and treatment of hepatic sar-coidosis. Curr Treat Options Gastroenterol 2006; 9(6): 475−83.

Saito A, Takano M, Kaise S, Utsumi Y, Gunji N, Ishihata R, et al. Usefulness of ursodeoxycholic acid in a case of hepatic sarci-odosis. Nihon Shokakibyo Gakkai Zasshi 2010; 107(4): 632−8. (Japanese)