Abstract
Development of hepatocellular carcinoma (HCC) in patients with autoimmune liver disease is less common than in those with other types of chronic liver disease. Here we report a rare case of overlap syndrome consisting of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) that was subsequently complicated with HCC. A 72-year-old man was initially diagnosed as being in the cirrhotic stage of AIH on the basis of blood chemistry tests and histological examinations. Computed tomography and magnetic resonance cholangiography 20 months later showed diffuse stricturing of the intrahepatic bile duct with dilatation of the areas between the strictures, compatible with the findings of PSC, which resulted in a diagnosis of AIH/PSC overlap syndrome. The level of serum protein induced by vitamin K absence or antagonist II increased 22 months later, and HCC was diagnosed by radiological examinations. Four cycles of transarterial infusion therapy with cisplatin were performed, but the patient died one year later. Sequential overlap of PSC may have played a part in accelerating AIH disease progression, leading to the development of HCC in this patient. Therefore, HCC surveillance may be important in advanced stages of autoimmune disease, especially in the cirrhotic stage.
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Czala AL, Manns MP. Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology. 2010;139:58–72.
Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010;51:660–78.
Kaya M, Angulo P, Lindor KD. Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol. 2000;33:537–42.
Floreani A, Rizzotto ER, Ferrara F, Carderi I, Caroli D, Blasone L, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol. 2005;100:1516–22.
Lüth S, Kanzler S, Frenzel C, Kasper HU, Dienes HP, Schramm C, et al. Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. J Clin Gastroenterol. 2009;43:75–80.
Bruix J, Sherman M. AASLD practice guideline. Management of hepatocellular carcinoma: an update. Hepatology. 2011;3:1020–2.
Yeoman AD, Al-Chalabi T, Karani JB, Quaglia A, Devlin J, Mieli-Vergani G, et al. Evaluation of risk factors in the development of hepatocellular carcinoma in autoimmune hepatitis: implications for follow-up and screening. Hepatology. 2008;48:863–70.
Wong RJ, Gish R, Frederick T, Bzoweji N, Frenette C. Development of hepatocellular carcinoma in autoimmune hepatitis patients: a case series. Dig Dis Sci. 2011;56:578–85.
Hennes EM, Zeniya M, Czaja AJ, Pares A, Dalekos GN, Krawitt EJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008;48:169–76.
Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, et al. Autoimmune hepatitis/sclerosing overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33:544–53.
Abdalian R, Dhar P, Jhaveri K, Haider M, Guindi M, Heathcote EJ. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging. Hepatology. 2008;47:949–57.
Lewin M, Vilgrain V, Ozenne V, Lemoine M, Wendum D, Paradis V, et al. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: a prospective magnetic resonance imaging and histological study. Hepatology. 2009;50:528–37.
Abdo AA, Bain VG, Kichian K, Lee SS. Evolution of autoimmune hepatitis to primary sclerosing cholangitis: a sequential syndrome. Hepatology. 2002;36:1393–9.
Buuren HR, Hoogstraten HJF, Terkivatan T, Schalm SW, Vleggaar FP. High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol. 2000;33:543–8.
Takikawa H, Manabe T. Primary sclerosing cholangitis in Japan-analysis of 192 cases. J Gastroenterol. 1997;32:919–35.
Takiguchi J, Ohira H, Rai T, Shishido S, Tojo J, Sato Y, et al. Autoimmune hepatitis overlap** with primary sclerosing cholangitis. Intern Med. 2002;41:696–700.
Montano-Loza AJ, Carpenter HA, Czaja AJ. Predictive factors for hepatocellular carcinoma in type 1 autoimmune hepatitis. Am J Gastroenterol. 2008;103:1944–51.
Burak K, Angulo P, Pasha TM, Egan K, Petz J, Lindor KD. Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis. Am J Gastroenterol. 2004;99:523–6.
Prytz H, Keiding S, Bjornsson E. Dynamic FDG-PET is useful for detection of cholangiocarcinoma in patients with PSC listed for liver transplantation. Hepatology. 2006;44:1572–80.
Harnois DM, Gores JG, Ludwig J, Steers JL, LaRusso NE, Wiesner RH. Are patients with cirrhotic stage primary sclerosing cholangitis at risk for the development of hepatocellular cancer? J Hepatol. 1997;27:512–6.
Leidenius M, Hockersted K, Broome U, Ericzon BG, Friman S, Olauson M, et al. Hepatobiliary carcinoma in primary sclerosing cholangitis: a case control study. J Hepatol. 2001;34:792–8.
Maeda C, Tamano M, Murohisa T, Yamagishi T, Hashimoto T, Kojima K, et al. Hepatocellular carcinoma associated with noncirrhotic autoimmune hepatitis. Clin J Gastroenterol. 2010;3:111–5.
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Fukuda, K., Kogita, S., Tsuchimoto, Y. et al. Overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis complicated with hepatocellular carcinoma. Clin J Gastroenterol 5, 183–188 (2012). https://doi.org/10.1007/s12328-012-0294-5
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DOI: https://doi.org/10.1007/s12328-012-0294-5