A rare presentation of hypopituitarism in hepatic overlap syndrome of autoimmune hepatitis and autoimmune cholangitis

Vikas Gupta, Harpreet Singh, Paulomi Talapatra, Sucharita Ray
DOI: 10.15305/ijrci/v4i1/192


Autoimmune cholangitis is the antimitochondrial antibody-negative autoimmune hepatopathy with clinical and histological features similar to that of primary biliary cirrhosis. Autoimmune cholangitis has a predominant cholestatic phase. However, transaminasemia might be dominant in certain patients, indicating associated autoimmune hepatitis. Such an autoimmune hepatopathy has been termed as hepatic overlap syndrome. Due to the autoimmune nature of the disease, associated diseases of other organs have been reported. We report a case of an adult female with a very rare presentation of hepatic overlap syndrome of autoimmune cholangitis and autoimmune hepatitis associated with hypopituitarism.



Gupta V, Singh H, Talapatra P, Ray S. IJRCI. 2016;4(1):CS3 DOI: 10.15305/ijrci/v4i1/192


autoimmune hepatitis; autoimmune cholangitis; overlap syndrome; hypopituitarism


1. Cho Y, Han D, Kim T, Jang S, Jeon Y, Sohn J et al. Negative conversion of antimitochondrial antibody in primary biliary cirrhosis: a case of autoimmune cholangitis. Journal of Korean Medical Science.1999;14(1):102.

2. Beuers U. Hepatic overlap syndromes. Journal of Hepatology. 2005;42(1):S93-S99.

3. Heathcote J. Autoimmune cholangitis. Gut. 1997;40(4):440-442.

4. Alvarez F, Berg P, Bianchi F, Bianchi L, Burroughs A, Cancado E et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. Journal of Hepatology. 1999;31(5):929-938.

5. Jones S, Trainer P, Perry L, Wass J, Besser G, Grossman A. An audit of the insulin tolerance test in adult subjects in an acute investigation unit over one year.ClinEndocrinol. 1994;41(1):123-128.

6. Hurel S, Thompson C, Watson M, Harris M, Baylis P, Kendall-Taylor P. The short Synacthen and insulin stress tests in the assessment of the hypothalamic-pituitary-adrenal axis. ClinEndocrinol. 1996;44(2):141-146.

7. Miyakawa H, Tanaka A, Kikuchi K, Matsushita M, Kitazawa E, Kawaguchi N, et al. Detection of antimitochondrial autoantibodies in immunofluorescent AMA-negative patients with primary biliary cirrhosis using recombinant autoantigens. Hepatology 2001;34: 243–248.

8. Tsuneyama K, van de Water J, van Thiel D, Coppel R, Ruebner B, Nakanuma Y, et al. Abnormal expression of PDC-E2 on the apical surface of biliary epithelial cells in patients with antimitochondrial antibody-negative primary biliary cirrhosis. Hepatology 1995;22: 1440–1446.

9. Nishiki M, Murakami Y, Koshimura K, Sohmiya M, Tanaka J, Yabe S et al. A Case of Autoimmune Hypophysitis Associated with Asymptomatic Primary Biliary Cirrhosis. Endocrine Journal. 1998;45(5):697-700.

10. Bellastella A, Bizzarro A, Coronella C, Bellastella G, Sinisi A, De Bellis A. Lymphocytic hypophysitis: a rare or underestimated disease?European Journal of Endocrinology. 2003;149(5):363-376.

11. Karaca Z, Tanriverdi F, Unluhizarci K, Kelestimur F, Donmez H. Empty Sella may be the Final Outcome in Lymphocytic Hypophysitis. Endocrine Research. 2009;34(1-2):10-17.

12. Menon S K, Sarathi V, Bandgar T R, Menon P S, Goel N, Shah N S.Autoimmunehypophysitis: a single centre experience :Singapore Med J 2009; 50(11) :1080-84.