Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cirrhosis: A Clinical Enigma

Pratap Singh, BM Singh Lamba, Dinesh Meher, Sanjay Kumar


“Overlap syndrome” is a term used to describe variant forms of autoimmune hepatitis (AIH) which present with characteristics of AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Patients with overlap syndrome present with both hepatitic and cholestatic serum liver test and the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. AIH-PBC is the most common form of overlap syndrome, affecting almost 10% of adults with AIH or PBC. Transitions from PBC to AIH-PBC overlap syndrome have also been reported. Ursodeoxycholic acid is usually combined with immunosuppressive therapy but end-stage disease requires liver transplantation. We present a case of Overlap Syndrome (AIHPBC)
in a 16 year old girl who presented to us with features of obstructive jaundice. She had six months history of generalised itching, yellow discoloration of skin and urine, decreased appetite and intermittent abdominal pain. Liver function test revealed conjugated hyperbilirubinemia, with moderate elevation
of liver enzymes. Antinuclear, anti-muscle M2 and anti-glycoprotein 210 antibodies were positive. Liver biopsy showed features of primary biliary cirrhosis. The patient responded to ursodeoxycholic acid and immunosuppression.


Autoimmune hepatitis, Primary biliary cirrhosis, Primary sclerosing cholangitis, Overlap syndrome

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Arrospide M, Vega J, Scavino Y, Luna E. Sindrome de

sobreposicion entre hepatitis autoinmune y cirrosis

biliar primaria: Reporte de caso. Rev. Gastroenterol.

Peru 2004; 24: 349-352.

Czaja AJ. The variant forms of autoimmune hepatitis.

Ann Intern Med 1996; 125: 588-598.

Dienes Hans, Erberich Heike, Dries Volker, Schirmacher

Peter, Lohse Ansgar. Autoimmune hepatitis and overlap

syndromes. Clin Liver Dis 2002; 6: 349-362.

Guerrero I, Montano A, Gallegos JF, Weimersheimer

M. Hepatitis autoinmune y colangitis esclerosante

primaria: .asociacion dependiente o independiente?

Rev Gastroenterol Mex 2007; 72 (3): 240-243.

Hola, Karen. Sindrome de sobreposicion. Gastr

Latinoam 2007; 18 (2): 198-199.Okuno T, Seto Y,

Okanoue T, Takino T. Chronic active hepatitis with

histological features of primary biliary cirrhosis. Dig

Dis Sci 1987; 32: 775-779.

Neuberger J. Autoimmune liver disease: Primary biliary

cirrhosis. Medicine 2006; 35 (2): 79-82.

Czaja AJ. Frequency and nature of the variant

syndromes of autoimmune liver diseases. Hepatology

; 28: 360-365.

Chazouilleres O, Wendum D, Serfaty L, Montembault

S, Rosmorduc O, Poupon R. Primary biliary cirrhosis–

autoimmune hepatitis overlap syndrome: clinical

features and response to therapy. Hepatology

;28:296- 301.

Boberg KM, Chapman RW, Hirschfield GM, Lohse AW,

Manns MP, Schrumpf E. Overlap syndromes: the

International Autoimmune Hepatitis Group (IAIHG)

position statement on a controversial issue. J Hepatol

;54:374- 385.

Czaja AJ. The overlap syndromes of autoimmune

hepatitis. Dig Dis Sci 2013; 58:326-343.

Czaja AJ. Diagnosis and management of the

overlap syndromes of autoimmune hepatitis. Can J

Gastroenterol 2013;27:417-423.

Beuers U, Boberg KM, Chapman RW, Chazouilleres

O, Invernizzi P, Jones DEJ, et al. EASL clinical practiceguidelines: management of cholestatic liver diseases.

J Hepatol 2009;51:237-267.

Gatselis NK, Zachou K, Papamichalis P, Koukoulis GK,

Gabeta S, Dalekos GN, et al. Comparison of simplified

score with the revised original score for the diagnosis

of autoimmune hepatitis: a new or a complementary

diagnostic score? Dig Liver Dis 2010;42:807-812.

Heathcote E J. Overlap syndromes. In: Bircher JBJ,

McIntyre N, Rizzeto M, Rodès J, eds. Oxford Textbook

of Clinical Hepatology ,Vol. 2. Oxford: Oxford University

Press, 1999;1135–40.

Overlap syndromes among autoimmune liver diseases.

Pietro Invernizzi, MD; Ian R Mackay, MD, World J

Gastroenterol 2008;14:3368-3373.


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