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Case Report

Navigating the Overlap: A Case of Autoimmune Hepatitis with Primary Biliary Cholangitis



Abstract
The spectrum of immune mediated injury to the liver comprises of Autoimmune hepatitis, Primary Biliary sclerosis, Primary sclerosing Cholangitis, Immunoglobulin G-4 associated cholangitis and their poorly differentiated overlap syndromes. The diagnosis is based on histological, biochemical, serological and clinical parameters. Overlap syndromes can occur in 3 - 7 percent of the total cases. This is a case of a 51-year-old patient who presented in a tertiary hospital in Chennai, Tamil Nadu, with complaints of abdominal pain and vomiting for 1 week, who on progressive evaluation was diagnosed to be a case of overlap syndrome of Autoimmune hepatitis and Primary biliary cirrhosis.

Keywords:
 Autoimmune hepatitis; Primary biliary sclerosis; Primary sclerosing cholangitis; Immunoglobulin G-4 associated cholangitis

Introduction
Autoimmune hepatitis of the liver can present asymptomatically, acute or chronic liver disease and also as liver cirrhosis. It is characterized by unregulated T cell mediated immune damage to the hepatocyte and cholangiocyte. It can be triggered by a variety of other factors like environmental, viruses, bacteria, drugs, Eg: minocycline, toxins etc. They are characterized by predisposition from young to middle aged females, elevated transaminases, elevated gamma globulins organ specific and nonspecific auto antibodies.

Case Report
A 51-Year-old female had presented in a tertiary hospital in Chennai with complaints of abdominal pain for 1 week and vomiting for 1 week and upper gastrointestinal bleed (Figures 1-3). She was taken for emergency endoscopic variceal ligation. On examination the patient had abdominal distension with massive spleenomegaly:10cms below the left costal margin and pancytopenia, elevated total bilirubin, direct bilirubin, elevated transaminases and serum alkaline phosphatase (Figures 4,5). 

The following tables show the liver and renal function tests and hemogram of the patient (Tables 1,2).

Table 1:
Liver and renal function tests.

Total bilirubin

18

Direct bilirubin

14

 SGOT

130

SGPT

99

Total protein

7.4

Serum Albumin

3

Alkaline phosphatase

353

Urea

20

Creatinine

0.6

Table 2: Hemogram of the patient


Hemoglobin

8.9

Total count

7000

Platelets

48000

MCV

83

MCH

27

MCHC

32


Figure 1: Interface Hepatitis 


Figure 2: Ballooning of Hepatocytes



Figure 3: sinusoidal congestion with lymphocytic infiltration


Figure 4: Slide in high power field stained using Reticulin