6360abefb0d6371309cc9857
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 |
|
Hemoglobin |
8.9 |
|
Total count |
7000 |
|
Platelets |
48000 |
|
MCV |
83 |
|
MCH |
27 |
|
MCHC |
32 |
Figure
2: Ballooning of
Hepatocytes
Figure 3: sinusoidal congestion with lymphocytic infiltration
Figure 4: Slide in high power field stained using
Reticulin