1. Primary biliary cirrhosis, cirrhotic stage
Chapter 4
Diseases of the Biliary Tract 1
Primary biliary cirrhosis, cirrhotic stage
Case 4.4

2. Clinical Presentation4
Diseases of the Biliary Tract 2
Clinical Presentation
A 50-year-old woman was noted to have abnormal liver tests on routine workup.
She had no clinical signs or symptoms of liver disease.

3. Laboratory Values Course in Hospital Serum IgM: 330 (elevated)4
Diseases of the Biliary Tract 3
Laboratory Values
Serum IgM: 330 (elevated)
Serum IgG: 1846 (elevated)
Ceruloplasmin: Normal
Serologies:
HBsAg, anti-HCV, HIV, SMA: Negative
AMA positive high titer
ANA positive
AST: 64
ALT: 69
Alk Phos: 158
Total Bilirubin: 0.3
Total Protein: 7.8
Albumin: 4.3
Course in Hospital
Because of the liver test abnormalities, primary biliary cirrhosis (PBC) was suspected. Additionally because of the positive ANA and elevated serum IgG, an autoimmune hepatitis – PBC overlap syndrome was also considered and a liver biopsy was performed.

4. 4
Diseases of the Biliary Tract 4
Pathology
A well-established cirrhosis was present, with the fibrous septa exhibiting a prominent predominantly lymphocytic infiltrate; little periseptal interface inflammatory activity was present and virtually no necroinflammatory change was seen further within the regenerative nodules (a, b).
Figure 4.4(a)
Figure 4.4(b)

5. 4
Diseases of the Biliary Tract 5
Pathology
In addition there was absence of interlobular bile ducts throughout the biopsy (c).
Figure 4.4(c)

6. Diagnosis Primary biliary cirrhosis (PBC), cirrhotic stage (stage 4)
Diseases of the Biliary Tract 6
Diagnosis
Primary biliary cirrhosis (PBC), cirrhotic stage (stage 4)
Note: Absence of periseptal and intralobular necroinflammatory change is strongly against a diagnosis of autoimmune hepatitis – PBC overlap syndrome.

7. 4
Diseases of the Biliary Tract 7
Comment
This is an example of PBC with cirrhosis and loss of interlobular bile ducts (stage 4 disease).
The patient interestingly had no signs of chronic liver disease, with a normal serum albumin, yet a well-established cirrhosis was present, one more reason liver biopsy is crucial in diagnosis despite liver test results.
The positive ANA with elevated serum IgG also initially raised the possibilty of an autoimmune hepatitis – PBC overlap syndrome; however, the virtual absence of periseptal interface inflammatory activity and intralobular inflammation is strongly against that possibility.
In addition patients with PBC alone can have a positive ANA.