Primary Biliary Cirrhosis with a normal Alkaline Phosphatase: a case report
Joshua M Brostoff1, Sukaina Rashid, Daniel McCrea
134 Fitzjohns Avenue, London , NW3 5NB, UK. joshbrostoff@doctors.org.uk.
Cases Journal
|July 16, 2008
Summary
Primary biliary cirrhosis can present without elevated alkaline phosphatase, a key diagnostic marker. This case highlights atypical clinical manifestations of this autoimmune liver disease.
Area of Science:
- Hepatology
- Autoimmune Diseases
- Clinical Case Study
Background:
- Primary biliary cirrhosis (PBC) is a chronic autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts.
- Elevated alkaline phosphatase (ALP) is a hallmark biochemical indicator in most PBC cases.
- Clinical presentation of PBC can vary, but typically includes fatigue and pruritus.
Purpose of the Study:
- To report a case of primary biliary cirrhosis presenting with atypical biochemical findings.
- To emphasize the importance of considering PBC even in the absence of elevated ALP.
- To discuss the diagnostic and management implications of such atypical presentations.
Main Methods:
- Case report of a 78-year-old female patient.
- Clinical presentation: abdominal swelling, fatigue, anemia, mild hypoalbuminemia.
- Diagnostic workup included computed tomography (CT) scan, serological tests (anti-mitochondrial antibodies, anti-nuclear antibodies), and liver function tests (gamma-glutamyl-transferase).
Main Results:
- CT scan revealed hepatosplenomegaly and mild ascites.
- Serological tests showed strongly positive anti-mitochondrial antibodies and anti-nuclear antibodies.
- Gamma-glutamyl-transferase was elevated, but alkaline phosphatase was normal.
- Diagnosis of primary biliary cirrhosis was established despite the normal ALP levels.
Conclusions:
- Primary biliary cirrhosis can manifest clinically without the typical elevation in alkaline phosphatase.
- This case underscores the need for a comprehensive diagnostic approach, including serological markers, in suspected PBC.
- Early recognition and management are crucial for improving outcomes in primary biliary cirrhosis.
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