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Antimitochondrial antibody-negative primary biliary cirrhosis
M A Lacerda1, J Ludwig, E R Dickson
1Division of Gastroenterology, Mayo Clinic, Rochester, Minnesota.
The American Journal of Gastroenterology
|February 1, 1995
Summary
Primary biliary cirrhosis patients lacking antimitochondrial antibodies (AMA) share clinical and biochemical traits with AMA-positive cases. AMA-negative patients more frequently exhibit antinuclear or anti-smooth-muscle antibodies, suggesting a spectrum of autoimmune cholangitis.
Area of Science:
- Hepatology
- Autoimmunology
- Gastroenterology
Background:
- Primary biliary cirrhosis (PBC) is an autoimmune liver disease.
- Antimitochondrial antibodies (AMA) are a hallmark of PBC.
- A subset of PBC patients lack AMA, presenting a diagnostic challenge.
Purpose of the Study:
- To investigate the characteristics of patients with histological PBC but negative AMA.
- To compare AMA-negative PBC patients with AMA-positive PBC patients.
- To explore the concept of autoimmune cholangitis as a spectrum.
Main Methods:
- Retrospective review of 597 PBC patients from 1976-1992.
- Identification of 35 patients (5.8%) negative for AMA with normal cholangiography.
- Comparison of clinical, biochemical, and serological features between AMA-positive and AMA-negative groups.
Main Results:
- AMA-negative PBC patients showed no differences in age, gender, or basic biochemistry compared to AMA-positive patients.
- Higher IgM and gamma-globulin levels were observed in AMA-positive patients.
- Antinuclear antibody (ANA) or anti-smooth-muscle antibodies (ASMA) were present in 96% of AMA-negative patients versus 56% of AMA-positive patients (p < 0.05).
- Five AMA-negative patients showed a comparable response to ursodeoxycholic acid (UDCA) as AMA-positive patients.
Conclusions:
- Histologically confirmed PBC patients, regardless of AMA status, exhibit similar clinical and biochemical profiles.
- ANA and ASMA are more prevalent in AMA-negative PBC.
- AMA-negative and AMA-positive PBC may represent a spectrum of autoimmune cholangitis, characterized by chronic cholestasis, specific histological findings, and diverse autoantibodies.