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Updated: Jun 10, 2026

Anti-Nuclear Antibody Screening Using HEp-2 Cells
Published on: June 23, 2014
Clinical characteristics of patients with autoimmune hepatitis seropositive for anticentromere antibody
Takashi Himoto1, Masayuki Murota, Hirohito Yoneyama
1Departments of Gastroenterology and Neurology, Kagawa University School of Medicine, Kagawa, Japan.
Insights
Autoimmune hepatitis (AIH) patients with anticentromere antibodies (ACA) show a higher frequency of concurrent autoimmune diseases and lower immunoglobulin G (IgG) levels. However, ACA presence does not define a distinct AIH entity.
Area of Science:
- Hepatology
- Immunology
- Autoimmune Diseases
Background:
- Primary biliary cirrhosis patients with anticentromere antibody (ACA) often develop portal hypertension.
- Clinical characteristics of autoimmune hepatitis (AIH) with ACA positivity are not well-defined.
Purpose of the Study:
- To investigate the clinical features of AIH patients positive for ACA (ACA-AIH).
- To compare ACA-AIH patients with AIH patients positive for other antinuclear antibody (ANA) patterns (other-AIH).
Main Methods:
- AIH diagnosis based on the International Autoimmune Hepatitis Group scoring system.
- ACA seropositivity determined by immunofluorescence assay.
- Histological activity index (HAI) used for grading and staging liver histology.
Main Results:
- Eight of 47 (17%) AIH patients were ACA-positive.
- ACA-AIH patients had a significantly higher frequency of concurrent autoimmune diseases (75% vs 36%).
- ACA-AIH patients showed significantly lower mean immunoglobulin G (IgG) levels (2176 vs 3013 mg/dL).
- No significant differences observed in age, sex, disease onset, hepatic failure, relapse rates, ANA titers, HAI scores, or HLA-DR4 positivity.
Conclusions:
- ACA positivity in AIH is associated with increased concurrent autoimmune diseases and lower serum IgG levels.
- The emergence of ACA in AIH does not represent a distinct clinical entity.
- Further research may clarify the specific role of ACA in AIH pathogenesis.
Aim:
Recent studies have revealed that primary biliary cirrhosis patients with anticentromere antibody (ACA) commonly develop portal hypertension. However, the clinical characteristics of autoimmune hepatitis (AIH) remain uncertain. We investigated the clinical features of patients with AIH seropositive for ACA (ACA-AIH), comparing them with those of patients with AIH seropositive for other immunofluorescent patterns of antinuclear antibodies (ANA) (other-AIH).
Methods:
AIH was diagnosed on the basis of the scoring system proposed by the International Autoimmune Hepatitis Group. Seropositivity for ACA was determined by a discrete speckled pattern on HEp-2 cells by an immunofluorescent technique. The severity of histological grading and staging was evaluated by the histological activity index (HAI) score.
Results:
Eight (17%) of 47 patients with AIH had ACA. No significant differences in age, sex, onset pattern of the disease, progression to hepatic failure and relapse rate were present between the ACA-AIH and other-AIH groups. The frequency of concurrent autoimmune diseases in ACA-AIH was significantly higher than that in other-AIH (75% vs 36%, P = 0.0406). Biochemical analysis revealed a significantly lower mean immunoglobulin G (IgG) level than that in other-AIH (2176 +/- 641 vs 3013 +/- 923 mg/dL, P = 0.0150). However, there were no differences in serum alanine aminotransferase levels, titers of ANA, HAI scores or the positive rate of human leukocyte antigen (HLA)-DR4 between the groups.
Conclusion:
These results suggest that the emergence of ACA is not a distinct entity of AIH, despite its clinical characteristics of a significantly higher frequency of concurrent autoimmune diseases and lower serum IgG levels.
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