Autoimmune liver diseases (AILD) include a group of rare disorders. This study is designed to determine their frequency and characteristics in a hepatology clinic in Upper Egypt.

 Patients and methods: The study involved three groups; the first included 12 cases suspected to have AILD based on a scoring system of clinical, biochemical and immunological findings. The second group (n = 13) involved cases of viral hepatitis-related chronic liver disease. In addition to a control group (n = 15) of healthy volunteers. All patients were subjected to thorough clinical examination, abdominal ultrasonography, full liver function tests, complete blood count, ESR, and a battery of autoantibodies assayed with indirect immunoflourescence technique including antinuclear antibodies (ANA), anti-smooth muscle antibodies (ASMA), anti-mitochondrial antibodies (AMA), Anti liver/ kidney microsomal antibodies type 1 (LKM-1) and perinuclear staining anti-neutrophil cytoplasmic antibobies (pANCA).

Results:The study revealed high frequency of detection of autoantibodies in cases suspected to have AIH especially LKM-1 (50%), and pANCA (40%). This was statistically significant in comparison to viral hepatitis and control groups. ASMA were also detected in 50% of AIH cases but they were also found in 33.3% of control group and 30.7% of hepatitis group.

Conclusion: Autoantibodies are considered the main but not the only criterion for diagnosis of AILD in general, and AIH in particular.