Table 1

Differential diagnosis of AIH following non-invasive work-up*

ConditionPrevalence (vs AIH)DemographyRisk factorsRaised liver enzymesAutoantibodiesRaised serum immunoglobulins
Drug-induced liver injury++SimilarM=F; all agesDrugs/herbs (medicinal/ recreational)VariesANA or ASMA in 20%Usually not
Primary biliary cholangitis22++SimilarOver 30 years, 90% F×10-fold in 1st-degree relatives; other autoimmune diseasesALP usually; ALT rarely >150 U/LAMA+ in 90% if absent, often ANA+IgM
Primary sclerosing cholangitis (PSC)24++SimilarM=F; all agesInflammatory bowel disease: about 5% of whom have PSCALP usually; ALT rarely >150 U/LNone typicalUsually normal
Non-alcoholic fatty liver disease26+++More commonM=F; all agesMetabolic syndromeALT usually; rarely >200 U/LANA+ or ASMA+ in 20%13 IgG
Wilson’s disease25RarerM=F; aged <40 yearsAutosomal recessiveVariableANA+/ASMA+ describedSometimes
AIH –All ages, 75% F×5-fold in 1st-degree relatives; other autoimmune diseasesALT (median 400 U/L) Spontaneously resolves in 20%80% ANA+/ASMA+, 8% AMA+, 2% LKM+, 30% SLA+Serum IgG in 80%–90%
  • Coexists in 5%–10% (++) and in 25%–30% (+++) of patients with AIH.

  • *Viral hepatitis is not included in the differential diagnosis, as it should have been excluded by serology.

  • †If suspected, arrange serum caeruloplasmin 24-hour urinary copper, slit-lamp examination for Kayser-Fleischer rings, MRI brain scan and genetic testing.

  • AIH, autoimmune hepatitis; ANA, antinuclear antibody; ASMA, anti-smooth muscle antibody; LKM, liver–kidney microsomal-1; SLA, soluble liver antigen.