Differential diagnosis of AIH following non-invasive work-up*
| Condition | Prevalence (vs AIH) | Demography | Risk factors | Raised liver enzymes | Autoantibodies | Raised serum immunoglobulins |
| Drug-induced liver injury++ | Similar | M=F; all ages | Drugs/herbs (medicinal/ recreational) | Varies | ANA or ASMA in 20% | Usually not |
| Primary biliary cholangitis22++ | Similar | Over 30 years, 90% F | ×10-fold in 1st-degree relatives; other autoimmune diseases | ALP usually; ALT rarely >150 U/L | AMA+ in 90% if absent, often ANA+ | IgM |
| Primary sclerosing cholangitis (PSC)24++ | Similar | M=F; all ages | Inflammatory bowel disease: about 5% of whom have PSC | ALP usually; ALT rarely >150 U/L | None typical | Usually normal |
| Non-alcoholic fatty liver disease26+++ | More common | M=F; all ages | Metabolic syndrome | ALT usually; rarely >200 U/L | ANA+ or ASMA+ in 20%13 | IgG |
| Wilson’s disease25† | Rarer | M=F; aged <40 years | Autosomal recessive | Variable | ANA+/ASMA+ described | Sometimes |
| AIH | – | All ages, 75% F | ×5-fold in 1st-degree relatives; other autoimmune diseases | ALT (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.