Diagnosis
Autoimmune hepatitis
An autoimmune inflammatory disorder of the liver that can progress to hepatic failure or liver cirrhosis.
Also known as: Autoimmune chronic active hepatitis, Autoimmune liver disease, Lupoid hepatitis, Plasma cell hepatitis
Etiology
Cause
Unknown
Genetics: certain HLA haplotypes are more susceptible
Pathogenesis [dmv]
Environmental triggers (infections, medications, and toxins)
Failure of immune tolerance --> T-cell mediated inflammation
Untreated individuals --> cirrhosis --> liver failure --> death
Epidemiology
Incidence per 100.000 [ib7][swh][ed8][qt2][gge]
Prevalence per 100.000 [ib7][swh][ed8][qt2][gge]
Symptoms & findings
Symptoms
Amenorrhea, Anorexia, Arthralgia, Confusion, Dark urine, Diarrhea, Edema, Fatigue, Hepatomegaly, Hirsutism, Myalgia, Nausea, Pruritus, Spider nevus, Splenomegaly, Vomiting, Weight loss
Clinical findings
Anemia, Ascites, Elevated ALAT, Elevated ASAT, Elevated Bilirubin, Elevated PT-INR, Elevated Sedimentation Rate, Eosinophilia, Jaundice, Thrombocytopenia
Anamneses
None listed.
Localized findings
Approach
Clinical diagnosis suported by: [dmv]
Blood test: ASAT, ALAT, antinuclear antibody (ANA), smooth muscle antibodies (SMA), hypergammaglobulinemia (specifically elevated IgG levels)
Biopsy
Exclusion of other liver diseases: Wilson disease, drug-induced hepatitis, NASH, viral hepatitis,PBC, and PSC
Treatment response to steroids
Treatment
Immunsuppresion: if elevated ASAT/ALAT (5-10 times the upper limit), elevated Gamma globulin (2 times upper limit), or necrosis in liver biopsy
Corticosteroids leads to complete remission and in most cases
Induction dose: Prednisone 60 mg daily (lower for combination therapy)
Maintenance dose: Prednisone 20 mg daily (lower for combination therapy)
Budesonide: reduce the adverse effects associated with steroid treatment
Azathioprine: combination of budesonide and azathioprine is emerging as an alternative first-line therapy
Other alternatives: Mycophenolate Mofetil, Tacrolimus
Liver transplantation:
Liver cirrhosis can develop in about 7% to 40% of treated patients (risk of HCC)
Differential diagnoses
Acute liver failure, Alcoholic liver disease, Drug-induced liver injury, Nonalcoholic fatty liver disease, Primary biliary cirrhosis, Primary sclerosing cholangitis, Systemic lupus erythematosus, Viral hepatitis, Wilson's disease
References
[1] Linzay CD, Sharma B, Pandit S. Autoimmune Hepatitis. [Updated 2023 Aug 14]: https://www.ncbi.nlm.nih.gov/books/NBK459186/
[2] Grønbæk L, Vilstrup H, Jepsen P. Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study. J Hepatol. 2014 Mar;60(3):612-7.
[3] Hahn JW, Yang HR, Moon JS, Chang JY, Lee K, Kim GA, Rahmati M, Koyanagi A, Smith L, Kim MS, López Sánchez GF, Elena D, Shin JY, Shin JI, Kwon R, Kim S, Kim HJ, Lee H, Ko JS, Yon DK. Global incidence and prevalence of autoimmune hepatitis, 1970-2022: a systematic review and meta-analysis. EClinicalMedicine. 2023 Oct 17;65:102280.
[4] Lamba M, Ngu JH, Stedman CAM. Trends in Incidence of Autoimmune Liver Diseases and Increasing Incidence of Autoimmune Hepatitis. Clin Gastroenterol Hepatol. 2021 Mar;19(3):573-579.e1.
[5] Puustinen L, Barner-Rasmussen N, Pukkala E, Färkkilä M. Incidence, prevalence, and causes of death of patients with autoimmune hepatitis: A nationwide register-based cohort study in Finland. Dig Liver Dis. 2019 Sep;51(9):1294-1299.
[6] Tunio NA, Mansoor E, Sheriff MZ, Cooper GS, Sclair SN, Cohen SM. Epidemiology of Autoimmune Hepatitis (AIH) in the United States Between 2014 and 2019: A Population-based National Study. J Clin Gastroenterol. 2021 Nov-Dec 01;55(10):903-910.
[7] https://emedicine.medscape.com/article/172356