Autoimmune hepatitis is a disease of unknown etiology characterized by interface hepatitis, hypergammaglobulinemia, circulating autoantibodies and a favorable response to immunosuppression.
Professor Dalekos and colleagues from Greece reviewed recent advancements in understanding aetiopathogenesis, clinical, serological and histological features, diagnostic criteria and treatment strategies of autoimmune hepatitis.
Published studies on autoimmune hepatitis extracted mainly from PubMed during the last 15 years.
Autoimmune hepatitis has a global distribution affecting any age, both sexes and all ethnic groups.
|Liver histology and autoantibodies still remain the hallmark for diagnosis|
|Alimentary Pharmacology & Therapeutics|
The team report that clinical manifestations are variable ranging from no symptoms to severe acute hepatitis and only seldom to fulminant hepatic failure.
Autoimmune attack is perpetuated, possibly via molecular mimicry mechanisms, and favored by the impaired control of regulatory T-cells.
A typical laboratory finding is hypergammaglobulinemia with selective elevation of IgG, although in 15–25% of patients – particularly children, elderly and acute cases – IgG levels are normal.
The research team found that liver histology and autoantibodies, although not pathognomonic, still remain the hallmark for diagnosis.
The researchers report that immunosuppressive treatment is mandatory and life-saving.
However, to meet strict response criteria, the conventional therapy with prednisolone with or without azathioprine is far from ideal.
Professor Dalekos' team concludes, "Autoimmune hepatitis remains a major diagnostic and therapeutic challenge."
"The clinician, the hepato-pathologist and the laboratory personnel need to become more familiar with different expressions of the disease, interpretation of liver histology and autoimmune serology."
"According to the strict definition of treatment response issued by the 2010 AASLD guidelines, many patients are nonresponders to conventional treatment."
"Newer immunosuppressive agents targeting pathogenetic mechanisms can improve patient management, which needs to be tailored on a case-by-case basis."