Autoimmune hepatitis (AIH) is not a homogenous disease. Children present and respond differently than adults as do different ethnic groups.
“There is an increased incidence of autoimmune overlap with sclerosing cholangitis in children,” said Nanda Kerkar, MD, Professor of Pediatrics and Director of Pediatric Liver Disease & Liver Transplant Program at the University of Rochester Medical Center New York. “Ethnicity needs to be taken into account in the management of AIH, and consideration should be given for more aggressive therapies, earlier in some populations.”
Dr. Kerkar discussed presentation and treatment of AIH during the Cholestatic and Autoimmune Special Interest Group session on Friday. While AIH is a progressive inflammatory disorder of the liver characterized by elevated serum aminotransaminases and immunoglobulin G levels, current data suggest that the specifics of presentation and treatment are different in different populations.
Multiple studies have compared AIH across ethnic groups. And while the specific results vary by study, the general message is that ethnicity matters in both presentation and treatment.
Emory University found that African Americans with AIH present at an earlier age than Caucasians and are more than twice as likely to have cirrhosis in their initial liver biopsy. Response to immunosuppression was similar in both populations, but African Americans required higher levels of steroids to maintain AIH remission.
A Charleston, SC study also found different ethnic patterns. Compared to African American patients, Caucasian and Hispanic patients were more likely to be diagnosed with cirrhosis on initial presentation and more likely to be younger. AIH caused cirrhosis at a younger age regardless of ethnicity compared to cryptogenic NAFLD or NASH.
A San Francisco study found that Hispanics have the highest prevalence of biopsy-confirmed cirrhosis but that Asians had poorer survival outcomes.
A British study found that non-European patients tended to be younger at presentation, had cholestatic biochemistry and showed poor initial response to standard therapy. Another British study found that black patients are more likely to have cirrhosis, liver failure at initial presentation and liver transplantation referral than Caucasian patients. Black males were more likely to have more aggressive disease at presentation, less likely to respond to conventional immunosuppression and most likely to have the worst outcomes.
“What this tells us is that ethnicity matters in both the presentation and response to therapy in autoimmune hepatitis,” Dr. Kerkar said. “Ethnicity needs to be taken into account in the management of this disease and is likely to have an important role as we develop personalized medicine approaches to AIH.”
Dr. Kerkar also noted that children are another special population.
Pediatric patients with type 1 AIH, ANA/SMA positive are older, with a median age of 11, and are more likely to have cirrhosis than children with type 2 AIH, LKM/anti liver cytosol positive. Type 1 AIH also has an overlap with sclerosing cholangitis and is associated with inflammatory bowel disease.
Patients with type 2 AIH are younger with a median age of seven, and are more likely to have more severe liver disease and acute liver failure compared to type 1 patients. Most type 2 patients do not tolerate weaning off immunosuppression and are unlikely to have overlap with sclerosing cholangitis.
Autoimmune diseases tend to flare during puberty, Dr. Kerkar added. Patients should not be weaned off immunosupressants until puberty has passed.