By Craig Lammert, M.D., AIHA Executive Director
Two types of autoimmune hepatitis (AIH) have historically been recognized: Type 1 and Type 2. The type 2 variety, identified as having antibodies anti-LKM1 or LC1, has been identified as more common in children than adults. Also, it has been thought from prior studies that it may be associated with a more severe form of disease with harder-to-control liver inflammation, increased need for transplantation, and limited ability to ever stop immunosuppression.
Authors of a new paper published this year highlight an expanded experience that may change some thinking. The paper available in the Journal of Hepatology describes the experience of 117 children with AIH with a median follow-up time of 20 years from a single center in Italy. Included in this study: 65 patients with type 1 AIH and 52 with type 2 AIH.
Key takeaway: There was no difference observed between the type 1 and 2 patients regarding any outcome followed – including differences in ability to normalize liver tests, success of removing therapy, and overall survival and need for transplantation. This is a very different message than promoted from older studies with less follow up.
Treatment: The authors found that normalization of liver tests were observed in >90% of patients, and 19% were able to have immunosuppression removed without having resulting increased liver tests for more than 4 years of follow up.
Survival: 85% of patients were alive at the end of study follow up. The estimated survival at 10 years beyond diagnosis was 94%, 85% at 20 years and 81% at 30 years. This is great news – especially since many (80 out of the 117 patients) had cirrhosis at diagnosis.
Conclusion: Type 1 and type 2 AIH may have similar responses and outcomes, which are generally good. This is reassuring news for patients and their families. Ultimately, the authors did see that patients with abnormally long prothrombin time (a measure of liver function) may have higher risk of needing liver transplant during follow up.