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Type I autoimmune hepatitis is primarily a disease of later life
1Department of Gastroenterology, Morriston Hospital, Swansea, UK.
Insights
Type 1 autoimmune hepatitis (AIH) predominantly affects older adults, contrary to prior beliefs. This study in South Wales found it typically presents as acute hepatitis in individuals over 60, responding well to immunosuppression.
Area of Science:
- Hepatology
- Immunology
- Internal Medicine
Background:
- Previous studies on type 1 autoimmune hepatitis (AIH) often used selected patient groups.
- Understanding the epidemiology and presentation in unselected populations is crucial.
Purpose of the Study:
- To characterize type 1 AIH in a stable, unselected population in South Wales.
- To describe the age distribution, clinical presentation, and treatment outcomes.
Main Methods:
- Retrospective analysis of 41 sequentially diagnosed patients with type 1 AIH over 11 years.
- Inclusion based on International AIH Group criteria.
- Assessment of clinical data, liver biopsy findings, treatment response, and survival.
Main Results:
- Median age at diagnosis was 62 years, with most patients over 60, differing from classical descriptions.
- The most common presentation was acute icteric hepatitis.
- Immunosuppression led to complete or partial remission in 36 of 39 treated patients.
- Overall 5-year survival was 64%.
Conclusions:
- Type 1 AIH in this population is a disease of later life, typically presenting as acute hepatitis.
- Immunosuppressive therapy is effective and safe for most patients.
- Survival rates are comparable across different age groups.
Abstract:
Previous studies of type I autoimmune hepatitis (AIH) have been based on selected populations attending specialist liver units. We describe 41 patients with type I AIH from a stable population in South Wales sequentially diagnosed over an 11-year period. All were Caucasian; 32 women, nine men; median follow-up 4 years. All cases conformed with International AIH Group criteria. Median age at diagnosis was 62 years. At presentation, only five patients were under 40 years old, 23 were over 60. This was very different from the classical age distribution. The commonest presentation was acute icteric hepatitis (29/41); median duration of illness 3 months. Co-existing acute arthralgia was common (10/41), as were other autoimmune diseases. Liver biopsy revealed piecemeal necrosis or interface hepatitis in 30; others showed non-diagnostic acute hepatitis. Fourteen also had cirrhosis. Median AsT fell from 508 (73-2385) to 32 (13-607) U/I after immunosuppression, representing complete remission in 28, partial remission in eight, and two treatment failures. Three patients were not treated, because of either quiescent disease or spontaneous remission. To date, there have been eight deaths (median age 64, range 37-86); five due to liver failure, one to variceal haemorrhage, one to acute myocardial infarction and one to upper gastrointestinal haemorrhage caused by acute idiopathic thrombocytopenia. Contrary to previous descriptions, type 1 AIH is a disease of later life in this stable, unselected population; peak incidence was in the seventh decade, and few cases occurred before the fifth decade. Presentation was usually as painless acute icteric hepatitis. Immunosuppression was effective and safe in most cases; 5-year survival rates were 61% (> 60 years at diagnosis), 66% (< 60) and 64% (overall).