Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly

Abstract

Autoimmune hepatitis is classically a disease of young women. Our aims were to determine its occurrence, clinical phenotype, and outcome in elderly patients and contrast findings to young adults. Two-hundred-and-five white North American adults with definite type 1 autoimmune hepatitis were grouped according to age at presentation and the groups compared. Forty-seven patients (23%) were aged >60 years (median age, 68 years), and 31 patients (15%) were aged <30 years (median age, 25 years). The patients >60 years had a higher frequency of cirrhosis at presentation than the patients <30 years (33% versus 10%, P ‫؍‬ .03). They also had thyroid or rheumatic diseases more commonly (42% vs. 13%, P ‫؍‬ .006). HLA DR3 occurred more frequently in the patients <30 years than in those >60 years (58% vs. 23%, P ‫؍‬ .004), and HLA DR4 occurred more often in the patients >60 years (47% vs. 13%, P ‫؍‬ .003). Patients aged >60 years failed corticosteroid treatment less commonly than those aged <30 years (5% vs. 24%, P ‫؍‬ .03). Autoimmune hepatitis occurred in patients aged 18-30 years (15%), 31-39 years (15%), 40-49 years (21%), 50-59 years (25%), and >60 years (23%). Differences in age distribution, HLA frequencies, and treatment outcome occurred after age >40 years. In conclusion, elderly patients have a greater frequency of cirrhosis at presentation and HLA DR4 than patients <30 years, and they have a lower occurrence of treatment failure. Transitions in clinical and genetic phenotypes occur after age >40 years. Genetic susceptibilities may favor etiologic factors that are age-related. (HEPATOLOGY 2006;43:532-538.)

Clinical Features of Japanese Elderly Patients with Type 1 Autoimmune Hepatitis

“…Human leukocyte antigen DR status has been reported to affect the clinical features of patients with autoimmune hepatitis (1). Thus, the clinical features of Japanese elderly patients may differ from those of previous reports (3)(4)(5)(6). Here, we evaluated the clinical features of Japanese elderly patients (!65 years) with type 1 autoimmune hepatitis com-pared to those of Japanese younger patients (<65 years).…”

“…Recent reports from the patients with a main susceptibility of human leukocyte antigen DR3 and DR4, however, reveal that peak occurrences are between ages 10 and 30 years and between 40 and 50 years, and elderly patients have higher frequencies of human leukocyte antigen DR4, concurrent autoimmune disease, and cirrhosis at presentation (3)(4)(5)(6).…”

Review article: the prevention and reversal of hepatic fibrosis in autoimmune hepatitis

“…Increased doses of the original drugs or the introduction of different immunosuppressive agents (mycophenolate mofetil or calcineurin inhibitors) are strategies that warrant formal evaluation. 264,265 Young adults with autoimmune hepatitis aged <40 years and those with HLA DRB1*0301 can be slow or poor responders to conventional therapy, 18,98,266 and they may require an enhanced treatment regimen (high dose therapy, calcineurin inhibitors, or mycophenolate mofetil) at the time of presentation. 267,268 Alternatively, elderly patients aged ≥60 years and individuals with HLA DRB1*0401 can respond rapidly to standard therapy, 18,266,269 and they may require less intense treatment (low dose therapy or budesonide).…”

“…264,265 Young adults with autoimmune hepatitis aged <40 years and those with HLA DRB1*0301 can be slow or poor responders to conventional therapy, 18,98,266 and they may require an enhanced treatment regimen (high dose therapy, calcineurin inhibitors, or mycophenolate mofetil) at the time of presentation. 267,268 Alternatively, elderly patients aged ≥60 years and individuals with HLA DRB1*0401 can respond rapidly to standard therapy, 18,266,269 and they may require less intense treatment (low dose therapy or budesonide). 267,268,270 Ethnicity is another host-dependent factor that can affect the rapidity and completeness of the treatment response and require individualisation.…”

Autoimmune hepatitis: new paradigms in the pathogenesis, diagnosis, and management

“…As with most autoimmune diseases, AIH is more common in females (70%) and it affects all ages [1] and ethnic groups [2], although with different incidences. The prevalence in developed countries is 1:5,000-1:10,000.…”