Autoimmune Hepatitis Associated with Anti-Actin Antibodies in Children and Adolescents

Maggiore, Giuseppe; Veber, Florence; Bernard, Olivier; Hadchouel, Michelle; Homberg, J C; Álvarez, Fernando; Hadchouel, P; Alagille, D

doi:10.1097/00005176-199311000-00007

Cited by 124 publications

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“…5 Age also affects immune responses, including incidence of several autoimmune diseases. In AIH, 40% of cases of type 1 are diagnosed before the age of 18 years, with a mean age at onset of 10 years, 6,7 and 80% of cases of type 2 are diagnosed before the age of 18 years, with a mean age at onset of 6.5 years. 6,7 A second peak of incidence of AIH has also been reported in women after menopause.…”

Section: Foxp3mentioning

confidence: 99%

“…In AIH, 40% of cases of type 1 are diagnosed before the age of 18 years, with a mean age at onset of 10 years, 6,7 and 80% of cases of type 2 are diagnosed before the age of 18 years, with a mean age at onset of 6.5 years. 6,7 A second peak of incidence of AIH has also been reported in women after menopause. 8 These prepubertal and postmenopausal peaks of incidence suggest that the hormonal status could influence susceptibility to AIH.…”

Section: Foxp3mentioning

confidence: 99%

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Forkhead Box P3+ Regulatory T Cell Underlies Male Resistance to Experimental Type 2 Autoimmune Hepatitis

et al. 2010

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Autoimmune hepatitis (AIH), like many autoimmune diseases, is most prevalent in young women. The immunological basis of this age and sex susceptibility bias was investigated in a murine model of AIH. Xenoimmunization of 7-week-old female C57BL/6 mice resulted in more severe AIH with higher levels of liver inflammation, serum alanine aminotransferase, specific T-cell cytotoxicity, and autoantibody than younger and older females. Vaccinated males developed minimal liver inflammation and higher percentages of CD4 FoxP31 regulatory T cell in peripheral blood mononuclear cells, spleen, and liver than females. Regulatory T cells (Tregs) were virtually absent in liver-lymphocytes infiltrates of females. Castration of C57BL/6 mice, with or without 17b-estradiol supplementation, did not modify susceptibility in males, nor Treg numbers, suggesting minimal contribution of testosterone and estradiol to autoimmune hepatitis (AIH) susceptibility. Xenoimmunized Aire(1/0) mouse displayed similar AIH susceptibility, sex bias, and Tregs numbers as C57BL/6 mice, suggesting that susceptibility in females is not the result of less stringent thymic central tolerance. Autoreactive B cell response against formiminotransferase-cyclodeaminase correlated with disease activity, possibly linking B-cell autoreactivity and AIH pathogenesis. Conclusion: Peripheral tolerance and development of regulatory T cells after self-mimicking antigen exposure, and not sexual hormone nor central tolerance, are the main factors for susceptibility to AIH in females. (HEPATOLOGY 2010;51:1789-1798 P revalence of most autoimmune diseases shows a striking sex difference, with women being affected more often than men.1,2 The ratio of female patients to male ranges from 20:1 in Sjögren's syndrome, to 3:2 in multiple sclerosis.2 Less frequently, the ratio approaches 1:1, as in ulcerative colitis and diabetes.2 In autoimmune hepatitis (AIH), the female to male ratio ranges from 3:1 in type 1 AIH to 9:1 in type 2 AIH. 3Differences in the immune response have also been observed between women and men. Higher level of antibodies and stronger T cell activation are observed in women after vaccination. 4 Women also have higher absolute numbers of CD4 þ T cells and produce higher levels of Th1 cytokines than men.5 Age also affects immune responses, including incidence of several autoimmune diseases. In AIH, 40% of cases of type 1 are diagnosed before the age of 18 years, with a mean age at onset of 10 years, 6,7 and 80% of cases of type 2 are diagnosed before the age of 18 years, with a mean age at onset of 6.5 years.6,7 A second peak of incidence of AIH has also been reported in women after menopause.8 These prepubertal and postmenopausal peaks of incidence suggest that the hormonal status could influence susceptibility to AIH.Research on autoimmune diseases sex bias is scarce. Studies on AIH susceptibility factors, including its sex bias, have been severely limited by the lack of experimental models. Recently, a murine experimental model of AIH has been produced 9 in whi...

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Section: Foxp3mentioning

confidence: 99%

Section: Foxp3mentioning

confidence: 99%

Forkhead Box P3+ Regulatory T Cell Underlies Male Resistance to Experimental Type 2 Autoimmune Hepatitis

et al. 2010

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“…Hypoalbuminemia was found with a rate of 35% and prolongation in prothrombin time was found with a rate of 42%. Autoimmune hepatitis is associated with autoinflammatory diseases with a rate of 22-50% (17,20). We found autoimmune hemolytic anemia in 2 of our subjects (7%) and familial Mediterranean fever in one.…”

Section: Discussionmentioning

confidence: 47%

“…We found female subjects with a rate of 64.5% and the mean age to be 12 years. The time between the age at the time of onset of the disease and the diagnosis was found to be 6 months in a study (20) and 8 months in 2 other studies (18,19). We found this time to be 10 months (2-25 months).…”

Section: Discussionmentioning

confidence: 66%

Otoimmün hepatitli olgularımızın irdelenmesi: 11 yıllık deneyimimiz

Gulec¹,

Urgancı²,

Demirel³

et al. 2012

tpa

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Aim:This study aimed to review a series of patients with autoimmune hepatitis in terms of disease pattern, laboratory results, treatment outcomes and adverse effects of treatment. Material and Method: Children with autoimmune hepatitis were retrospectively reviewed. In all patients, viral and metobolic etiologies were excluded. Patients were classified as type-1, type-2 and non-classified type, as well as acute and chronic groups. Treatment response and cessation of treatment were evaluated. Results: Patients were beetween 4 and 17 years old (12±2.68 years). Twenty patients were female, 11 patients were male. Seventeen percent of the patients were in the acute group and 14% were in the chronic group; 18% were in the type-1 group, 6% were in the type-2 group and 7% were in the non-classified type group. Deflazacort was started in all patients. Azothiopurine (2 mg/kg/day) was added in 10 patients with late response at the end of the third month. Deflazacort dosage was decreased at 6-8 weeks intervals and continued at a maintenance dosage of 5 mg/day. After a two-year period complete response was obtained in 24 patients and partial response was obtained in 5 patients. No response was obtained in 2 patients. During the nine-year follow-up period, treatment was ended in 6 patients. In one patient, there was a recurrance in 6 months; the remaining 5 patients are still being followed up without a problem. Despite treatment, portal hypertension was observed in 5 patients. Conclusions: Early diagnosis and treatment of childhood autoimmune hepatitis can decrease the risk of progression to cirrhosis and can increase the survival. Deflazocort can be a choice instead of prednisolone because of its efficiency in treatment and lesser side effects. (Turk Arch Ped 2012; 47: 29-34)

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“…Higher serum bilirubin levels and transaminases at presentation are noted and patients have a higher propensity to present with fulminant hepatic failure [87,88]. Approximately 50% of children will be cirrhotic at presentation [87,[89][90][91]. Type 1 AIH (ANA/ASMA+) is the most common form in adults and tends to present between the ages of 40-50 years, although, it may present at any age level…”

Section: Children Vs Adultsmentioning

confidence: 99%

The management of childhood liver diseases in adulthood

Joshi

Gupta

Samyn

et al. 2017

Journal of Hepatology

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SummaryAn increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e., biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e., autoimmune hepatitis or Wilson's disease. To successfully manage these young adults, a dynamic and responsive transition service is essential. In this review, we aim to describe the successful components of a transition service highlighting the importance of self-management support and a multi-disciplinary approach. We will also review some of the liver specific aetiologies which are unique to young adults, offering an update on pathogenesis, management and outcomes. Ó

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Autoimmune Hepatitis Associated with Anti-Actin Antibodies in Children and Adolescents

Cited by 124 publications

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Forkhead Box P3+ Regulatory T Cell Underlies Male Resistance to Experimental Type 2 Autoimmune Hepatitis

Forkhead Box P3+ Regulatory T Cell Underlies Male Resistance to Experimental Type 2 Autoimmune Hepatitis

Otoimmün hepatitli olgularımızın irdelenmesi: 11 yıllık deneyimimiz

The management of childhood liver diseases in adulthood

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