Autoimmune Hepatitis

Research output: Contribution to journalReview articlepeer-review

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Autoimmune Hepatitis. / Mieli-Vergani, G; Heller, S; Jara, P; Vergani, D; Chang, MH; Fujisawa, T; Gonzalez-Peralta, RP; Kelly, Deirdre; Mohan, N; Shah, U; Murray, KF.

In: Journal of Pediatric Gastroenterology and Nutrition, Vol. 49, No. 2, 01.08.2009, p. 158-164.

Research output: Contribution to journalReview articlepeer-review

Harvard

Mieli-Vergani, G, Heller, S, Jara, P, Vergani, D, Chang, MH, Fujisawa, T, Gonzalez-Peralta, RP, Kelly, D, Mohan, N, Shah, U & Murray, KF 2009, 'Autoimmune Hepatitis', Journal of Pediatric Gastroenterology and Nutrition, vol. 49, no. 2, pp. 158-164. https://doi.org/10.1097/MPG.0b013e3181a1c265

APA

Mieli-Vergani, G., Heller, S., Jara, P., Vergani, D., Chang, MH., Fujisawa, T., Gonzalez-Peralta, RP., Kelly, D., Mohan, N., Shah, U., & Murray, KF. (2009). Autoimmune Hepatitis. Journal of Pediatric Gastroenterology and Nutrition, 49(2), 158-164. https://doi.org/10.1097/MPG.0b013e3181a1c265

Vancouver

Mieli-Vergani G, Heller S, Jara P, Vergani D, Chang MH, Fujisawa T et al. Autoimmune Hepatitis. Journal of Pediatric Gastroenterology and Nutrition. 2009 Aug 1;49(2):158-164. https://doi.org/10.1097/MPG.0b013e3181a1c265

Author

Mieli-Vergani, G ; Heller, S ; Jara, P ; Vergani, D ; Chang, MH ; Fujisawa, T ; Gonzalez-Peralta, RP ; Kelly, Deirdre ; Mohan, N ; Shah, U ; Murray, KF. / Autoimmune Hepatitis. In: Journal of Pediatric Gastroenterology and Nutrition. 2009 ; Vol. 49, No. 2. pp. 158-164.

Bibtex

@article{689ed1fa1d5545fd9341422bd98b5179,
title = "Autoimmune Hepatitis",
abstract = "Autoimmune hepatitis is characterized by inflammatory liver histology, circulating nonorgan-specific autoantibodies, and increased levels of immunoglobulin G, in the absence of a known etiology. Two types of juvenile autoimmune hepatitis (AIH) are recognized according to seropositivity for smooth muscle and/or anti-nuclear antibody (AIH type 1) or liver kidney microsomal antibody (AIH type 2). There is a female predominance in both. AIH type 2 presents more acutely, at a younger age and commonly with immunoglobulin A deficiency, whereas duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the 2 groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to non-adherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AM type I patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. Differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress. In this article we review the state of the art of diagnosis, monitoring, and treatment for children with AIH. JPGN 49:158-164, 2009.",
keywords = "Pediatrics, Liver disease, Autoimmune hepatitis, hepatitis, Immunosuppression",
author = "G Mieli-Vergani and S Heller and P Jara and D Vergani and MH Chang and T Fujisawa and RP Gonzalez-Peralta and Deirdre Kelly and N Mohan and U Shah and KF Murray",
year = "2009",
month = aug,
day = "1",
doi = "10.1097/MPG.0b013e3181a1c265",
language = "English",
volume = "49",
pages = "158--164",
journal = "Journal of Pediatric Gastroenterology and Nutrition",
issn = "0277-2116",
publisher = "Lippincott Williams and Wilkins",
number = "2",

}

RIS

TY - JOUR

T1 - Autoimmune Hepatitis

AU - Mieli-Vergani, G

AU - Heller, S

AU - Jara, P

AU - Vergani, D

AU - Chang, MH

AU - Fujisawa, T

AU - Gonzalez-Peralta, RP

AU - Kelly, Deirdre

AU - Mohan, N

AU - Shah, U

AU - Murray, KF

PY - 2009/8/1

Y1 - 2009/8/1

N2 - Autoimmune hepatitis is characterized by inflammatory liver histology, circulating nonorgan-specific autoantibodies, and increased levels of immunoglobulin G, in the absence of a known etiology. Two types of juvenile autoimmune hepatitis (AIH) are recognized according to seropositivity for smooth muscle and/or anti-nuclear antibody (AIH type 1) or liver kidney microsomal antibody (AIH type 2). There is a female predominance in both. AIH type 2 presents more acutely, at a younger age and commonly with immunoglobulin A deficiency, whereas duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the 2 groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to non-adherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AM type I patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. Differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress. In this article we review the state of the art of diagnosis, monitoring, and treatment for children with AIH. JPGN 49:158-164, 2009.

AB - Autoimmune hepatitis is characterized by inflammatory liver histology, circulating nonorgan-specific autoantibodies, and increased levels of immunoglobulin G, in the absence of a known etiology. Two types of juvenile autoimmune hepatitis (AIH) are recognized according to seropositivity for smooth muscle and/or anti-nuclear antibody (AIH type 1) or liver kidney microsomal antibody (AIH type 2). There is a female predominance in both. AIH type 2 presents more acutely, at a younger age and commonly with immunoglobulin A deficiency, whereas duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the 2 groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to non-adherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AM type I patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. Differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress. In this article we review the state of the art of diagnosis, monitoring, and treatment for children with AIH. JPGN 49:158-164, 2009.

KW - Pediatrics

KW - Liver disease

KW - Autoimmune hepatitis

KW - hepatitis

KW - Immunosuppression

U2 - 10.1097/MPG.0b013e3181a1c265

DO - 10.1097/MPG.0b013e3181a1c265

M3 - Review article

C2 - 19561543

VL - 49

SP - 158

EP - 164

JO - Journal of Pediatric Gastroenterology and Nutrition

JF - Journal of Pediatric Gastroenterology and Nutrition

SN - 0277-2116

IS - 2

ER -