Seronegative autoimmune hepatitis in children: Spectrum of disorders

Giuseppe Maggiore, Gérard Socie, Marco Sciveres, Anne Marie Roque-Afonso, Silvia Nastasio, Catherine Johanet, Fréderic Gottrand, Sébastien Fournier-Favre, Emmanuel Jacquemin, Olivier Bernard

Research output: Contribution to journalArticle

Abstract

BACKGROUND: A few children with acute or chronic liver disease display histological features compatible with autoimmune hepatitis, but lack specific serological markers.

AIM: To describe features, management and outcome of childhood seronegative autoimmune hepatitis.

METHODS: From 1988 to 2010, 38 children were included under the following criteria: negative virological studies, no serum autoantibodies, exclusion of other causes of liver diseases, and liver histology compatible with autoimmune hepatitis.

RESULTS: Four groups were identified: (1) 12 with increased serum gamma globulin concentrations; (2) 10 with normal or low serum gamma globulins and no combined blood disease; (3) 10 with combined aplastic anemia; and (4) 6 with peripheral thrombocytopenia with/without neutropenia. Immunosuppressive treatment was associated with aminotransferases normalization in all but one child who required liver transplantation. Relapses occurred in 10 children. Lymphocytopenia was found at the time of the diagnosis of hepatitis in 13 children, 12 in groups 3 or 4. All 38 children are alive after 4-17 years, 18 still under immunosuppression.

CONCLUSIONS: Childhood seronegative autoimmune hepatitis includes a spectrum of disorders. Early liver histology is recommended and, if compatible with autoimmune hepatitis, immunosuppressive treatment should be started. Initial lymphocytopenia may indicate future hematological complication.

Original languageEnglish
Pages (from-to)785-91
Number of pages7
JournalDigestive and Liver Disease
Volume48
Issue number7
DOIs
Publication statusPublished - Jul 2016

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Autoimmune Hepatitis
Serum Globulins
Lymphopenia
gamma-Globulins
Immunosuppressive Agents
Liver Diseases
Histology
Aplastic Anemia
Hematologic Diseases
Liver
Transaminases
Neutropenia
Thrombocytopenia
Liver Transplantation
Autoantibodies
Immunosuppression
Hepatitis
Chronic Disease
Recurrence
Therapeutics

Keywords

  • Journal Article

Cite this

Maggiore, G., Socie, G., Sciveres, M., Roque-Afonso, A. M., Nastasio, S., Johanet, C., ... Bernard, O. (2016). Seronegative autoimmune hepatitis in children: Spectrum of disorders. Digestive and Liver Disease, 48(7), 785-91. https://doi.org/10.1016/j.dld.2016.03.015

Seronegative autoimmune hepatitis in children : Spectrum of disorders. / Maggiore, Giuseppe; Socie, Gérard; Sciveres, Marco; Roque-Afonso, Anne Marie; Nastasio, Silvia; Johanet, Catherine; Gottrand, Fréderic; Fournier-Favre, Sébastien; Jacquemin, Emmanuel; Bernard, Olivier.

In: Digestive and Liver Disease, Vol. 48, No. 7, 07.2016, p. 785-91.

Research output: Contribution to journalArticle

Maggiore, G, Socie, G, Sciveres, M, Roque-Afonso, AM, Nastasio, S, Johanet, C, Gottrand, F, Fournier-Favre, S, Jacquemin, E & Bernard, O 2016, 'Seronegative autoimmune hepatitis in children: Spectrum of disorders', Digestive and Liver Disease, vol. 48, no. 7, pp. 785-91. https://doi.org/10.1016/j.dld.2016.03.015
Maggiore, Giuseppe ; Socie, Gérard ; Sciveres, Marco ; Roque-Afonso, Anne Marie ; Nastasio, Silvia ; Johanet, Catherine ; Gottrand, Fréderic ; Fournier-Favre, Sébastien ; Jacquemin, Emmanuel ; Bernard, Olivier. / Seronegative autoimmune hepatitis in children : Spectrum of disorders. In: Digestive and Liver Disease. 2016 ; Vol. 48, No. 7. pp. 785-91.
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AU - Johanet, Catherine

AU - Gottrand, Fréderic

AU - Fournier-Favre, Sébastien

AU - Jacquemin, Emmanuel

AU - Bernard, Olivier

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N2 - BACKGROUND: A few children with acute or chronic liver disease display histological features compatible with autoimmune hepatitis, but lack specific serological markers.AIM: To describe features, management and outcome of childhood seronegative autoimmune hepatitis.METHODS: From 1988 to 2010, 38 children were included under the following criteria: negative virological studies, no serum autoantibodies, exclusion of other causes of liver diseases, and liver histology compatible with autoimmune hepatitis.RESULTS: Four groups were identified: (1) 12 with increased serum gamma globulin concentrations; (2) 10 with normal or low serum gamma globulins and no combined blood disease; (3) 10 with combined aplastic anemia; and (4) 6 with peripheral thrombocytopenia with/without neutropenia. Immunosuppressive treatment was associated with aminotransferases normalization in all but one child who required liver transplantation. Relapses occurred in 10 children. Lymphocytopenia was found at the time of the diagnosis of hepatitis in 13 children, 12 in groups 3 or 4. All 38 children are alive after 4-17 years, 18 still under immunosuppression.CONCLUSIONS: Childhood seronegative autoimmune hepatitis includes a spectrum of disorders. Early liver histology is recommended and, if compatible with autoimmune hepatitis, immunosuppressive treatment should be started. Initial lymphocytopenia may indicate future hematological complication.

AB - BACKGROUND: A few children with acute or chronic liver disease display histological features compatible with autoimmune hepatitis, but lack specific serological markers.AIM: To describe features, management and outcome of childhood seronegative autoimmune hepatitis.METHODS: From 1988 to 2010, 38 children were included under the following criteria: negative virological studies, no serum autoantibodies, exclusion of other causes of liver diseases, and liver histology compatible with autoimmune hepatitis.RESULTS: Four groups were identified: (1) 12 with increased serum gamma globulin concentrations; (2) 10 with normal or low serum gamma globulins and no combined blood disease; (3) 10 with combined aplastic anemia; and (4) 6 with peripheral thrombocytopenia with/without neutropenia. Immunosuppressive treatment was associated with aminotransferases normalization in all but one child who required liver transplantation. Relapses occurred in 10 children. Lymphocytopenia was found at the time of the diagnosis of hepatitis in 13 children, 12 in groups 3 or 4. All 38 children are alive after 4-17 years, 18 still under immunosuppression.CONCLUSIONS: Childhood seronegative autoimmune hepatitis includes a spectrum of disorders. Early liver histology is recommended and, if compatible with autoimmune hepatitis, immunosuppressive treatment should be started. Initial lymphocytopenia may indicate future hematological complication.

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