Seronegative autoimmune hepatitis in children: Spectrum of disorders.

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.

• Authors: Giuseppe Maggiore, Gérard Socie, Marco Sciveres, Anne-Marie Roque-Afonso, Silvia Nastasio, Catherine Johanet, Fréderic Gottrand, Sébastien Fournier-Favre, Emmanuel Jacquemin, Olivier Bernard
• Journal: Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver (Dig Liver Dis) Vol. 48 Issue 7 Pg. 785-91 (Jul 2016) ISSN: 1878-3562 [Electronic] Netherlands
• PMID: 27079745 (Publication Type: Journal Article)
• Copyright: Copyright © 2016 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

Chemical References

• Autoantibodies
• Immunosuppressive Agents
• gamma-Globins
• Transaminases

Topics

• Adolescent
• Anemia, Aplastic (epidemiology)
• Autoantibodies (blood)
• Child
• Child, Preschool
• Female
• France
• Hepatitis, Autoimmune (blood, complications, therapy)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Infant
• Italy
• Liver (pathology)
• Liver Failure (epidemiology)
• Liver Transplantation
• Male
• Thrombocytopenia (epidemiology)
• Transaminases (blood)
• gamma-Globins (analysis)