Yusuf Aydemir1, Zuhal Akcoren2, Hulya Demir3, Inci Nur Saltik Temizel3, Hasan Ozen3, Aysel Yuce3. 1. Department of Pediatric Gastroenterology and Hepatology, Eskisehir Osmangazi University School of Medicine, Eskisehir, Turkey. 2. Department of Pediatric Pathology, Hacettepe University School of Medicine, Ankara, Turkey. 3. Department of Pediatric Gastroenterology and Hepatology, Hacettepe University School of Medicine, Ankara, Turkey.
Abstract
BACKGROUND AND AIM: We aimed to examine the frequency and the characteristics of immunoglobulin G4 (IgG4)-associated autoimmune hepatitis among pediatric patients with autoimmune hepatitis. METHODS: Immunostaining for IgG and IgG4 was performed in liver biopsies of 40 pediatric patients with autoimmune hepatitis. The patients with more than 10 IgG4-positive plasma cells/high-power field were defined as IgG4-associated autoimmune hepatitis. Clinic, laboratory, and histopathological results were compared between groups. RESULTS: Among the 40 pediatric patients, 34 patients were type 1 and 6 patients were type 2 autoimmune hepatitis. Six patients (15%), four of the type 1 and two of the type 2 autoimmune hepatitis patients, were diagnosed with IgG4-associated autoimmune hepatitis. Clinical, laboratory, and histopathological data were initially similar in both forms. There was a positive correlation between IgG4-positive plasma cell count and degree of portal (r: 0.406, P: 0.009) and lobular inflammation (r: 0.37, P: 0.019), grade of interface hepatitis (r: 0.33, P: 0.03), and fibrosis (r: 0.318, P: 0.046). Time required for normalization of liver transaminases and serum IgG level was significantly shorter in IgG4-associated autoimmune hepatitis (3.3 ± 0.5 vs 6.6 ± 3.5 for alanine aminotransferase, 3.7 ± 0.8 vs 6.7 ± 1.2 for aspartate aminotransferase, 4.3 ± 1.2 vs 7.1 ± 2.7 for gamma-glutamyl transpeptidase, and 7.2 ± 3.1 vs 12.8 ± 4.5 for IgG). CONCLUSIONS: Immunoglobulin G4-associated autoimmune hepatitis can be found in pediatric age group and also in type 2 autoimmune hepatitis patients. As steroid response may be better in IgG4-associated autoimmune hepatitis, biopsy specimens should be evaluated for this entity at diagnosis.
BACKGROUND AND AIM: We aimed to examine the frequency and the characteristics of immunoglobulin G4 (IgG4)-associated autoimmune hepatitis among pediatric patients with autoimmune hepatitis. METHODS: Immunostaining for IgG and IgG4 was performed in liver biopsies of 40 pediatric patients with autoimmune hepatitis. The patients with more than 10 IgG4-positive plasma cells/high-power field were defined as IgG4-associated autoimmune hepatitis. Clinic, laboratory, and histopathological results were compared between groups. RESULTS: Among the 40 pediatric patients, 34 patients were type 1 and 6 patients were type 2 autoimmune hepatitis. Six patients (15%), four of the type 1 and two of the type 2 autoimmune hepatitispatients, were diagnosed with IgG4-associated autoimmune hepatitis. Clinical, laboratory, and histopathological data were initially similar in both forms. There was a positive correlation between IgG4-positive plasma cell count and degree of portal (r: 0.406, P: 0.009) and lobular inflammation (r: 0.37, P: 0.019), grade of interface hepatitis (r: 0.33, P: 0.03), and fibrosis (r: 0.318, P: 0.046). Time required for normalization of liver transaminases and serum IgG level was significantly shorter in IgG4-associated autoimmune hepatitis (3.3 ± 0.5 vs 6.6 ± 3.5 for alanine aminotransferase, 3.7 ± 0.8 vs 6.7 ± 1.2 for aspartate aminotransferase, 4.3 ± 1.2 vs 7.1 ± 2.7 for gamma-glutamyl transpeptidase, and 7.2 ± 3.1 vs 12.8 ± 4.5 for IgG). CONCLUSIONS: Immunoglobulin G4-associated autoimmune hepatitis can be found in pediatric age group and also in type 2 autoimmune hepatitispatients. As steroid response may be better in IgG4-associated autoimmune hepatitis, biopsy specimens should be evaluated for this entity at diagnosis.
Authors: J-Matthias Löhr; Ulrich Beuers; Miroslav Vujasinovic; Domenico Alvaro; Jens Brøndum Frøkjær; Frank Buttgereit; Gabriele Capurso; Emma L Culver; Enrique de-Madaria; Emanuel Della-Torre; Sönke Detlefsen; Enrique Dominguez-Muñoz; Piotr Czubkowski; Nils Ewald; Luca Frulloni; Natalya Gubergrits; Deniz Guney Duman; Thilo Hackert; Julio Iglesias-Garcia; Nikolaos Kartalis; Andrea Laghi; Frank Lammert; Fredrik Lindgren; Alexey Okhlobystin; Grzegorz Oracz; Andrea Parniczky; Raffaella Maria Pozzi Mucelli; Vinciane Rebours; Jonas Rosendahl; Nicolas Schleinitz; Alexander Schneider; Eric Fh van Bommel; Caroline Sophie Verbeke; Marie Pierre Vullierme; Heiko Witt Journal: United European Gastroenterol J Date: 2020-06-18 Impact factor: 4.623
Authors: Jiachen Liu; Wei Yin; Lisa S Westerberg; Pamela Lee; Quan Gong; Yan Chen; Lingli Dong; Chaohong Liu Journal: Front Immunol Date: 2021-09-01 Impact factor: 7.561
Authors: Brian H Horwich; Tom Z Liang; Jennifer L Dodge; Shefali Chopra; Jeffrey A Kahn; Takeshi Saito Journal: Transpl Int Date: 2022-03-18 Impact factor: 3.842