Clinical and histopathological features of immunoglobulin G4‐associated autoimmune hepatitis in children. Issue 4 (14th November 2018)

• Record Type: Journal Article
• Authors: Aydemir, Yusuf; Akcoren, Zuhal; Demir, Hulya; Saltik Temizel, Inci Nur; Ozen, Hasan; Yuce, Aysel
• Is Part Of: Journal of gastroenterology and hepatology. Volume 34:Issue 4(2019)
• Page Start: 742
• Page End: 746
• Publication Date: 2018-11-14
• Subjects: autoimmune hepatitis -- IgG4‐associated AIH -- IgG4‐related diseases; Gastroenterology -- Periodicals; Digestive organs -- Diseases -- Periodicals; Liver -- Diseases -- Periodicals; Gastroenterology -- Periodicals; Liver Diseases -- Periodicals; 616.33
• DOI: 10.1111/jgh.14525
• Languages: English
• ISSNs: 0815-9319

Abstract

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.