Posttransplant Immune Hepatitis in Pediatric Liver Transplant Recipients: Incidence and Maintenance Therapy With Azathioprine

Abstract

A total of 2.35% of our transplant children present evidence of immune hepatitis after transplantation. Patients do not respond to increasing cyclosporine or tacrolimus levels and require steroid and azathioprine. In view of this specific treatment, systematic screening for "autoimmune" markers is advised in children with liver transplant.

Rejection and Steroid Dependence: Unique Risk Factors in the Development of Pediatric Posttransplant De Novo Autoimmune Hepatitis

“…However, in univariate analysis there was not a significant difference in the development of d-AIH and primary immunosuppressant (Table 3, p = 0.40). Previous studies have shown variability in the type of primary immunosuppression and the development of d-AIH (3)(4)(5)(6)(7)(8). One of the weaknesses of a retrospective study such as ours is that while it may demonstrate associations, it does not lend itself well toward evaluating the role that one medication versus another may play in the development of d-AIH.…”

“…In the series by Gupta et al all patients were on low-dose monotherapy calcineurin inhibitors at the time of d-AIH diagnosis, which the authors speculate may have triggered d-AIH (6). On the contrary, Andries et al reported that prior to the diagnosis of d-AIH 10 of 11 patients required daily prednisone to keep their LFTs within normal limits (4). Such variability highlights differences in management of immunosuppression from center to center.…”

“…followed by maintenance with either MMF or AzA (3)(4)(5)(6)(7)9,11 Awareness that successful treatment of d-AIH is possible with steroids plus AzA or MMF has led to excellent graft and patient survival. Once therapy for d-AIH was started, LFTs decreased rapidly in all of our patients, and remained significantly reduced at 3 months follow-up.…”

“…The diagnostic criteria for d-AIH are shown in Figure 1. This disorder affects between 2.1% and 5.2% of pediatric LT patients (3)(4)(5)(6)(7)(8)(9)(10)(11)(12), and between 0.4% and 3.4% of adult recipients (13)(14)(15)(16). The specific long-term treatment and outcome of d-AIH are not well established (4,15).…”

Alloimmune hepatitis following peripheral stem cell transplantation

“…1 Occasional examples of de novo autoimmune-like hepatitis have been reported following liver, bone marrow or stem cell transplantation. [3][4][5][6][7][8] Other etiologies including viral hepatitis, non-alcoholic steatohepatitis and metabolic liver disease were excluded. The diagnosis was distinguished from chronic GVHD as liver histopathology did not reveal any changes consistent with GVHD (Table 2).…”