Treatment of Fulminant Autoimmune Hepatitis: Corticosteroid Therapy or Liver Transplantation? A Case Report and Literature Review

Abstract

Autoimmune hepatitis initially presenting as fulminant hepatic failure is rare in clinical practice. Although corticosteroid is considered as a good therapeutic agent in treating autoimmune hepatitis in the literature, the effect of corticosteroid in treating fulminant autoimmune hepatitis is still controversial. Because corticosteroid therapy for fulminant autoimmune hepatitis can sometimes overlook any future treatment such as delay the timing of liver transplantation and precipitate postoperative complications. We report a case of a 41-year-old female who was admitted to our hosptal because of acute hepatitis with severe jaundice. Type 1 autoimmune hepatitis complicated by fulminant hepatic failure was diagnosed on the basis of her clinical course and laboratory findings. Although we prescribed aggressive medical treatment, plasma transfusion, and plasma exchange therapy, her liver function deteriorated progressively and she developed hepatic coma later. Finally, her fulminant hepatic fuilure gained dramatic improvement after receiving an orthotopic liver transplant from her younger brother. High MELD score and poor treatment response of corticosteroid therapy are indicators of poor prognosis and need of prompt OLT. Moreover, the preoperative interventions should be applied carefully ensuring that they do not delay OLT or precipitate postoperative complications such as infection, bleeding, or poor wound healing.

Keywords: Acute; Autoimmune; Hepatitis; Liver failure; Liver transplantation; Plasma exchange; Steroids.

Figure 1

The explanted liver was shrunken, measuring 17.5 x 13.0 x 6.0 cm in size and 520 gm in weight.

Figure 2

Microscopic sections of the explanted liver showed massive hepatocellular necrosis with collapse, proliferation of the bile ducts, and infiltration of numerous inflammatory cells (hematoxylin and eosin staining; x 100).

Figure 3

The time course of changes in the serum levels of liver enzymes and total bilirubin.