Acute Severe Autoimmune Hepatitis: Corticosteroids or Liver Transplantation?

Abstract

Acute severe presentations of autoimmune hepatitis (AIH) represent a challenge for the transplant community. As a disease, it is poorly characterized, and there is a weak evidence base to guide diagnosis and treatment. Early identification of acute severe AIH is key because it determines the initiation of corticosteroids, which can be lifesaving. However, their use in this setting remains controversial. The Model for End‐Stage Liver Disease score, severity of coagulopathy, and grade of encephalopathy may be predictors of outcome with corticosteroid therapy. The optimal timing of liver transplantation (LT) can be difficult to determine and, as such, the decision to proceed to transplantation should not be delayed by protracted courses of corticosteroids. The aim of this review is to better characterize this subset of patients; to differentiate them clinically, serologically, and histologically from chronic AIH and other causes of acute liver failure; and to present the role, predictors, and optimal timings of corticosteroid therapy and LT. Although this review is specific to adults, many principles hold true for the pediatric population.

Acute Severe Autoimmune Hepatitis: Corticosteroids or Liver Transplantation?

“…We read with interest the review article by Rahim et al (1) who summarized acute autoimmune hepatitis (AIH), acute-severe AIH, and acute-severe AIH with acute liver failure (ALF). This review is brief, to the point, and valuable for understanding acute AIH, for which there is no gold standard for diagnosis and, therefore, for treatment.…”

Acute Severe Autoimmune Hepatitis: Corticosteroids or Liver Transplantation?

“…We read with interest the review by Rahim et al, (1) in which they gave an elegant summary of the role, predictors, and optimal timing of corticosteroids and liver transplantation (LT) in patients with acute severe autoimmune hepatitis (AS-AIH). There is no doubt that almost all AS-AIH patients should be considered for a trial of corticosteroids as soon as possible (the sooner the better) before full-blown acute liver failure develops.…”

Reply

“…We suggested assessing response at 7 days with the change in Model for End-Stage Liver Disease-sodium (MELD-Na) scores or bilirubin levels. (1) Fujiwara et al (2) suggest waiting 2 weeks before assessing the response to CS. In their study where 20 patients with autoimmune hepatitis (AIH)-induced ALF were treated with CS, the cumulative incidence of infections was 6% at 7 days, 17% at 10 days, 35% at 14 days, and 54% at 21 days from the induction of CS.…”

“…We appreciate the interest and comments from Fujiwara et al in response to our review article in a recent issue of Liver Transplantation . The aim of this review was to better define the entity of acute severe autoimmune hepatitis (AS‐AIH) and to give clinicians a more structured approach to assessing patients who have been commenced on corticosteroids (CS) . Recognizing the limitations, complications, and futility of CSs in certain patients and avoiding delay to inevitable liver transplantation (LT) is important in preventing worsening liver failure, multiorgan failure, septic complications, missing the “window” for successful LT, and death.…”

“…At the present time, no defined criteria exist for CS response in AS‐AIH. We suggested assessing response at 7 days with the change in Model for End‐Stage Liver Disease–sodium (MELD‐Na) scores or bilirubin levels …”