Autoimmune Hepatitis: Diagnostic Dilemma in the Setting of Suspected Iron Overload

Ashish K Tiwari,Heather S Laird-Fick,Srujan Ameda,In Chul An

doi:10.1155/2013/872987

Ashish K Tiwari, Heather S Laird-Fick + Show 2 more

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https://doi.org/10.1155/2013/872987

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Abstract

Autoimmune hepatitis (AIH) is an inflammatory condition of the liver that has a multitude of clinical presentations from chronic hepatitis to acute fulminant hepatitis. AIH diagnosis is typically suspected after ruling out other causes of hepatitis (such as vial hepatitis, hemochromatosis, Wilson's disease, and primary biliary cirrhosis) through serological tests and by findings of high titers of certain autoantibodies (ANA and anti-SMA for type 1 AIH and anti-LKM-1 for type 2 AIH). AIH like most inflammatory conditions is associated with increased ferritin levels (acute-phase reactant) but typically near-normal transferrin saturation. The presence of excessive ferritin level in absence of high-transferrin saturation helps differentiate secondary iron overload from hemochromatosis where transferrin saturation is typically high. We herein describe a case of AIH that presented with high ferritin levels and transferrin saturation suggesting a diagnosis of hemochromatosis and needed arduous work-up to arrive at accurate diagnosis of AIH.

Highlights

Autoimmune hepatitis (AIH) is an inflammatory condition of the liver that has a multitude of clinical presentations from chronic hepatitis to acute fulminant hepatitis [1, 2]
We present a challenging case of AIH with unusually high transferrin saturation (89%) that required arduous evaluation to arrive at final diagnosis
Many chronic liver diseases such as nonalcoholic fatty liver disease (NAFLD), chronic hepatitides B and C, primary biliary cirrhosis (PBC), and alpha-1 antitrypsin deficiency are associated with mild iron overload with near-normal transferrin saturation rarely exceeding 45% [3, 4]

Summary

Introduction

Autoimmune hepatitis (AIH) is an inflammatory condition of the liver that has a multitude of clinical presentations from chronic hepatitis to acute fulminant hepatitis [1, 2]. Typical symptoms are nonspecific and can include fatigue, jaundice, nausea, and abdominal pain. It is a relatively rare disease affecting women more than men (3.6 : 1 ration) with mean incidence of 1-2 per 100,000 persons per year and prevalence of 11–17 per 100,000 persons [1, 2]. We present a challenging case of AIH (type 1) with unusually high transferrin saturation (89%) that required arduous evaluation to arrive at final diagnosis

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