S2454 Eosinophilic Cholangitis – An Uncommon Cause of Elevated Liver Enzymes

Abstract

INTRODUCTION: Eosinophilic cholangitis (EC) is a rare disease with only a few cases reported. It is known to cause an obstructive pattern of abnormal liver function enzymes due to eosinophilic infiltration along the biliary channels. We present a case of a young woman who was found to have EC without biliary strictures on liver biopsy. CASE DESCRIPTION/METHODS: A 42 year old woman with history of hypertension presented with a rash of 1 week after eating salmon. Vitals were normal except for a heart rate of 120. Physical exam was notable for bilateral conjunctival injection, erythematous rash on face, chest, and upper extremities, desquamating rash on fingertips (Figure 1), tachycardia with regular rhythm, and a soft, non-tender abdomen with no hepatosplenomegaly. Labs on presentation were notable for a leukocytosis of 10.9 K/uL (neutrophil predominant), total bilirubin 3.8 mg/dL (direct 2.9), ALP 231 U/L, AST 102 U/L, ALT 237 U/L, GGT 314 U/L, ESR 126 U/L, and no eosinophilia. Abdominal ultrasound was unremarkable. Peripheral eosinophilia (0.63 K/uL) developed on day 3 of admission. Liver biopsy revealed severe acute cholangitis with numerous portal eosinophils consistent with eosinophilic cholangitis, without necrosis or fibrosis (Figure 2). Serologic work-up was negative for infectious or autoimmune etiology. Prednisone was started with improvement in liver enzymes and eosinophilia within 2 weeks. DISCUSSION: EC is a rare diagnosis typically associated with biliary strictures. However, no established diagnostic criteria exist. Our case shows biliary strictures are not always associated with EC, and peripheral eosinophilia may be absent on presentation. EC may be an overlapping condition with other causes of sclerosing cholangitis such as IgG4 related cholangiopathy and primary sclerosing cholangitis. Drug Reaction with Eosinophilia and Systemic Symptoms differs by having both a longer duration of liver involvement after initiation of prednisone and histologic features of cytotoxic phenotype with atypical lymphoid infiltrates and erythrophagocytosis. EC is often mistaken for cholangiocarcinoma given the radiographic similarities, with diagnosis established only after biopsy or surgical intervention. These patients may undergo unnecessary procedures for a condition that can be medically treated with high dose steroids. Thus, EC should be on the differential for a patient presenting with rash and acutely abnormal liver enzymes in a cholestatic or mixed pattern, with or without presence of biliary strictures.Figure 1.: Desquamating rash on fingertips.Figure 2.: (Percutaneous Liver Biopsy): Clinicopathologically consistent with eosinophilic cholangitis. A) dense portal inflammation rich in eosinophils, B) acute cholangitis with numerous portal eosinophils, and C) minimal lobular steatosis and no necrosis or fibrosis.