Cholestatic Hepatitis in a Patient With Eosinophilic Granulomatosis and Polyangiitis

Abstract

Introduction: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a multisystem disorder, commonly treated with chronic steroid therapy, characterized by asthma, chronic rhinosinusitis, and prominent eosinophilia. EGPA can involve any organ system, lungs being most common. Eosinophilic gastroenteritis is the most common gastrointestinal involvement, typically presenting with abdominal pain and diarrhea. Liver/biliary involvement in EGPA is reported but rare. The case below is a patient with EGPA who has had a several year history of RUQ abdominal pain, intermittent elevated LFTs, and jaundice and further evaluation revealed EGPA-related cholestatic hepatitis by liver biopsy. Case Description: The patient is a 57 y/o male with a history of EGPA on chronic steroids, GERD, and gallstone pancreatitis that has had several admissions since 2009 for chronic RUQ pain, nausea, elevated LFTs and jaundice. He underwent cholecystectomy in 2009 with liver biopsy at that time which revealed portal and periportal hepatitis as well as centrilobular cholestasis. On his most recent presentation, it was thought he had choledocholithiasis and possible cholangitis. His bilirubin remained elevated despite an ERCP that was done with extraction of small stones and sludge. He underwent a second ERCP with sludge extraction and common bile duct stent placement but his bilirubin remained elevated. Chronic liver disease markers were negative. Given his previous biopsy results, another liver biopsy was obtained and demonstrated liver parenchyma with cholestasis, predominantly in pericentral zones and accompanying mild to moderate mixed acute and chronic lobular inflammation with evidence of hepatocyte damage. This is consistent with a cholestatic hepatitis seen in patients with Churg-Strauss Syndrome/EGPA. His steroids were increased to 60mg daily for 1 month. Weekly labs were obtained and his liver chemistries normalized over two months. His prednisone was tapered down to his original dose of 20mg daily. Discussion: Liver involvement in EGPA is rare but should be considered in patients with a history of EGPA who present with cholestasis. Better control of EGPA with immunosuppression has shown significant improvement in these cases.Figure 1Table 1: Liver Function Tests Before and After Treatment Course