John S. Herndon, MD1, Swaroop Vitta, MD2, Frederick H. Weber, Jr., MD3, Deepti Dhall, MD3; 1University of Alabama, Birmingham, AL; 2University of Alabama School of Medicine, Birmingham, AL; 3UAB, Birmingham, AL
Introduction: This case highlights a rare, important and reversible diagnostic consideration for eosinophilia in gastrointestinal and liver disease.
Methods: A 27 year old female of Asian Indian descent with no significant medical history and on no medications was referred for 2 months of bloody diarrhea associated with lower abdominal cramping and 10-lb weight loss. Clinical exam was unremarkable. Laboratory studies demonstrated an alkaline phosphatase (AP) of 221 U/L, ALT of 55 U/L, AST of 41 U/L, normal bilirubin, leukocyte count 28 X 103/cmm (34% eosinophils)and elevated fecal leukocytes, ESR and CRP. Colonoscopy and biopsies were consistent with chronic active ulcerative pancolitis with no increased tissue eosinophilia. After lack of response to mesalamine and budesonide, she had a complete clinical, endoscopic, and histologic response to vedolizumab but the eosinophilic leukocytosis worsened reaching a peak WBC of 40 x 103/cmm (46% eosinophils) and cholestatic liver function tests worsened with peak AP 500, AST 135, ALT 290, with normal bilirubin. Extensive infectious and parasitic investigations were negative. Flow cytometry of blood and bone marrow along with a bone marrow biopsy showed increased eosinophils but no evidence of a malignant process. MRCP was notable for subtle beading of the right and left intrahepatic biliary ducts suggestive of small duct PSC (Figure A). Liver biopsy (Figure B) showed moderately active lobular inflammation with prominent eosinophilic infiltrates and mild ductal injury. The patient was started on prednisone and had normalization of eosinophilic leukocytosis and liver function tests confirming the diagnosis of eosinophilic cholangitis (EC). Discussion: Approaches to diagnosis and management of EC are limited to case reports typically noting the following diagnostic findings: biliary obstruction or ductal thickening, histopathological eosinophilic infiltration, and biliary abnormalities that resolve without intervention or following corticosteroid therapy. Our case is the first to describe a possible association between EC and UC given the concomitant presentations temporally. EC is a rare but reversible condition, without a known cause, that can mimic PSC and is an important consideration for patients with evidence of biliary abnormalities associated with peripheral eosinophilia.
MRCP image with mild beading of the left hepatic duct
Moderately active hepatitis with prominent eosinophilic infiltrate and mild duct injury
Disclosures: John Herndon indicated no relevant financial relationships. Swaroop Vitta indicated no relevant financial relationships. Frederick Weber indicated no relevant financial relationships. Deepti Dhall indicated no relevant financial relationships.