Eosinophilic Cholangiopathy

Case Presentation 52 year-old Caucasian man referred for cholestatic laboratory tests Thirty-two years earlier had frequent loose bowel movements – Dx: ulcerative colitis Biopsies showed multiple eosinophils in the colonic mucosa Symptoms resolved with oral olsalazine ERCP with biliary strictures - Dx: PSC

History Corticosteroid and ursodeoxycholic acid Rx with improvement in hepatic biochemical tests Occasional right upper quadrant abdominal pain Pruritus Fatigue Denied nausea, vomiting, jaundice, weight loss, or gastrointestinal bleeding

History PMHx: HTN, CAD PSHx: PTCA Meds: Metoprolol, olsalazine, ursodiol Allergies: Iodine Social Hx: 2 beers/month Married engineer Family Hx: No liver disease, CA, or IBD

Physical Examination General NAD HEENT No icterus CV RRR Chest Clear Abd +BS, soft, NT, ND Hepatomegaly. Spleen not palpable. No ascites Ext No C/C/E. No asterixis Skin No jaundice, telangiectasias, or palmar erythema

Laboratories WBC 18,900 cells/mm3 Eosinophil count 13,300 cells/mm3 AST 59 U/L ALT 88 U/L Alkaline phosphatase 648 U/L Negative viral and autoimmune markers PT 9.6 seconds aPTT 25 seconds Tbili 0.7 mg/dL Albumin 3.9 g/dL Total protein 6.7 g/dL CA 19-9 15 U/mL Stools negative for ova and parasites

Studies Abdominal ultrasound - no evidence of biliary dilation or gall stones MRCP - normal bile ducts and liver Bone marrow biopsy - no increased number of eosinophils or eosinophil progenitor cells

Liver Biopsy without Treatment

Dx: Hypereosinophilic Syndrome with Eosinophilic Cholangiopathy

Follow-up Rx: oral prednisone with reduction of aminotransferases and alkaline phosphatase Absolute eosinophil count normalized Maintenance treatment with azathioprine 100 mg once daily Prednisone 5 mg daily Ursodeoxycholic acid 1000mg BID AST 31 U/L ALT 39 U/LA Alkaline phosphatase steadily decreasing with serial values of 362; 200; and 170 U/L Peripheral eosinophilia resolved

Biopsy on Treatment