1. Eosinophilic Cholangiopathy

2. 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

3. 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

4. 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

5. 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

6. 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 U/mL
Stools negative for ova and parasites

7. 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

8. Liver Biopsy without Treatment

9. Dx: Hypereosinophilic Syndrome with Eosinophilic Cholangiopathy

10. 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

11. Biopsy on Treatment