vanishing bile duct syndrome (idiopathic ductopenia)

Introduction

Disorder resembling graft versus host disease (GVHD) & liver transplant rejection.

Etiology

• autoimmune is suggested
• occurs in patients with Hodgkin's disease
• occurs as hepatotoxic effect of:
  • chlorpromazine
  • other phenothiazines
  • other medications

Pathology

• paucity of bile ducts in the portal areas

Laboratory

• mildly elevated transaminases
• increased alkaline phosphatase
• increased gamma-glutamultransferase (GGT)
• elevated bilirubin
• liver biopsy

Radiology

• cholangiography is normal

More general terms

• cholangitis