Idiopathic Adulthood Ductopenia: 'It Is Out There'

Abstract

Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic entity of unknown origin characterized by loss of inter-lobular bile ducts that was first described two decades ago. Although the diagnostic criteria have been described in detail, IAD continues to be a rare diagnosis. Our thorough literature search revealed less than a hundred cases of IAD reported. Here we present a 34-year-old female with no significant past history who was evaluated for persistent elevation of serum alkaline phosphate levels. Serology was negative for all viral hepatitides, and a chronic liver disease workup was unremarkable. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography did not reveal any abnormalities in the biliary tree. Finally, a liver biopsy demonstrated ductopenia involving greater than 50% of the portal triads, making a diagnosis of IAD. Since the disease can progress rapidly, close follow-up is warranted, so liver transplantation can be pursued if deemed necessary.

Vanishing Bile Duct Syndrome in a Patient with Uterine Cancer and Paraneoplastic Systemic Sclerosis

“…Because etiologies of VBDS are often related to immunologic causes, immunosuppression has been proposed as a potential treatment; however there is minimal evidence supporting its use 18. Patients with severe progression with liver decompensation should be considered for liver transplantation because there are case reports of successful transplantation without recurrence 19 , 20…”

Idiopathic adulthood ductopenia with elevated transaminase only: A case report

“…IAD was first described in 1988 by Ludwig et al [ 1 ], with interlobular bile ducts disappearing without a clear origin[ 2 ]. So far, less than 100 cases have been reported[ 3 ]. In recent years, the pathology and pathogenesis of bile duct loss have been widely studied, and a number of hepatobiliary diseases have been added to the list of ductopenic diseases[ 4 ].…”