Idiopathic adulthood ductopenia

Idiopathic adult-onset ductopenia (IAD) is a very rare (<100 cases in the world literature) chronic cholestatic entity of unknown origin characterized by loss of interlobular bile ducts, first described two decades ago (Bilal M et al. 2016). Liver biopsies by definition show the phenomenon of "ductopenia" and its complications.

It is likely that IAD is a polyetiological syndrome. Causative factors are blamed on:

• Late-onset non-syndromic deficiency of intrahepatic bile ducts.
• Primary sclerosing cholangitis ( pericholangitis ) without involvement of a major duct, without signs of inflammatory bowel disease
• Non-suppurative viral cholangitis - for example with hepatitis C
• Autoimmune cholangitis or cholangitis in autoimmune hepatitis in the absence of typical autoantibodies (cryptogenic chronic hepatitis).

IAD most commonly affects young or middle-aged adults (total spectrum of affected patients, 15 to 77 years) and occurs with a marked male preponderance. The average age of male and female patients among 48 published and unpublished cases was 30 and 36 years, respectively (in 9 cases the exact age had not been reported).

Cholestatic profile; however, no changes that are diagnostic or suggestive of other biliary disease.

In approximately half of the published cases, the condition appeared to have a benign course; this contrasted with cases of IAD with a fatal course or requiring liver transplantation.

1. Bilal M et al (2016) Idiopathic Adulthood Ductopenia: 'It Is Out There'. Case Rep Gastroenterol 10:95-98.
2. Ludwig J (1998) Idiopathic adulthood ductopenia: an update. Mayo Clin Proc 73:285-291.
3. LaRusso NF (1988) Idiopathic adulthood ductopenia: a cause of chronic cholestatic liver disease and biliary cirrhosis. J Hepatol. 7: 193-199
4. Zafrani ES et al (1990) Idiopathic biliary ductopenia in adults: a report or five cases. Gastroenterology 99: 1823-1828