Idiopathic adulthood ductopenia

Last updated on: 18.12.2020

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Definition
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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.

Etiopathogenesis
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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).

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Manifestation
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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 The average age of male and female patients among 48 published and unpublished cases was 30 and 36 years, respectively (in 9 cases the exact age had not been reported).

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

Progression/forecast
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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.

Literature
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  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

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Last updated on: 18.12.2020