Jaundice in non-cirrhotic primary biliary cirrhosis: the premature ductopenic variant
Open accessAbstract
The clinical and pathological findings of four females with primary biliary cirrhosis (PBC) with an unusual and hitherto not well recognised course are reported. Patients suffered severe pruritus and weight loss with progressive icteric cholestasis which did not respond to such treatments as ursodeoxycholic acid and immunosuppressives. In all cases liver histology revealed marked bile duct loss without however significant fibrosis or cirrhosis. Further diagnostic studies and repeat biopsies confirmed the absence of liver cirrhosis as well as other potential causes of hyperbilirubinaemia. Comparison of the fibrosis-ductopenia relationship for our cases with that for a group of 101 non-cirrhotic PBC patients indicated that in the former the severity of bile duct loss relative to the amount of fibrosis was significantly higher. The proportion of portal triads containing an interlobular bile duct was 3%, 4%, 6%, and 10% compared with 45% (median; range 8.3–100%) for controls (p<0.001). Three patients received a liver transplant 6–7 years after the first manifestation of PBC because of progressive cholestasis, refractory pruritus, and weight loss, while the fourth patient is considering this option. In one case cirrhosis had developed at the time of transplantation while the others still had non-cirrhotic disease. These cases suggest that cholestatic jaundice in non-cirrhotic PBC may be secondary to extensive “premature” or accelerated intrahepatic bile duct loss. Although the extent of fibrosis may be limited initially, progression to cirrhosis appears to be inevitable in the long run. Despite intact protein synthesis and absence of cirrhotic complications, liver transplantation in the pre-cirrhotic stage for preventing malnutrition and to improve quality of life should be considered for these patients.
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A premature ductopenic variant of PBC has been described in which severe pruritus is associated with progressive icteric cholestasis and is not responsive to UDCA.
“A premature ductopenic variant of PBC has been described, in which severe pruritus is associated with progressive icteric cholestasis, and is not responsive to UDCA.”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [68]
In this ductopenic variant, histology reveals bile duct loss without significant fibrosis or cirrhosis, and patients usually progress to needing transplantation.
“Histology reveals bile duct loss without significant fibrosis or cirrhosis, and these patients usually progress to needing transplantation [68].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [68]
Patients with the ductopenic variant of PBC have particular problems with itch.
“Patients with the ductopenic variant of PBC have particular problems with itch [68].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [68]
Patients with the ductopenic variant of PBC have particular problems with itch.
“Patients with the ductopenic variant of PBC have particular problems with itch.127”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [127]
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Chapter 12 Primary Biliary Cirrhosis
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Stratified medicine and primary biliary cholangitis
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Acute hepatitis E unmasking primary biliary cholangitis and Takayasu aortoarteritis in a patient
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Colangite (ex cirrosi) biliare primitiva
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- Authors
- Vleggaar, F. P., van Buuren, H. R., Zondervan, P. E., ten Kate, F. J. W., Hop, W. C. J., & the Dutch Multicentre PBC study group
- Affiliations
- Erasmus MC (NL)
- aDepartment of Gastroenterology and Hepatology, University Hospital Rotterdam, the Netherlands, bDepartment of Pathology, University Hospital Rotterdam, the Netherlands, cDepartment of Pathology, Academic Medical Centre, Amsterdam, the Netherlands, dDepartment of Epidemiology and Biostatistics, Erasmus University Rotterdam, the Netherlands
- ORCID
- Vleggaar, F P
- Venue
- Gut
- Publisher
- BMJ
- Published
- 2001-08-01
- Volume
- 49
- Issue
- 2
- Pages
- 276-281
- ISSN
- 0017-5749 (ISSN-L)
- 1468-3288
- PMC
- PMC1728410
- OpenAlex
- W2142917855
- Semantic Scholar
- a63f19291cbc70595c985826b304b06ace3e0387
- Cited by
- 87
- References
- 28
- Citations by year
- 2026: 3 · 2025: 2 · 2024: 5 · 2023: 3 · 2022: 5 · 2021: 2 · 2020: 2 · 2019: 8 · 2018: 9 · 2017: 3 · 2016: 4 · 2015: 3 · 2014: 4 · 2013: 2 · 2012: 2
- FWCI
- 1.99
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Gut (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion, other-oa)
- UvA-DARE (University of Amsterdam) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion, other-oa)
- RePub (Erasmus University Rotterdam) (submittedVersion, other-oa)
- HighWire Press Open Archive (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion, other-oa)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Hepatology › Liver Disease and Transplantation
- Keywords
- primary biliary cirrhosis, cholestatic jaundice, bile duct loss, liver transplantation, pruritus, ursodeoxycholic acid, interlobular bile ducts, hyperbilirubinemia, disease progression
- MeSH terms
- Adult, Aged, Biopsy, Cholestasis, Intrahepatic, Female, Humans, Liver Cirrhosis, Biliary, Male, Middle Aged, Pruritus, Weight Loss, Case-Control Studies, Liver Transplantation, Endoscopy, Gastrointestinal, Disease Progression
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