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Cited by 170Liver Diseases and ImmunityPediatric Hepatobiliary Diseases and TreatmentsIgG4-Related and Inflammatory Diseases

Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis

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Poupon, Raoul, Chazouilleres, Olivier, Corpechot, Christophe, Chrétien, Yves·2006·Hepatology
DOI 10.1002/hep.21229
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Abstract

Primary biliary cirrhosis (PBC)–autoimmune hepatitis (AIH) overlap syndrome is a clinical entity characterized by the occurence of both conditions at the same time in the same patient. In addition to PBC-AIH overlap syndrome, transitions from one autoimmune disease to another have been reported, but no systematic series have been published. We report a series of 12 patients with consecutive occurrence of PBC and AIH ( i.e. , PBC followed by AIH). Among 282 PBC patients, 39 were identified who fulfilled criteria for probable or definitive AIH. AIH developed in 12 patients (4.3%). The baseline characteristics of the patients were similar to those of patients with classical PBC. Time elapsed between the diagnosis of PBC and the diagnosis of AIH varied from 6 months to 13 years. Patients with multiple flares of hepatitis at the time of diagnosis of AIH had cirrhosis on liver biopsy. Ten patients were given prednisone ± azathioprine; short-term as well as sustained remissions were obtained in 8 of these, while two had multiple relapses and eventually died 8 and 7 years after diagnosis of AIH. In conclusion , the development of superimposed AIH could not be predicted from baseline characteristics and initial response to UDCA therapy. If not detected early, superimposed AIH can result in rapid progression toward cirrhosis and liver failure in PBC patients.

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  • The largest study found that only 8 of 1,476 patients with PBC later developed AIH.

    “while the largest study found that only 8 of 1,476 patients with PBC later developed AIH.(120)”

    From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [120]

    The Abstract and the ‘Frequency and Baseline Characteristics’ section report that 12 of 282 PBC patients developed AIH during follow-up, not 8 of 1,476. No page number is supplied for these passages in our copy. (LLM, conf=0.90)

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  • PBC patients with AIH features have a worse prognosis than those with PBC alone and tend to present with more advanced fibrosis.

    “The prognosis of patients with PBC and features of AIH is worse than for PBC alone and patients tend to present with more advanced fibrosis [160,173,174].”

    From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [160]

    Table 1 (Discussion; page unavailable) shows numerous fibrous septa or cirrhosis at PBC diagnosis in 34% of patients who later developed AIH and 41% with simultaneous PBC–AIH, versus 29% with PBC alone, consistent with a tendency toward more advanced fibrosis in those groups. The Abstract says superimposed AIH can cause rapid progression to cirrhosis and liver failure if not detected early, but the paper does not establish an overall worse prognosis for all patients with PBC and AIH features compared with PBC alone. (LLM, conf=0.85)

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  • PBC patients who meet the Paris criteria for AIH at presentation or during follow-up may benefit from additional immunosuppression in the short and medium term.

    “The available data suggest that patients who fulfil the Paris criteria for the diagnosis of AIH either at presentation or during the course of their PBC may benefit from additional immunosuppression in the short-and medium-term [160,162,175,176].”

    From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [160]

    The Development of AIH section reports short-term biochemical remission after prednisone in all 10 treated patients who developed AIH during PBC follow-up; the Abstract reports sustained remission in eight. The paper also identifies patients with simultaneous PBC–AIH, but does not report comparable treatment outcomes for that group, so the claimed benefit at presentation is not established by this study. Its Appendix gives the authors’ overlap criteria without naming them the Paris criteria. (LLM, conf=0.84)

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  • Two studies found that approximately 2.5% of PBC patients develop a subsequent acute AIH.

    “Two studies have found that approximately 2.5% of PBC patients develop a subsequent acute AIH, (119,121)”

    From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [121]

    The Results section (“Frequency and Baseline Characteristics,” p. not available) reports that 12 of 282 PBC patients (4.3%) developed AIH during follow-up, supporting the subsequent-development point. It does not support the cited figure of approximately 2.5%, and the quoted passage does not characterize the subsequent AIH as acute. (LLM, conf=0.88)

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  • PBC patients receiving UDCA can develop increasing hepatitis activity years after the initial PBC diagnosis.

    “PBC patients under UDCA treatment may develop increasing hepatitis activity even years after the initial PBC diagnosis was made [161,160].”

    From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [160]

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Cites

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Research works printed but not linked: 1

  1. [22]22. Kanzler S, Bozkurt S, Herkel J, Galle PR, Dienes HP, Lohse AW. Presence of SLA/LP autoantibodies in patients with primary biliary cirrhosis as a marker for secondary autoimmune hepatitis (overlap syndrome) [in Ger- man]. Dtsch Med Wochenschr 2001;126:450-456.

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Authors
Poupon, R., Chazouilleres, O., Corpechot, C., & Chrétien, Y.
Affiliations
Inserm (FR)
Corresponding author
Poupon, Raoul
ORCID
Venue
Hepatology
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2006-07
Volume
44
Issue
1
Pages
85-90
ISSN
0270-9139 (ISSN-L)
DOI
10.1002/hep.21229
OpenAlex
W2089926610
Semantic Scholar
a29e87e6d41ccebb82161240a19a15e0f098d815
Cited by
170
References
25
Citations by year
2026: 4 · 2025: 6 · 2024: 12 · 2023: 9 · 2022: 3 · 2021: 3 · 2020: 8 · 2019: 6 · 2018: 9 · 2017: 5 · 2016: 7 · 2015: 4 · 2014: 7 · 2013: 11 · 2012: 16
FWCI
7.67
Type
journal article
Language
en
Open access
Licence
doi.wiley.com/10.1002/tdm_license_1.1 (tdm, 3349 d)
Locations
  • Hepatology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
  • Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
  • Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
Keywords
primary biliary cirrhosis, autoimmune hepatitis, PBC-AIH overlap syndrome, disease progression, prednisone, azathioprine, ursodeoxycholic acid, liver failure, relapse
MeSH terms
Adult, Aged, Biopsy, Diagnosis, Differential, Female, Follow-Up Studies, France, Humans, Liver Cirrhosis, Biliary, Male, Middle Aged, Prognosis, Retrospective Studies, Incidence, Disease Progression, Hepatitis, Autoimmune

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