Evidence map›Paper›PMID 41124063›Full record

ArticleUnited European gastroenterology journal2025

Trends in Primary Biliary Cholangitis: Prospective Cohort Study From the European Reference Network Registry (R-LIVER).

A Gerussi, E Nofit, D P Bernasconi, C Kroll, F Groß, I Schregel, A Marini, L Cristoferi, F Malinverno, M Papp and 14 more

Erratum issuedAbstract readMulticenter Study
In one paragraph

Article in United European gastroenterology journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

24 authors.

A GerussiCentre for Autoimmune Liver Diseases and Division of Gastroenterology, European Reference Network (ERN) RARE-LIVER, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
E NofitCentre for Autoimmune Liver Diseases and Division of Gastroenterology, European Reference Network (ERN) RARE-LIVER, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
D P BernasconiDepartment of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.ORCID https://orcid.org/0000-0001-8771-5428
C KrollDepartment of Medicine, University Medical Centre Hamburg-Eppendorf (UKE), Hamburg, Germany.
F GroßDepartment of Medicine, University Medical Centre Hamburg-Eppendorf (UKE), Hamburg, Germany.
I SchregelDepartment of Medicine, University Medical Centre Hamburg-Eppendorf (UKE), Hamburg, Germany.
A MariniDepartment of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy.
L CristoferiCentre for Autoimmune Liver Diseases and Division of Gastroenterology, European Reference Network (ERN) RARE-LIVER, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.ORCID https://orcid.org/0000-0001-9846-7474
F MalinvernoCentre for Autoimmune Liver Diseases and Division of Gastroenterology, European Reference Network (ERN) RARE-LIVER, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
M PappEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.ORCID https://orcid.org/0000-0003-3662-4010
G DalekosEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.ORCID https://orcid.org/0000-0001-7075-8464
E RigopoulouEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.ORCID https://orcid.org/0000-0003-1978-4602
M K JanikEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.ORCID https://orcid.org/0000-0003-1941-0336
P MilkiewitzEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.
H YttingEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.ORCID https://orcid.org/0000-0001-5018-5232
A LeburgueEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.
F BraunDepartment of Medicine, University Medical Centre Hamburg-Eppendorf (UKE), Hamburg, Germany.
C CasarEuropean Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hamburg, Germany.ORCID https://orcid.org/0000-0002-8624-1045
M SebodeDepartment of Medicine, University Medical Centre Hamburg-Eppendorf (UKE), Hamburg, Germany.
A LohseDepartment of Medicine, University Medical Centre Hamburg-Eppendorf (UKE), Hamburg, Germany.ORCID https://orcid.org/0000-0002-8823-3129
B HansenDepartment of Epidemiology & Biostatistics, Erasmus MC, Rotterdam, the Netherlands.
M CarboneCentre for Autoimmune Liver Diseases and Division of Gastroenterology, European Reference Network (ERN) RARE-LIVER, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.ORCID https://orcid.org/0000-0003-1445-0443
C SchrammDepartment of Medicine, University Medical Centre Hamburg-Eppendorf (UKE), Hamburg, Germany.ORCID https://orcid.org/0000-0002-4264-1928
P InvernizziCentre for Autoimmune Liver Diseases and Division of Gastroenterology, European Reference Network (ERN) RARE-LIVER, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.ORCID https://orcid.org/0000-0003-3262-1998

Funding

R-LIVER registry 945761
6 · The paper itself

Abstract

BACKGROUND AND

aimsThe European Reference Network on Hepatological Diseases (ERN RARE-LIVER) is a Europe-wide network for centers of excellence in the management of rare liver diseases. We aimed to evaluate the current diagnostic and therapeutic trends of primary biliary cholangitis (PBC).

methodsProspective data of PBC cases diagnosed from 2017 to March 2024 were extracted from the R-LIVER registry of ERN-RARE LIVER. Cases without two follow-ups within 24 months were excluded from the treatment analysis. Biochemical response according to Toronto criteria and normalization of alkaline phosphatase (ALP) values after 12 months of Ursodeoxycholic Acid (UDCA) were evaluated.

resultsThis study included 327 incident cases from six centers. Median age was 56 years, 89.3% were female. At the time of diagnosis, median values of ALP were 1.37 x ULN, and median bilirubin was 0.49 x ULN. Transient elastography (TE) was performed in 230 patients (70.3%) at baseline; median liver stiffness was 6.2 kPa. Out of 316 subjects, treatment with UDCA was started in 312 patients (98.7%); 246 (85.1%) achieved ALP values < 1.67 x ULN at 12 months. Normalization of ALP values occurred in 143 subjects (49.5%) at 12 months. Among 43 patients with inadequate response, 18 (41.9%) were treated with second-line therapies, and had worse liver biochemistry at baseline.

conclusionIn the current era, patients with PBC are diagnosed at an early stage using non-invasive methods and are almost all treated with UDCA. The biochemical response rate is 85.1%, but the use of second-line therapies for inadequate responders remains suboptimal.

Indexed as

Cholagogues and CholereticsLiver Cirrhosis, BiliaryUrsodeoxycholic AcidAdultAgedAlkaline PhosphataseElasticity Imaging TechniquesEuropeFemaleHumansLiverMaleMiddle AgedProspective StudiesRegistriesTreatment OutcomeAlkaline PhosphataseCholagogues and CholereticsUrsodeoxycholic AcidautoimmunitycholestasisepidemiologyliverUDCA

Identifiers

PMID41124063
PMCPMC12704578

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.