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Colangite biliar primária
ORPHA:186CID-10 · K74.3CID-11 · DB96.1PCDT · SUSDOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
DigestivoInício adolescenteHerança Complex/Unknown
Também conhecida comoSíndrome HanotCirrose biliar primáriaPBCCBP
Sinônimos clínicos: Síndrome Hanot · Cirrose biliar primária

A colangite biliar primária (CBP) é uma doença crônica e de progressão lenta do fígado, causada por um problema autoimune. Ela se caracteriza por um dano nos pequenos canais que transportam a bile dentro do fígado, o que pode, com o tempo, levar à falência do fígado.

Última verificação: Fonte: verificadoEm construçãohistóricoSugerir correção
Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

💬
EM LINGUAGEM SIMPLES

A colangite biliar primária é uma doença autoimune e crônica do fígado em que os pequenos canais responsáveis pelo transporte da bile sofrem lesões progressivas. Com frequência, a pessoa percebe uma sensação constante de cansaço (fadiga), pele ou olhos amarelados (icterícia) e escurecimento cutâneo. O diagnóstico costuma ser identificado por meio de enzimas hepáticas aumentadas e pela presença do anticorpo antimitocondrial no sangue. No Brasil, o SUS dispensa o medicamento ácido ursodesoxicólico em comprimidos de 50 mg, 150 mg e 300 mg para o tratamento da doença por meio de seu protocolo clínico oficial.

📋
Informacoes curadas por IA — podem conter imprecisoes

A colangite biliar primária (CBP) é uma doença crônica e de progressão lenta do fígado, causada por um problema autoimune. Ela se caracteriza por um dano nos pequenos canais que transportam a bile dentro do fígado, o que pode, com o tempo, levar à falência do fígado.

Pesquisas ativas
57 ensaios
209 total registrados no ClinicalTrials.gov
Publicações científicas
2.941 artigos
Último publicado: 2026 Apr 15
Medicamentos
2 com registro
Ursodiol (URSODIOL), Livdelzi (SELADELPAR LYSINE)

Tem tratamento?

✓ 2 medicamentos específicos desta doença (registro ANVISA, FDA ou SUS/CEAF)
Ver detalhes, fases e interações →
URSODIOL (URSODIOL)LIVDELZI (SELADELPAR LYSINE)

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
21.05
Worldwide
Início
Adolescent
+ adult, elderly
🏥
SUS: Cobertura parcialScore: 0%
PCDT disponívelCID-10: K74.3
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
12 sintomas
🧬
Pele e cabelo
2 sintomas
🛡️
Imunológico
2 sintomas
👂
Ouvidos
1 sintomas
🦴
Ossos e articulações
1 sintomas
🧠
Neurológico
1 sintomas

+ 20 sintomas em outras categorias

Características mais comuns

90%prev.
Positividade do anticorpo antimitochondrial
Muito frequente (99-80%)
90%prev.
Cirrose
Muito frequente (99-80%)
90%prev.
Hiperpigmentação da pele
Muito frequente (99-80%)
90%prev.
Cirrose biliar
Muito frequente (99-80%)
90%prev.
Urticária dermatográfica
Muito frequente (99-80%)
90%prev.
Hiperbilirrubinemia conjugada
Muito frequente (99-80%)
41sintomas
Muito frequente (6)
Frequente (20)
Ocasional (14)
Muito raro (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 41 características clínicas mais associadas, ordenadas por frequência.

Positividade do anticorpo antimitochondrialAntimitochondrial antibody positivity
Muito frequente (99-80%)90%
CirroseCirrhosis
Muito frequente (99-80%)90%
Hiperpigmentação da peleHyperpigmentation of the skin
Muito frequente (99-80%)90%
Cirrose biliarBiliary cirrhosis
Muito frequente (99-80%)90%
Urticária dermatográficaDermatographic urticaria
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.941PubMed
Últimos 10 anos200publicações
Pico2026103 papers
Linha do tempo
2026Hoje · 2026🧪 1989Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

8 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Unknown.

IRF5Interferon regulatory factor 5Major susceptibility factor inTolerante
VIAS BIOLÓGICAS (3)
Interferon gamma signalingInterferon alpha/beta signalingSLC15A4:TASL-dependent IRF5 activation
MECANISMO DE DOENÇA

Inflammatory bowel disease 14

A chronic, relapsing inflammation of the gastrointestinal tract with a complex etiology. It is subdivided into Crohn disease and ulcerative colitis phenotypes. Crohn disease may affect any part of the gastrointestinal tract from the mouth to the anus, but most frequently it involves the terminal ileum and colon. Bowel inflammation is transmural and discontinuous; it may contain granulomas or be associated with intestinal or perianal fistulas. In contrast, in ulcerative colitis, the inflammation is continuous and limited to rectal and colonic mucosal layers; fistulas and granulomas are not observed. Both diseases include extraintestinal inflammation of the skin, eyes, or joints.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
112.8 TPM
Baço
39.3 TPM
Pulmão
19.5 TPM
Rim - Medula
19.1 TPM
Sangue
18.6 TPM
OUTRAS DOENÇAS (6)
systemic lupus erythematosusprimary biliary cholangitisdiffuse cutaneous systemic sclerosislimited cutaneous systemic sclerosis
HGNC:6120UniProt:Q13568
IL12RB1Interleukin-12 receptor subunit beta-1Major susceptibility factor inTolerante
VIAS BIOLÓGICAS (2)
Interleukin-12 signalingInterleukin-23 signaling
MECANISMO DE DOENÇA

Immunodeficiency 30

A form of Mendelian susceptibility to mycobacterial disease, a rare condition caused by impairment of interferon-gamma mediated immunity. It is characterized by predisposition to illness caused by moderately virulent mycobacterial species, such as Bacillus Calmette-Guerin (BCG) vaccine, environmental non-tuberculous mycobacteria, and by the more virulent Mycobacterium tuberculosis. Other microorganisms rarely cause severe clinical disease in individuals with susceptibility to mycobacterial infections, with the exception of Salmonella which infects less than 50% of these individuals. Clinical outcome severity depends on the degree of impairment of interferon-gamma mediated immunity. Some patients die of overwhelming mycobacterial disease with lepromatous-like lesions in early childhood, whereas others develop, later in life, disseminated but curable infections with tuberculoid granulomas. IMD30 has low penetrance, and affected individuals have relatively mild disease and good prognosis. BCG disease and salmonellosis are the most frequent infections in IMD30 patients.

EXPRESSÃO TECIDUAL(Tecido-específico)
Baço
17.5 TPM
Linfócitos
16.6 TPM
Sangue
9.8 TPM
Intestino delgado
5.0 TPM
Pulmão
4.6 TPM
OUTRAS DOENÇAS (2)
Mendelian susceptibility to mycobacterial diseases due to complete IL12RB1 deficiencyprimary biliary cholangitis
HGNC:5971UniProt:P42701
IL12AInterleukin-12 subunit alphaMajor susceptibility factor inTolerante
VIAS BIOLÓGICAS (4)
Interleukin-10 signalingInterleukin-4 and Interleukin-13 signalingInterleukin-12 signalingInterleukin-35 Signalling
EXPRESSÃO TECIDUAL(Tecido-específico)
Linfócitos
7.4 TPM
Esôfago - Mucosa
6.6 TPM
Baço
3.5 TPM
Pulmão
2.9 TPM
Fibroblastos
2.9 TPM
OUTRAS DOENÇAS (2)
Behcet diseaseprimary biliary cholangitis
HGNC:5969UniProt:P29459
TNPO3Transportin-3Major susceptibility factor inTolerante
MECANISMO DE DOENÇA

Muscular dystrophy, limb-girdle, autosomal dominant 2

An autosomal dominant myopathy characterized by proximal muscle weakness primarily affecting the lower limbs, but also affecting the upper limbs in most patients. Affected individuals also have distal muscle weakness of the hands and lower leg muscles. The disease has generally a benign clinical course but some individuals with childhood or juvenile onset manifest severe widespread myopathy, leading to wheelchair dependency and respiratory insufficiency. Muscle biopsy shows dystrophic changes with abnormal nuclei, rimmed vacuoles, and filamentous inclusions.

EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
79.5 TPM
Fibroblastos
47.6 TPM
Testículo
40.2 TPM
Cérebro - Hemisfério cerebelar
35.1 TPM
Ovário
32.1 TPM
OUTRAS DOENÇAS (2)
autosomal dominant limb-girdle muscular dystrophy type 1Fprimary biliary cholangitis
HGNC:17103UniProt:Q9Y5L0
SPIBTranscription factor Spi-BMajor susceptibility factor inAltamente restrito
EXPRESSÃO TECIDUAL(Tecido-específico)
Linfócitos
78.1 TPM
Baço
21.9 TPM
Intestino delgado
15.6 TPM
Cólon transverso
13.3 TPM
Sangue
3.1 TPM
OUTRAS DOENÇAS (1)
primary biliary cholangitis
HGNC:11242UniProt:Q01892
POU2AF1POU domain class 2-associating factor 1Major susceptibility factor inTolerante
EXPRESSÃO TECIDUAL(Tecido-específico)
Linfócitos
264.3 TPM
Baço
63.5 TPM
Intestino delgado
17.4 TPM
Glândula salivar
9.7 TPM
Estômago
6.7 TPM
OUTRAS DOENÇAS (1)
primary biliary cholangitis
HGNC:9211UniProt:Q16633
MMEL1Membrane metallo-endopeptidase-like 1Major susceptibility factor inTolerante
EXPRESSÃO TECIDUAL(Tecido-específico)
Testículo
35.0 TPM
Intestino delgado
1.5 TPM
Pulmão
1.5 TPM
Estômago
1.4 TPM
Hipotálamo
1.4 TPM
OUTRAS DOENÇAS (1)
primary biliary cholangitis
HGNC:14668UniProt:Q495T6
TNFSF15Tumor necrosis factor ligand superfamily member 15Major susceptibility factor inTolerante
VIAS BIOLÓGICAS (1)
TNFs bind their physiological receptors
EXPRESSÃO TECIDUAL(Baixa expressão)
Glândula salivar
4.9 TPM
Rim - Medula
4.3 TPM
Próstata
4.2 TPM
Cólon sigmoide
3.2 TPM
Pulmão
2.2 TPM
OUTRAS DOENÇAS (1)
primary biliary cholangitis
HGNC:11931UniProt:O95150

Medicamentos aprovados (FDA)

2 medicamentos encontrados nos registros da FDA americana.

💊 Ursodiol (URSODIOL)
💊 Livdelzi (SELADELPAR LYSINE)
Ver no DailyMed/FDA

Variantes genéticas (ClinVar)

162 variantes patogênicas registradas no ClinVar.

🧬 TNFSF15: GRCh37/hg19 9p24.3-q34.3(chr9:203861-141020389)x3 ()
🧬 TNFSF15: GRCh37/hg19 9p24.3-q34.11(chr9:203861-131603223)x3 ()
🧬 TNFSF15: GRCh37/hg19 9q32-34.11(chr9:116422275-131713233) ()
🧬 TNFSF15: GRCh37/hg19 9q31.2-32(chr9:109265628-117650999) ()
🧬 TNFSF15: GRCh37/hg19 9q31.1-33.3(chr9:104604851-126253089) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 1 variantes classificadas pelo ClinVar.

1
Patogênica (100.0%)
VARIANTES MAIS SIGNIFICATIVAS
TJP2: NM_004817.4(TJP2):c.3371C>T (p.Thr1124Met) [Conflicting classifications of pathogenicity]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
✓Aprovado2
3Fase 326
2Fase 218
1Fase 112
·Pré-clínico42
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 100 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Colangite biliar primária

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

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🟢 Recrutando agora

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Publicações mais relevantes

Timeline de publicações
2.942 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 2.942

#1

Anti-MRPS27 Antibody: A Novel Diagnostic Biomarker for Autoimmune Hepatitis Identified via a Human Proteome Microarray.

Hepatology research : the official journal of the Japan Society of Hepatology2026 Mar 23

To identify autoimmune hepatitis (AIH)-specific autoantibodies using a high-content human protein microarray and evaluate their diagnostic performance. Pretreatment sera from untreated patients with AIH (n = 68), primary biliary cholangitis (PBC, n = 19), metabolic dysfunction-associated steatotic liver disease (MASLD, n = 39), chronic hepatitis C (CHC, n = 27), and drug-induced liver injury (DILI, n = 18) and healthy controls (HC, n = 37) were screened with a wheat-germ, cell-free microarray displaying > 16,000 proteins. Candidate antigens were validated by ELISA, and antibody titers were correlated with clinical variables, treatment response, and outcomes. Mitochondrial ribosomal protein S27 (MRPS27) emerged as the dominant AIH-associated antigen. The mean anti-MRPS27 antibody titer in untreated patients with AIH (50.2 AU) exceeded that in patients with PBC (9.3 AU), MASLD (12.8 AU), CHC (24.8 AU), DILI (19.8 AU), and HC (7.5 AU, all p < 0.01). A cutoff of 35.4 AU (HC mean + 2 SD) yielded a sensitivity of 61.8%, a specificity of 87.1%, a positive predictive value of 70.0%, and a negative predictive value of 82.4%. Positivity was unrelated to baseline histology, biochemistry or IgG, and titers were unchanged after corticosteroid induction. Interestingly, patients with low titers experienced more liver-related events during follow-up. The anti-MRPS27 antibody is a promising adjunctive serological marker that enhances the specificity of AIH diagnosis. The pathogenic significance and prognostic value of this antibody warrant prospective validation.

#2

Geographic distribution and demographic patterns of primary biliary cholangitis and autoimmune hepatitis in Colombia: a national population-based administrative study (2018-2024).

Annals of gastroenterology2026

Autoimmune liver diseases, including primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH), are rare chronic conditions with significant morbidity. However, population-based epidemiological data from Latin America remain limited. This study aimed to describe the geographic distribution and demographic patterns of PBC and AIH in Colombia between 2018 and 2024. A descriptive, cross-sectional population-based study was conducted using data from the Comprehensive Social Protection Information System (SISPRO) of the Colombian Ministry of Health. Individuals with at least 1 recorded diagnosis coded as K74.3 (PBC) or K75.4 (AIH) according to the International Classification of Diseases, 10th Revision (ICD-10), were identified. Administrative prevalence estimates were calculated per 100,000 population, using official population projections from the National Administrative Department of Statistics (DANE). Age- and sex-specific distributions were analyzed, and geographic variation was visualized using choropleth maps at the regional level. A total of 6504 PBC cases and 11,225 AIH cases were identified. The estimated national administrative prevalence was 14.7 per 100,000 population for PBC and 17.0 per 100,000 for AIH. Both conditions showed marked female predominance. PBC was more frequent in women aged 50-69 years. However, AIH demonstrated a broader age distribution, affecting individuals from young adulthood to older age. Higher prevalence estimates were observed in multiple northern regions, particularly in the Caribbean region. This first nationwide administrative assessment of PBC and AIH in Colombia demonstrates marked sex differences and regional variation, highlighting the need to improve diagnostic awareness and strengthen epidemiological surveillance of autoimmune liver diseases in Latin America.

#3

Consensus statements of the Hellenic Autoimmune Liver Diseases Study Group on the diagnosis and current management of primary biliary cholangitis.

Annals of gastroenterology2026

Primary biliary cholangitis (PBC) is an autoimmune epithelitis of small intrahepatic bile ducts that affects predominately females, and is characterized by chronic cholestasis, circulating PBC-related autoantibodies, and progressive disease at the histological level. Key manifestations include pruritus, fatigue, hyperpigmentation, dry-gland syndrome, xanthelasmas and frequent concurrent extrahepatic autoimmune diseases, although approximately half the patients are nowadays completely asymptomatic at diagnosis. The current Consensus Statements of the Hellenic Autoimmune Liver Diseases Study Group aim to provide updated and practical statements to clinicians for PBC diagnosis and management. The presence of antimitochondrial antibodies is a key diagnostic marker for PBC. PBC-specific antinuclear antibodies (anti-gp210 and anti-sp100) also bear diagnostic and prognostic significance. Following diagnosis, this document provides guidance on the comprehensive assessment and risk stratification of patients, using demographic factors, clinical and biochemical laboratory findings, liver autoimmune serology and fibrosis stage. After 6-12 months of therapy with first-line treatment (13-15 mg/kg/day ursodeoxycholic acid [UDCA]), a new risk-stratification procedure should be performed, based on the assessment of biochemical response using a continuous scoring system (either GLOBE or UK-PBC score). In non-responders, add-on treatment to UDCA with a second-line agent, a proliferator-activated receptor agonist (PPAR), either elafibranor (PPARα/δ agonist) or seladelpar (PPARδ agonist), is recommended. The treatment target-also known as deep response-should aim to achieve bilirubin within the normal range, specifically at values <0.6× upper limit of normal, along with normalization of alkaline phosphatase. The disease-associated major symptoms (pruritus, fatigue and cognitive dysfunction) should also be promptly recognized and managed in a holistic manner, as they negatively affect the patient's health-related quality of life.

#4

Liver transplant outcomes in primary biliary cholangitis and metabolic dysfunction-associated steatotic liver disease overlap.

Annals of hepatology2026 Mar 12

Primary biliary cholangitis (PBC) frequently overlaps with metabolic dysfunction-associated steatotic liver disease (MASLD). The impact of concurrent MASLD on liver transplant (LT) in PBC remains unclear. This study compared pre- and post-LT outcomes between PBC with and without MASLD. We conducted a retrospective study using the UNOS/OPTN database to compare adult LT candidates with PBC or concomitant PBC/MASLD from 2002 to 2024. Nearest neighbor 1:1 propensity matching by multiple variables ensured cohort comparability. Outcomes included transplant probability, waitlist dropout, and post-LT patient and graft survival, analyzed with Kaplan-Meier and adjusted Cox regression. Before matching, PBC/MASLD had higher BMI (pre-transplant: 31.9 vs 26.8; post-transplant: 32.0 vs 26.6) and more diabetes (pre-transplant: 45% vs 15%; post-transplant: 42% vs 14%) compared to PBC-only. After matching (215 pre- and 151 post-transplant), PBC-only showed higher waitlist dropout (p = 0.005). Post-transplant patient and graft survival rates were similar between groups. Among PBC/MASLD, diabetes was associated with significantly lower long-term patient survival (10-year survival: 46% vs 76% in PBC/MASLD without diabetes, p = 0.01). On multivariate analysis, PBC/MASLD with diabetes remained the strongest independent predictor of post-transplant mortality (aHR 2.31, p = 0.003), followed by MELD score (aHR 1.03 per point, p = 0.02). PBC is associated with increased waitlist dropout compared to PBC/MASLD. Post-LT survival is comparable between groups unless diabetes is present, which significantly impairs long-term outcomes in PBC/MASLD. Prioritized diabetes screening and metabolic management post-LT is essential in this group. Future research should investigate factors affecting waitlist dropout and optimal glycemic control strategies in PBC patients.

#5

Mast Cells at the Crossroad of Gut-Derived Signals Through Aryl Hydrocarbon Receptor Activation: A Microbial-Immune Dialogue in Liver Inflammation with Therapeutic Perspectives.

Cells2026 Mar 03

Mast cells (MCs) are multifunctional innate immune cells that regulate inflammation, tissue repair, and immune responses, and they are increasingly recognized as contributors to chronic liver disease. In parallel, the aryl hydrocarbon receptor (AhR) has emerged as a key environmental sensor activated by gut-derived tryptophan metabolites such as kynurenine and microbial indoles. The current literature separately describes the role of AhR in MC signaling, as well as the contributions of MCs to liver pathology and the disrupted gut-liver axis, which drives immune dysfunction in chronic liver disease. However, these aspects have been rarely considered together. This review aims to bridge these fragmented areas, providing an integrated framework where AhR-driven MC responses are examined within the gut-liver axis along with their impacts on liver inflammation and fibrosis. We discuss how this microbial-immune dialogue shapes autoimmune and cholestatic liver diseases, including autoimmune hepatitis, primary sclerosing cholangitis, and primary biliary cholangitis. Finally, we highlight translational perspectives, from microbiota modulation to AhR-targeting approaches, as potential strategies to control MC-driven hepatic inflammation. By integrating these currently separate concepts, this review offers a novel perspective on the role of MCs as important mediators at the interface of gut-derived signals and liver pathology via AhR signaling, while highlighting innovative therapeutic avenues through the modulation of the microbiota, targeting of AhR, and regulation of MC responses.

Publicações recentes

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📚 EuropePMC1.798 artigos no totalmostrando 196

2026

Research progress on the role of gut microbiota dysbiosis in the pathogenesis of immune-mediated liver diseases.

Frontiers in immunology
2026

Anti-MRPS27 Antibody: A Novel Diagnostic Biomarker for Autoimmune Hepatitis Identified via a Human Proteome Microarray.

Hepatology research : the official journal of the Japan Society of Hepatology
2026

Meta-Analysis: PPAR Agonists for Pruritus and Quality of Life in Primary Biliary Cholangitis.

Alimentary pharmacology &amp; therapeutics
2026

Geographic distribution and demographic patterns of primary biliary cholangitis and autoimmune hepatitis in Colombia: a national population-based administrative study (2018-2024).

Annals of gastroenterology
2026

Consensus statements of the Hellenic Autoimmune Liver Diseases Study Group on the diagnosis and current management of primary biliary cholangitis.

Annals of gastroenterology
2026

Zoledronic acid improves bone mineral density at the lumbar spine and total hip in adults with chronic liver disease.

Journal of clinical densitometry : the official journal of the International Society for Clinical Densitometry
2026

Primary Biliary Cholangitis and Prognostic Models-Reply.

JAMA
2026

Primary Biliary Cholangitis and Prognostic Models.

JAMA
2026

Letter: The association between liver biochemistry, biliary and fibrosis in primary biliary cholangitis.

Alimentary pharmacology &amp; therapeutics
2026

Primary Biliary Cholangitis Increases Mortality Irrespective of Presence or Absence of Cirrhosis.

Liver international : official journal of the International Association for the Study of the Liver
2026

Probiotic Lactobacillus rhamnosus mitigates PBC-like features in Mcpip1-deficient mice via modulation of gut-liver crosstalk.

Biochimica et biophysica acta. Molecular basis of disease
2026

Dual Positivity for AMA/AMA-M2 and Anti-gp210/sp100 Shows Highest Diagnostic Value for Primary Biliary Cholangitis.

Alimentary pharmacology &amp; therapeutics
2026

[Bullous pemphigoid and primary biliary cholangitis, a rare association with therapeutic challenge].

Orvosi hetilap
2026

Liver transplant outcomes in primary biliary cholangitis and metabolic dysfunction-associated steatotic liver disease overlap.

Annals of hepatology
2026

Conjugated bile acids facilitate cholangiocyte senescence to promote cholestatic liver diseases via STING signaling.

Journal of advanced research
2026

Mast Cells at the Crossroad of Gut-Derived Signals Through Aryl Hydrocarbon Receptor Activation: A Microbial-Immune Dialogue in Liver Inflammation with Therapeutic Perspectives.

Cells
2025

Risk factors for mortality in patients with primary biliary cholangitis: a nomogram to predict 5-year survival.

Frontiers in gastroenterology (Lausanne, Switzerland)
2026

Integrative Mendelian randomization and experimental validation unveil novel druggable targets in primary biliary cholangitis.

European journal of pharmacology
2026

Metabolic syndrome and its individual components but not MASLD are associated with advanced fibrosis in primary biliary cholangitis.

Therapeutic advances in gastroenterology
2026

Constructing a protein-protein interaction network for autoimmune liver diseases by integrating pQTL, rQTL, and mediation analyses.

Naunyn-Schmiedeberg's archives of pharmacology
2026

Optimising Primary thErapy in pRimAry biliary cholangitis (OPERA): protocol for a randomised, double-blind, placebo-controlled trial of enhanced primary therapy with obeticholic acid.

BMJ open
2026

Dual cut-offs and beyond: Expanding the role of transient elastography in primary biliary cholangitis.

World journal of gastroenterology
2026

Fatigue and circadian rhythm in non-cirrhotic primary biliary cholangitis: An exploratory comparison with primary sclerosing cholangitis and healthy controls.

World journal of hepatology
2026

Adipokine profiles reflect metabolic dysfunction but not fibrosis in patients with primary biliary cholangitis.

World journal of hepatology
2026

High-intensity hydrophilic versus lipophilic statins in patients with primary biliary cholangitis: a multi-institutional analysis.

BMC gastroenterology
2026

Can liver biopsy be spared for the diagnosis of autoimmune hepatitis in selected children? A multicenter retrospective study.

Annals of hepatology
2026

[Positive antinuclear antibodies suggest a high inflammatory burden phenotype: a multicenter retrospective analysis of patients with primary biliary cholangitis].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
2026

The PBC Ireland patient registry: study protocol for a national platform on primary biliary cholangitis.

Therapeutic advances in rare disease
2026

Australian best practice recommendations for transjugular intrahepatic portosystemic shunt (TIPS) in portal hypertension: a consensus statement.

Hepatology international
2026

Impact of Alkaline Phosphatase Normalization on Complication-Free Survival in Primary Biliary Cholangitis.

The American journal of gastroenterology
2026

Pyroptosis Plays a Key Role Through Macrophages in Primary Biliary Cholangitis of Mice.

BioMed research international
2026

Reconciling the role of ileal bile acid transporter inhibitors as symptom-directed therapy in primary biliary cholangitis: a focused meta-analysis.

European journal of gastroenterology &amp; hepatology
2026

Somatostatin Effect on Growth Factors in Hepatocellular Carcinoma.

Current issues in molecular biology
2026

How do oral pathogenic bacteria potentially contribute to inflammatory processes in autoimmune liver disease? Results from a systematic review.

Clinics and research in hepatology and gastroenterology
2026

Visualization analysis of the use of traditional Chinese medicine in the diagnosis and treatment of rare diseases in mainland China based on CiteSpace.

Intractable &amp; rare diseases research
2026

Comments on: Pre-Diagnosis Alkaline Phosphatase and Antimitochondrial Antibody Positivity Vary by Race/Ethnicity Among Patients With Primary Biliary Cholangitis.

Journal of gastroenterology and hepatology
2026

The ethics of unlicensed therapy use in primary biliary cholangitis: a counter view.

Nature reviews. Gastroenterology &amp; hepatology
2026

Mechanistic Rationale of Seladelpar's Clinical Efficacy through the Selective PPAR-δ Activation in Primary Biliary Cholangitis.

Pharmacological research
2026

Stains in primary biliary cholangitis: Untangling promise from bias.

Hepatology (Baltimore, Md.)
2026

Rheumatologic Manifestations of Patients With Type B Insulin Resistance.

Arthritis care &amp; research
2026

Long-Term Use of Fenofibrate as Second-Line Therapy in Primary Biliary Cholangitis: A Retrospective Study.

Alimentary pharmacology &amp; therapeutics
2026

Human leukocyte antigen variants and clinical features of primary biliary cholangitis: Cumulative contributions.

World journal of clinical cases
2026

Cost of hepatic decompensation and liver transplantation events in primary biliary cholangitis: a retrospective observational study.

Journal of comparative effectiveness research
2026

Plasma Biomarkers of Senescence in Cholestatic Liver Disease: A Signature of Risk Stratification and Progression.

Journal of gastroenterology and hepatology
2026

Mesenchymal stromal cell administration promotes macrophage-mediated bile duct regeneration.

Regenerative therapy
2026

Primary Biliary Cholangitis Pathogenesis: A Pathophysiology-Based Narrative Review.

International journal of molecular sciences
2026

A Review on Farnesoid X Receptor (FXR) Modulators Focusing on Benzimidazole Scaffold.

Molecules (Basel, Switzerland)
2026

The Involvement of Apoptosis Inhibitor of Macrophage in the Disease Severity of Primary Biliary Cholangitis.

Journal of clinical medicine
2026

Primary biliary cholangitis with inflammation involving zone 3 of the liver has a poor response to ursodeoxycholic acid treatment: a retrospective cohort study.

BMC gastroenterology
2026

Clinicopathological features of hepatobiliary cells in primary biliary cholangitis patients with incomplete response to ursodeoxycholic acid.

BMC gastroenterology
2026

Exploration and discovery of treatment targets for primary biliary cholangitis based on plasma and cerebrospinal fluid proteomics: A multicenter mendelian randomization study.

PloS one
2026

Direct bilirubin was superior to total bilirubin in predicting the long-term outcomes of patients with primary biliary cholangitis.

BMC gastroenterology
2025

Evaluation of Histological Criteria and Immunoserological Testing of Simplified Criteria for the Diagnosis of Autoimmune Hepatitis.

The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology
2025

Elevated Serum Soluble Tim-3 in Primary Biliary Cholangitis: Lack of Correlation with Cytokines, Chemokines, and Clinical Parameters.

The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology
2026

Longitudinal multi-omics pilot study: Small sample size human model of gut microbiota-mitochondrial metabolic dysregulation in primary biliary cholangitis.

Microbiological research
2026

Primary Biliary Cholangitis-associated Osteoporosis: Contemporary Review of Pathogenesis and Management.

Journal of clinical and translational hepatology
2026

Acute Graft-Versus-Host Disease After Deceased-Donor Liver Transplantation: A Case Report.

The American journal of case reports
2026

A multifaceted analysis of OTUD5 integrated MAVS in innate immunity of Primary Biliary Cholangitis.

PloS one
2026

Association between type 2 diabetes and autoimmune liver disease: An integrated analysis of Mendelian randomization and clinical samples.

Medicine
2026

Ileal Bile Acid Transporter Inhibitors in Cholestasis: Potential for More Than Just Paediatrics?

Liver international : official journal of the International Association for the Study of the Liver
2026

Autoimmune hepatitis overlap syndrome: Clinical presentation, treatment response, and disease evolution: Tertiary centre experience.

Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology Association
2026

Lipidome remodeling in primary biliary cholangitis.

Lipids in health and disease
2025

Use of Ursodeoxycholic Acid and Cancer Risk for Patients With Primary Biliary Cholangitis.

JAMA network open
2026

Association between statin use and hepatic decompensation in patients with primary biliary cholangitis: A target trial emulation study.

Hepatology (Baltimore, Md.)
2025

Primary Biliary Cholangitis: Novel and Emerging Therapies.

Gastroenterology &amp; hepatology
2025

Exploring Current and Emerging Therapies for Patients With Primary Biliary Cholangitis.

Gastroenterology &amp; hepatology
2025

Diagnostic potential of salivary biomarkers for primary biliary cholangitis: a systematic review.

Frontiers in medicine
2026

Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study.

Liver international : official journal of the International Association for the Study of the Liver
2026

Alkaline Phosphatase Normalization Occurs Less Frequently in Hispanic Patients With Primary Biliary Cholangitis.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
2026

IGFBP4 is a Metric for Primary Biliary Cholangitis and Attenuates Biliary Epithelial Cell Injury.

Liver international : official journal of the International Association for the Study of the Liver
2025

Bile acids as therapeutic agents.

Frontiers in pharmacology
2026

Awareness of Primary Biliary Cholangitis Among Turkish Physicians: A Cross-Sectional, Multicenter, Web-Based Survey.

Journal of clinical medicine
2025

Identification of Differentially Expressed Genes and Molecular Pathways Involved in Primary Biliary Cholangitis Using RNA-Seq.

Genes
2025

Elevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker.

Biomolecules
2026

Kupffer cell autophagy emerges as a central regulator of immune dysregulation in primary biliary cholangitis.

Gut
2026

Seladelpar Improves Cholestasis and Pruritus in Obeticholic Acid and Fibrate-experienced Patients With Primary Biliary Cholangitis.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
2026

Organ-specific autoimmunity in primary Sjögren's disease.

Scandinavian journal of rheumatology
2026

The role of gut microbiota in the pathogenesis of primary biliary cholangitis: an immunological perspective.

Expert review of clinical immunology
2025

Metabolic Dysfunction-Associated Steatohepatitis With Positive Antimitochondrial M2 Antibodies: Diagnostic Pitfall and Importance of Follow-Up.

Cureus
2026

Higher sun exposure is associated with a reduced risk of primary biliary cholangitis.

International journal of epidemiology
2026

ATF3 Mediates the Immunoregulatory Role of the Tim-3 Pathway in Primary Biliary Cholangitis.

Journal of gastroenterology and hepatology
2026

Factors associated with recompensation and criteria for etiological suppression in patients with primary biliary cholangitis.

Journal of gastroenterology
2026

FCRL3 as a potential link between Benzo[a]pyrene exposure and primary biliary cholangitis: insights from comparative toxicogenomics and multi-omics analysis.

BMC gastroenterology
2026

Impact of Type 2 Diabetes Mellitus on Liver Fibrosis and Hepatic Steatosis in Patients with Primary Biliary Cholangitis: A Longitudinal Study.

Digestive diseases and sciences
2025

Dysgeusia-Driven Nutritional Decline in Cirrhosis: A Case of Autoimmune Hepatitis and Primary Biliary Cholangitis.

Cureus
2026

Inferior Vena Cava-Atrial Anastomosis in Liver Transplant Recipient with Inferior Vena Cava Occlusion: A Case Report and Literature Review.

Journal of clinical medicine
2025

Redox Modulation in Hepatic Fibrosis: Translating NOX1/4 Inhibition to Therapy.

International journal of molecular sciences
2026

Evaluating the clinical care, quality of life and overall experiences of patients with primary biliary cholangitis (PBC) during the pandemic: A Canadian mixed-methods study.

PloS one
2026

[A case of recurrent hypoglycemic coma episodes associated with primary biliary cholangitis and literature review].

Zhonghua nei ke za zhi
2025

Genetic evidence supports primary biliary cholangitis as a risk factor for lacunar stroke.

iScience
2026

Variation in microbiome and metabolites is associated with advantageous effects of cholestyramine on primary biliary cholangitis with pruritus.

Microbiology spectrum
2026

Anti-mitochondrial M2 antibody links to cardiac involvement and immune-mediated inflammatory myopathy-like pathology in myositis.

Rheumatology (Oxford, England)
2025

Cell-specific epigenome-wide DNA methylation in peripheral CD4(+) lymphocytes from patients with primary biliary cholangitis.

Journal of translational autoimmunity
2025

Primary Biliary Cholangitis With Incomplete Response to Conventional Therapies But Had Complete Response to Baricitinib: A Case Report.

Case reports in hepatology
2025

Decoding the Gut Microbiome in Primary Sjögren's Syndrome and Primary Biliary Cholangitis: Shared Dysbiosis, Distinct Patterns, and Associations with Clinical Features.

Microorganisms
2025

Liver Innervation in Health and Disease: Neuroimmune-Neurovascular Interface and Future Therapeutic Implications.

Biomedicines
2025

Clinical characteristics and factors associated with liver stiffness in patients with autoimmune liver disease: a cross-sectional study from the Kashgar region.

BMC gastroenterology
2025

The Personalized Management of Primary Biliary Cholangitis in the Era of Precision Medicine: Current Challenges and Future Perspectives.

Journal of personalized medicine
2025

Gastric Squamous Cell Carcinoma as a Cause of Clinically Unstable Gastrointestinal Bleeding.

ACG case reports journal
2025

Vitamin D3 attenuates hepatic inflammation in primary biliary cholangitis by inhibiting TLR4/NF-κB mediated M1 macrophage polarization.

Scientific reports
2026

PBC-AIH variant syndrome: emerging new terminology and a new approach to diagnosis and management.

Current opinion in gastroenterology
2025

The causal relationship between inflammatory bowel disease and primary biliary cholangitis: A bidirectional two-sample Mendelian randomization study.

Medicine
2025

Autoimmune thyroid disease and human health: a systematic review of Mendelian randomization studies.

Frontiers in immunology
2025

Systemic inflammatory response index: a novel predictor for cardiovascular disease risk in patients with primary biliary cholangitis.

European journal of medical research
2025

Clinical outcomes and early- prognostic biomarkers of primary biliary cholangitis with ductopenia.

Frontiers in immunology
2026

Association between quality of life and inflammation and fibrosis markers in primary biliary cholangitis over three-year follow-up.

Scandinavian journal of gastroenterology
2026

Pleiotropic gene mapping reveals shared immune regulatory hubs across 15 autoimmune diseases.

Human immunology
2025

Mechanistic roles and therapeutic potential of bacteriophages in inflammatory gastrointestinal diseases.

Microbiome research reports
2026

Ubiquitin D Correlates with Disease Severity and T Cell Infiltration in Cholestasis: Evidence from Integrated Bioinformatics and Experimental Analyses.

International journal of medical sciences
2026

Primary biliary cholangitis (PBC): evolving approaches and expert perspectives.

Expert review of gastroenterology &amp; hepatology
2025

Assessing the concordance between centromere AC-3 immunofluorescence pattern and anti-centromere protein-B antibody, and analyzing clinical correlates of dual positivity: A retrospective study.

Medicine
2025

Transjugular intrahepatic portosystemic shunt for hepatic sinusoidal obstruction syndrome with primary biliary cholangitis and alcoholic liver disease: a case report.

Frontiers in medicine
2026

Autoimmune liver diseases in Latin America: Current landscape and challenges.

Hepatology communications
2026

A novel cell-permeable LOXL2 inhibitor PAT-1251 potently suppresses biliary liver fibrosis via collagen crosslinking-dependent and -independent mechanisms.

Hepatology communications
2025

Pruritus and health-related quality of life in chronic liver disease: a longitudinal, survey-based cohort study.

BMJ open gastroenterology
2025

Randomized Controlled Trial Evidence on Peroxisome Proliferator-Activated Receptor (PPAR) Agonists in Primary Biliary Cholangitis: A Systematic Review and Meta-Analysis.

International journal of hepatology
2025

Autoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets.

Journal of clinical medicine
2025

From Isolation to Information: Launching an Online Community for Patients with Primary Sclerosing Cholangitis, Primary Biliary Cholangitis, and Autoimmune Hepatitis in Romania-A Descriptive Study.

Healthcare (Basel, Switzerland)
2025

Autophagy of Kupffer cells modulates CD8+ T cell activation in primary biliary cholangitis.

Gut
2025

Necrotizing Fasciitis Caused by Serotype 7C Streptococcus pneumoniae in Overwhelming Post-splenectomy Infection.

Internal medicine (Tokyo, Japan)
2025

Beyond bones: Revisiting the role of vitamin D in chronic liver disease.

World journal of hepatology
2025

Unveiling the Spectrum of Liver Endurance in Systemic Lupus Erythematosus: A Single-Center Experience From Kashmir, India.

Cureus
2025

Clinical characteristics of newly diagnosed patients with primary biliary cholangitis (PBC) indicate the need for better awareness on timely diagnosis and adequate UDCA therapy.

Zeitschrift fur Gastroenterologie
2025

Association between elevations in alkaline phosphatase and healthcare utilization and costs among individuals with primary biliary cholangitis in the United States.

Journal of medical economics
2025

Effectiveness and tolerability of bezafibrate in primary biliary cholangitis - a nationwide real-world study.

The American journal of gastroenterology
2025

Diagnostic Pitfalls in Primary Biliary Cholangitis: Delta Bilirubin and Lipoprotein-X Interferences.

Clinical laboratory
2025

Quantitative detection of anti-centromere antibodies in primary biliary cholangitis: value of chemiluminescence immunoassay.

Scandinavian journal of clinical and laboratory investigation
2025

Tumor necrosis factor alpha-induced protein 3: Biomarker discovery and therapeutic advancement in primary biliary cholangitis.

World journal of hepatology
2025

Risk factors for autoimmune liver disease recurrence after liver transplantation.

World journal of transplantation
2025

Triple autoimmune overlap syndrome of the liver: a rare case of concomitant PBC, PSC, and AIH.

Future science OA
2026

Bile Acid Metabolites Control Th17/Treg Balance in Primary Biliary Cholangitis: Mechanisms of Disease Progression and Therapeutic Targets.

Journal of gastroenterology and hepatology
2026

Economic Burden of Primary Biliary Cholangitis by Line of Therapy in the United States.

Advances in therapy
2025

Smoking and liver diseases: an updated review of pathogenesis, progression, and therapeutic implications.

Clinical and experimental medicine
2025

Association between ABO blood group system and autoimmune liver disease.

Frontiers in medicine
2026

Association of Individual or Family History of Autoimmune Disease With Future Development of Type 1 Diabetes.

Diabetes/metabolism research and reviews
2026

Autoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.

Hepatology international
2025

Fracture events in patients with primary biliary cholangitis during treatment with seladelpar in the phase III RESPONSE trial.

Hepatology communications
2025

Mendelian randomization study of the causal relationship between autoimmune diseases and anemia using proteomic and genetic data.

Journal of translational autoimmunity
2025

AMA-Negative Primary Biliary Cholangitis and Autoimmune Hepatitis Overlap Syndrome Presenting With Bullous Pemphigoid: A Case Report.

Clinical case reports
2025

Altered sphingolipid profile in primary biliary cholangitis: associations with fibrosis and inflammation.

Scientific reports
2025

Golexanolone Attenuates Neuroinflammation, Fatigue, and Cognitive and Motor Impairment in Diverse Neuroinflammatory Disorders.

Pharmaceuticals (Basel, Switzerland)
2025

Profile of Patients with Primary Biliary Cholangitis and Evaluation of Response to Ursodeoxycholic Acid in a Romanian Center-Retrospective Study.

Journal of clinical medicine
2025

Proteomics identifies complement protein signatures in patients with primary biliary cholangitis.

European journal of medical research
2025

Systemic PCSK9 elevation characterises autoimmune liver disease across sexes.

Scientific reports
2025

Optimizing Care in Primary Biliary Cholangitis: Current Treatments and the Second-Line Decision.

Digestive diseases and sciences
2025

Real-world performance of transient elastography in assessing advanced fibrosis in Chinese patients with primary biliary cholangitis.

World journal of gastroenterology
2025

Primary biliary cholangitis. Treatment options in 2025. A narrative review.

Frontiers in immunology
2025

Lipoprotein X-Induced Hyperlipidemia Following Liver Transplantation in the Setting of Persistent Cholestasis and Antibody-Mediated Rejection.

ACG case reports journal
2025

Exploring Maralixibat for Treatment-Resistant Pruritus in Intrahepatic Cholestasis of Pregnancy and Primary Biliary Cholangitis: A Case Report.

The American journal of case reports
2025

Association of liver biomarker values beyond current thresholds and negative clinical outcomes in primary biliary cholangitis: analysis of a real-world healthcare claims database.

Journal of comparative effectiveness research
2025

What the European Reference Network Registry for Rare Liver Diseases Tells Us About Primary Biliary Cholangitis in European Practice.

United European gastroenterology journal
2025

The risk and prevalence of hepatic diseases in psoriasis: A systematic review and meta-analysis.

Journal of the European Academy of Dermatology and Venereology : JEADV
2025

Case Report: Novel IRF2BP2 variant in a Japanese patient with impaired B-cell differentiation, Th1 polarization, and systemic immune dysregulation.

Frontiers in immunology
2025

HSC-derived exosomal miR-122-5p inhibits EMT and fibrosis of intrahepatic biliary epithelial cells to alleviate primary biliary cholangitis.

Frontiers in immunology
2026

Simultaneous degenerative changes in 2 hepatic cavernous hemangiomas observed over 23 years of follow-up: A case report and review of the literature.

Radiology case reports
2025

Hepatocyte iron suppresses liver fibrosis via fibrolytic neutrophil recruitment in cholestasis.

JHEP reports : innovation in hepatology
2025

Numb-exon3 and full length Numb equivalently alleviate cholestatic liver fibrosis by inhibiting ductular reaction.

Scientific reports
2025

Optimizing clinic consultations in primary biliary cholangitis: International consensus recommendations.

Hepatology communications
2025

Spanish clinical practice guidelines for the diagnosis and management of cholestatic liver diseases in adult and pediatric population: Joint report from AEEH and SEGHNP.

Gastroenterologia y hepatologia
2025

PPARevolution. First PPARδ Agonist and a Dual PPARα/PPARδ Activator Approved for the Treatment of Primary Biliary Cholangitis.

Journal of cellular and molecular medicine
2025

Tepotinib-Induced Cholangitis in a Patient With Non-Small Cell Lung Cancer: A Case Report.

Respirology case reports
2025

Letter on 'Non-Response to Obeticholic Acid Is Associated With Heightened Risks of Developing Clinical Events in Primary Biliary Cholangitis'.

Alimentary pharmacology &amp; therapeutics
2025

Causal effects of immune cell phenotypes on the risk of autoimmune liver diseases: a bidirectional two-sample Mendelian randomization study.

Translational gastroenterology and hepatology
2025

Progress and prospects of gut microbiota-targeted therapy for primary biliary cholangitis.

Gut pathogens
2026

Primary Biliary Cholangitis.

JAMA
2025

Percutaneous Transvenous Retrograde Embolization of Adhesion-Related Small Bowel Varices: A Case Report.

Cureus
2025

Beyond Ursodeoxycholic Acid: A Comprehensive Review of Second-Line Agents in Primary Biliary Cholangitis.

Cureus
2026

FXR-targeted drug discovery: Recent advances and therapeutic perspectives.

European journal of medicinal chemistry
2025

Reevaluating the clinical course of AMA-positive patients with normal liver enzymes: A large retrospective cohort study.

Annals of hepatology
2025

Treatment of Refractory Pruritic Dermatitis in the Setting of Primary Biliary Cholangitis and CREST Syndrome With Upadacitinib.

Journal of drugs in dermatology : JDD
2025

Microbiome and gut-liver interactions: From mechanisms to therapies.

World journal of gastroenterology
2025

Single-Nuclei RNA Sequencing Shows the Engagement of PPAR-Delta Target Genes Primarily in Hepatocytes and Cholangiocytes by the Selective PPAR-Delta Agonist Seladelpar.

PPAR research
2026

Letter: Optimizing Risk Stratification in Primary Biliary Cholangitis-The Role of Biochemical Response as a Dynamic Predictor.

Alimentary pharmacology &amp; therapeutics
2026

Linerixibat in patients with primary biliary cholangitis and cholestatic pruritus (GLISTEN): a randomised, multicentre, double-blind, placebo-controlled, phase 3 trial.

The lancet. Gastroenterology &amp; hepatology
2026

Ileal bile acid transporter inhibitors for cholestatic pruritus in primary biliary cholangitis.

The lancet. Gastroenterology &amp; hepatology
2026

Treatment Persistence, Normal Alkaline Phosphatase and Clinical Outcomes in Primary Biliary Cholangitis.

Alimentary pharmacology &amp; therapeutics
2026

Research Communication: Real-World Clinical Experience With Seladelpar in Primary Biliary Cholangitis.

Alimentary pharmacology &amp; therapeutics
2025

Macrophages in Autoimmune Liver Diseases: From Immune Homeostasis to Precision-Targeted Therapy.

Biomedicines
2025

Second-Line Therapies in Primary Biliary Cholangitis: A Comparative Review of Obeticholic Acid, Fibrates, Seladelpar, and Elafibranor.

Biomedicines
2025

Noninvasive elastography-based assessment of liver fibrosis in primary biliary cholangitis.

Annals of hepatology
2025

The Genomics/Genetics of Primary Biliary Cholangitis: The Case for a Functional SNP rs10893900 in ETS1/FLI1 and Review of the Literature.

Clinical reviews in allergy &amp; immunology
2025

Multi-omics profiling uncovers paradoxical Epstein-Barr virus involvement in autoimmune liver disease pathogenesis.

AMB Express
2026

Autophagy Sustained the Activation and Survival of Liver-infiltrating CD8+ T Cells in Primary Biliary Cholangitis.

Immunological investigations
2025

The role of mitochondria-related key genes in primary biliary cholangitis was analyzed based on transcriptome sequencing data.

Frontiers in immunology
2025

Identification of galangin as a therapeutic candidate for primary biliary cholangitis via systematic druggable genome-wide Mendelian randomization analysis and experimental validation.

Frontiers in pharmacology
2026

Recurrent primary biliary cholangitis after liver transplantation: A global meta-analysis of epidemiology and risk factors.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
2025

Histological characteristics of primary biliary cholangitis in patients with incomplete responses to ursodeoxycholic acid.

Human pathology
2025

Assessment of adherence to guidelines for biochemical monitoring and ursodeoxycholic acid treatment response in a retrospective cohort of US patients with primary biliary cholangitis.

BMJ open gastroenterology
2025

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

United European gastroenterology journal
2025

Mixed Multiple Autoimmune Syndrome Type 3 With Coexistence of Primary Biliary Cholangitis, Inflammatory Myopathy, and Chronic Thyroiditis: A Case Report.

Cureus
2025

Advancing the management of primary biliary cholangitis: From pathogenesis to emerging therapies.

World journal of clinical cases
2025

Innate immunity of bile and cholangiocytes in primary biliary cholangitis.

Frontiers in immunology
2025

Future Treatment Options for Managing Primary Sclerosing Cholangitis and Cholestatic Pruritus.

Clinics in liver disease
2025

Recent Advances in Primary Biliary Cholangitis Treatment.

Clinics in liver disease
2025

Causes, associated exposures, and outcomes of cirrhosis and hepatocellular carcinoma in Malawi: an observational cohort and case-control study.

The Lancet. Global health
2026

The effects of lifestyle factors on primary biliary cholangitis ascertained by two-sample Mendelian randomization.

The American journal of the medical sciences
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Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Doença com base genética

Um médico geneticista pode ajudar no diagnóstico de Colangite biliar primária e no aconselhamento genético da família.

Vai consultar um especialista? Confirme o registro dele no conselho.
Conselhos e sociedades

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Protocolo oficial do Ministério da SaúdePCDT: Colangite Biliar Primária

Este é o documento que organiza diagnóstico, tratamento e dispensação de Colangite biliar primária no SUS. O que a farmácia entrega é o que está no PCDT vigente — não o que foi aprovado fora do Brasil.

Respostas geradas por IA a partir das fontes citadas

Trata-se de uma doença autoimune crônica e lenta em que ocorre a destruição gradual dos pequenos canais de bile no interior do fígado. Se não monitorada, essa inflamação contínua pode levar à fibrose e ao comprometimento avançado das funções hepáticas.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

10 publicações do grafo RarasNet (PubMed) · 8 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. ORPHA:186
    Orphanet
  2. Colangite Biliar Primária
    PCDT · Ministério da Saúde
  3. GARD:7459
    GARD (NIH)
  4. Q1072420
    Wikidata

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Citar este verbete

Raras. (s.d.). Colangite biliar primária. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/colangite-biliar-primaria

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Colangite biliar primária
Compêndio · Raras BR

Colangite biliar primária

ORPHA:186 · MONDO:0005388
🇧🇷 Brasil SUS
Geral
Prevalência
1-5 / 10 000
Herança
Multigenic/multifactorial, Unknown
CID-10
K74.3 · Cirrose biliar primária
CID-11
Ensaios
57 ativos
Início
Adolescent, Adult, Elderly
Prevalência
21.05 (Worldwide)
MedGen
UMLS
C0008312
Repurposing
1 candidato
obeticholic-acid — FXR agonist
EuropePMC
Wikidata
Wikipedia
Papers 10a

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Medicamentos aprovados FDA
fonte: FDA OpenFDA
Rara