Pular para o conteúdo
Colangite esclerosante primária
ORPHA:171CID-10 · K83.0CID-11 · DB96.2OMIM 613806DOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
DigestivoInício infantilHerança Complex
Também conhecida comoPSCCEP

A Colangite Esclerosante Primária (CEP) é uma doença rara e que avança lentamente no fígado, caracterizada pela inflamação e destruição dos canais da bile que ficam dentro e/ou fora do fígado. Isso leva ao acúmulo de bile, à formação de cicatrizes (fibrose) no fígado, à cirrose e, por fim, à falência do fígado.

Última verificação: Fonte: sem afirmação sobre o SUS a verificarEm construçãohistóricoSugerir correção
Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

💬
EM LINGUAGEM SIMPLES

A colangite esclerosante primária é uma doença rara do fígado que causa inflamação e cicatrizes nos canais responsáveis pelo transporte da bile. Com o passar dos anos, essa retenção da bile pode provocar dor na barriga, febre, fraqueza nos ossos e cansaço decorrente da alteração no fígado. A condição apresenta causa multifatorial com participação do sistema imunológico e pode surgir em diferentes momentos da vida. No Brasil, ela não consta na lista oficial de Protocolos Clínicos e Diretrizes Terapêuticas (PCDT) de doenças raras do SUS, devendo ser acompanhada nos serviços de referência da rede de saúde.

📋
Informacoes curadas por IA — podem conter imprecisoes

A Colangite Esclerosante Primária (CEP) é uma doença rara e que avança lentamente no fígado, caracterizada pela inflamação e destruição dos canais da bile que ficam dentro e/ou fora do fígado. Isso leva ao acúmulo de bile, à formação de cicatrizes (fibrose) no fígado, à cirrose e, por fim, à falência do fígado.

Pesquisas ativas
41 ensaios
127 total registrados no ClinicalTrials.gov
Publicações científicas
6.800 artigos
Último publicado: 2026 Apr 16

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
7.84
Europe
Início
Adolescent
+ adult, childhood, elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: K83.0
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
19 sintomas
🫘
Rins
5 sintomas
🦴
Ossos e articulações
3 sintomas
📏
Crescimento
3 sintomas
🧠
Neurológico
2 sintomas
🛡️
Imunológico
2 sintomas

+ 22 sintomas em outras categorias

Características mais comuns

90%prev.
Autoimunidade
Muito frequente (99-80%)
90%prev.
Morfologia anormal do trato biliar
Muito frequente (99-80%)
90%prev.
Colestase
Muito frequente (99-80%)
55%prev.
Esplenomegalia
Frequente (79-30%)
55%prev.
Veias abdominais superficiais dilatadas
Frequente (79-30%)
55%prev.
Hemangioma aracniforme
Frequente (79-30%)
59sintomas
Muito frequente (3)
Frequente (16)
Ocasional (28)
Muito raro (8)
Sem dados (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 59 características clínicas mais associadas, ordenadas por frequência.

AutoimunidadeAutoimmunity
Muito frequente (99-80%)90%
Morfologia anormal do trato biliarAbnormal biliary tract morphology
Muito frequente (99-80%)90%
ColestaseCholestasis
Muito frequente (99-80%)90%
EsplenomegaliaSplenomegaly
Frequente (79-30%)55%
Veias abdominais superficiais dilatadasDilated superficial abdominal veins
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico6.800PubMed
Últimos 10 anos200publicações
Pico2026104 papers
Linha do tempo
2026Hoje · 2026🧪 2002Primeiro 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

4 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial.

SEMA4DSemaphorin-4DDisease-causing germline mutation(s) inAltamente restrito
VIAS BIOLÓGICAS (3)
Sema4D induced cell migration and growth-cone collapseSema4D mediated inhibition of cell attachment and migrationOther semaphorin interactions
EXPRESSÃO TECIDUAL(Ubíquo)
Brain Spinal cord cervical c-1
109.8 TPM
Linfócitos
39.0 TPM
Baço
33.1 TPM
Sangue
30.2 TPM
Substância negra
28.5 TPM
OUTRAS DOENÇAS (1)
primary sclerosing cholangitis
HGNC:10732UniProt:Q92854
TCF4Transcription factor 4Major susceptibility factor inAltamente restrito
VIAS BIOLÓGICAS (2)
MyogenesisTGFBR3 expression
MECANISMO DE DOENÇA

Pitt-Hopkins syndrome

A syndrome characterized by intellectual disability, wide mouth and distinctive facial features, and intermittent hyperventilation followed by apnea. Features include intellectual disability with severe speech impairment, normal growth parameters at birth, postnatal microcephaly, breathing anomalies, severe motor developmental delay, motor incoordination, ocular anomalies, constipation, seizures, typical behavior and subtle brain abnormalities.

EXPRESSÃO TECIDUAL(Ubíquo)
Cérebro - Hemisfério cerebelar
38.9 TPM
Cerebelo
28.3 TPM
Tecido adiposo
27.6 TPM
Cervix Endocervix
24.4 TPM
Cervix Ectocervix
24.4 TPM
OUTRAS DOENÇAS (5)
Pitt-Hopkins syndromecorneal dystrophy, Fuchs endothelial, 3Fuchs' endothelial dystrophyprimary sclerosing cholangitis
HGNC:11634UniProt:P15884
GPR35G-protein coupled receptor 35Major susceptibility factor inDesconhecido
VIAS BIOLÓGICAS (1)
Class A/1 (Rhodopsin-like receptors)
EXPRESSÃO TECIDUAL(Ubíquo)
Intestino delgado
17.1 TPM
Cólon transverso
14.8 TPM
Fallopian Tube
7.1 TPM
Baço
6.6 TPM
Pituitária
5.8 TPM
INTERAÇÕES PROTEICAS (2)
OUTRAS DOENÇAS (1)
primary sclerosing cholangitis
HGNC:4492UniProt:Q9HC97
MST1Hepatocyte growth factor-like proteinMajor susceptibility factor inTolerante
VIAS BIOLÓGICAS (1)
Signaling by MST1
VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Ubíquo)
Fígado
395.8 TPM
Testículo
77.5 TPM
Tireoide
62.3 TPM
Rim - Córtex
46.1 TPM
Pituitária
43.0 TPM
OUTRAS DOENÇAS (1)
primary sclerosing cholangitis
HGNC:7380UniProt:P26927

Variantes genéticas (ClinVar)

621 variantes patogênicas registradas no ClinVar.

🧬 SEMA4D: NM_001371194.2(SEMA4D):c.508+5G>A ()
🧬 SEMA4D: NM_001371194.2(SEMA4D):c.1330+30A>G ()
🧬 SEMA4D: NM_001371201.1(SEMA4D):c.1664-5C>G ()
🧬 SEMA4D: GRCh37/hg19 9p24.3-q34.3(chr9:203861-141020389)x3 ()
🧬 SEMA4D: GRCh37/hg19 9p24.3-q34.11(chr9:203861-131603223)x3 ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

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.
3Fase 36
2Fase 230
1Fase 119
·Pré-clínico45
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 esclerosante 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

Ensaios clínicos abertos e novidades científicas recentes

Onde estão os ensaios

Com ensaio aberto em 27 países. O ponto verde marca onde há vaga agora.

🟢 Recrutando agora

27 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

NCT07387549 · A Study to Assess How Well and Safely Elafibranor Works in A…Recrutando
PHASE3🇧🇷 Brasil · 🇦🇷 Argentina · 🇦🇺 Austrália +22
NCT06723275 · LEOPARD Prospective Validation Cohort 1Recrutando
🇧🇪 Bélgica · 🇫🇷 França · 🇩🇪 Alemanha +2
NCT07341282 · Investigation of Vancomycin Efficacy in Patients With Ulcera…Recrutando
PHASE2🇨🇦 Canadá
NCT07607353 · Biodegradable Stents in Primary Sclerosing CholangitisRecrutando
NA🇬🇧 Reino Unido
NCT07229911 · A Study of TAK-781 in Healthy Volunteers and in Participants…Recrutando
PHASE1🇺🇸 Estados Unidos
NCT06699121 · A Study to Assess the Safety and Efficacy of LB-P8 in Patien…Recrutando
PHASE2🇺🇸 Estados Unidos
NCT06905054 · Pharmacologic Approaches to Preventing Primary Sclerosing Ch…Recrutando
PHASE2🇺🇸 Estados Unidos
NCT06675604 · LEOPARD Training and Validation Data Collection StudyRecrutando
🇦🇹 Áustria · 🇧🇪 Bélgica · 🇫🇷 França +4
NCT06455280 · A Study of SIPLIZUMAB in AILD and LT PatientsRecrutando
PHASE1🇺🇸 Estados Unidos
NCT06519162 · Liver-gut Axis Study Through Identification of Liver Disease…Recrutando
South Korea
NCT06286709 · FAecal Microbiota Transplantation in primaRy sclerosinG chOl…Recrutando
PHASE2🇬🇧 Reino Unido
NCT06197308 · Evaluation of an Oral Microbiota-based Therapeutic as a Trea…Recrutando
EARLY_PHASE1🇺🇸 Estados Unidos
NCT05835505 · Detoxification of the Liver In PSC (Dolphin)Recrutando
PHASE2🇺🇸 Estados Unidos
NCT05876182 · Vancomycin in Primary Sclerosing Cholangitis in ItalyRecrutando
PHASE2🇮🇹 Itália
NCT05912387 · Statin Therapy in Primary Sclerosing Cholangitis (PSC): a Mu…Recrutando
EARLY_PHASE1🇺🇸 Estados Unidos
NCT05295680 · Oral Hymecromone to Treat Adolescents and Adults With Primar…Recrutando
PHASE2🇺🇸 Estados Unidos
NCT04685200 · Unraveling the Mechanisms Underlying Primary Sclerosing Chol…Recrutando
🇺🇸 Estados Unidos
NCT05618145 · National Database on Primary Sclerosing Cholangitis (PSC)Recrutando
🇮🇹 Itália
NCT05750498 · A-LiNK: Improving Outcomes in Autoimmune Liver DiseaseRecrutando
🇺🇸 Estados Unidos
NCT04181138 · Primary Sclerosing Cholangitis in ChildrenRecrutando
🇨🇦 Canadá · 🇺🇸 Estados Unidos
NCT04753996 · Characterization of Biliary Cell-derived Organoids From Bile…Recrutando
🇺🇸 Estados Unidos
NCT03146936 · Swiss Primary Sclerosing Cholangitis Cohort StudyRecrutando
🇨🇭 Suíça
NCT03178630 · MRI Biomarkers in as Predictor of Clinical Endpoints in Pedi…Recrutando
🇺🇸 Estados Unidos
NCT03175471 · MRI Based Biomarkers in Pediatric Autoimmune Liver DiseaseRecrutando
🇺🇸 Estados Unidos
NCT03445585 · Biobank for Cholestatic Liver Diseases.Recrutando
🇺🇸 Estados Unidos
NCT06767605 · Spanish Registry of Autoimmune and Cholestatic Liver Disease…Recrutando
🇪🇸 Espanha
NCT02137668 · Treating Primary Sclerosing Cholangitis and Biliary Atresia …Recrutando
PHASE1🇺🇸 Estados Unidos

Outros ensaios clínicos

127 ensaios clínicos encontrados, 41 ativos.

Distribuição por fase
NCT07843420 · Nemolizumab for the Treatment of Moderate to Severe Chronic …Em breve
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PHASE2🇨🇳 China
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PHASE2
NCT06886360 · Study with Norucholic Acid Tablets in Patients with Primary …Em breve
PHASE3
NCT05462093 · Clinical Application of Annual Liver Multiscan and MRCP+ in …Em breve
NA
NCT04663308 · A Study to Evaluate Efficacy and Safety of an Investigationa…Ativo
PHASE2🇧🇷 Brasil · 🇦🇷 Argentina · 🇦🇺 Austrália +11
NCT05896137 · CS0159 in Chinese Patients With PSC (Primary Sclerosing Chol…Ativo
PHASE2🇨🇳 China
NCT06026449 · Gluten-free Diet in PSC and IBDAtivo
NA🇨🇿 Tchéquia
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NCT05740358 · Liver Cirrhosis Network Cohort StudyAtivo
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PHASE3🇸🇪 Suécia
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NA🇨🇦 Canadá
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NA🇵🇱 Polônia
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PHASE2South Korea
NCT05082779 · Study in Healthy Volunteers to Evaluate the Safety, Tolerabi…Concluído
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NCT06686810 · Endoscopy and Primary Sclerosing CholangitisConcluído
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NCT07784881 · Deciphering the Self-antigen Specific T Cell Signature of Au…Concluído
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NCT04595825 · CM-101 in PSC Patients -The SPRING StudyConcluído
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NCT04678219 · A Pilot Study Examining Diet in Primary Sclerosing Cholangit…Concluído
NA🇺🇸 Estados Unidos
NCT04480840 · Phase 2a Evaluation of Safety, Tolerability, and Pharmacokin…Concluído
PHASE2🇦🇺 Austrália · 🇦🇹 Áustria · 🇧🇪 Bélgica +6
NCT03722576 · Vidofludimus Calcium for Primary Sclerosing CholangitisConcluído
PHASE2🇺🇸 Estados Unidos
NCT06037577 · Subcutaneous Doses of CM-101 as a Treatment for Medical Cond…Concluído
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NCT03766035 · Cholangioscopy in Primary Sclerosing Cholangitis (PSC)Concluído
🇨🇦 Canadá · 🇳🇱 Países Baixos · 🇳🇴 Noruega +1
NCT03809559 · Repeatability and Reproducibility of Quantitative MRCPConcluído
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NCT02943460 · Study to Evaluate the Safety, Tolerability, and Efficacy of …Concluído
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NCT02808312 · Pharmacokinetics and Pharmacodynamics of Cilofexor in Adults…Concluído
PHASE1New Zealand · 🇺🇸 Estados Unidos
NCT02712736 · Quality of Life in Patients With Primary Sclerosing Cholangi…Concluído
NA🇺🇸 Estados Unidos
NCT02653625 · PERSEUS: Preliminary Efficacy and Safety of Cenicriviroc in …Concluído
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NCT01161992 · Genomics of Primary Sclerosing Cholangitis (PSC)Concluído
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NCT06351696 · The Effects of Bromelain Supplement in Patients With Ulcerat…UNKNOWN
NAIran
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NCT04981756 · PSC Clinical Epidemiology in ChinaUNKNOWN
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NCT04309773 · Efficacy of 24 Month of Bezafibrate in Primary Sclerosing Ch…UNKNOWN
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NCT05181332 · Predictive Models of Hepatic Decompensation and Survival Out…UNKNOWN
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NCT03951324 · Volumetric Laser Endomicroscopy's (VLE) Diagnostic Accuracy …UNKNOWN
🇺🇸 Estados Unidos
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Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
3.302 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 3.302

#1

Dysbiotic microbiota trigger colitis-associated colorectal cancer and imprint a distinctive bile acid profile in a PSC-IBD model.

Gut2026 Mar 23

Primary sclerosing cholangitis-associated UC (PSC-UC) carries excess colorectal neoplasia despite often mild-appearing endoscopy, implicating persistent microscopic inflammation and microbiota-bile acid (BA) dysfunction. To test whether PSC-UC neoplasia is driven by transferable microbiota-mediated inflammation linked to secondary BA loss. Surveillance colonoscopies (2012-2022) from PSC-UC (n=251) and UC-only (n=8839) were compared for segmental endoscopic/histological activity and dysplasia. We generated multidrug resistance protein 2 (MDR2)-/- × interleukin (IL)-10-/- double-knockout (DKO) mice and used germ-free (GF) derivation, faecal microbiota transplantation (FMT), antibiotic conditioning and cohousing with shotgun metagenomics and liquid chromatography-tandem mass spectrometry BA profiling. PSC-UC showed greater inflammatory activity and a right-shifted dysplasia burden versus UC-only. Under specific-pathogen-free conditions, DKO mice developed early right-predominant colitis and multifocal dysplasia progressing with age. DKO communities were depleted of 7α-dehydroxylation capacity with near absence of deoxycholic and lithocholic acids and no enrichment of canonical bacterial genotoxins. GF DKO mice were protected, whereas live DKO donor FMT reinstated severe colitis and dysplasia; sterile-filtered stool supernatant was inactive. IL-10-/- donor FMT or cohousing attenuated colitis and increased recipient secondary BA, whereas wild-type/MDR2-/- donor transfers were non-colitogenic. In GF DKO mice, direct deoxycholic acid repletion caused hepatotoxicity. PSC-UC neoplasia associates with transmissible microbiota-dependent inflammation and secondary BA deficiency. Controlled restoration of BA-transforming microbial functions, rather than indiscriminate secondary BA replacement, is a rational translational direction.

#2

Primary sclerosing cholangitis.

Lancet (London, England)2026 Mar 20

Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by biliary inflammation and fibrosis. Inflammatory bowel disease co-occurs in 50-80% of individuals with primary sclerosing cholangitis and there is an increased risk for hepatobiliary and colorectal cancers. Primary sclerosing cholangitis presentation is highly variable but there is usually a slowly progressive fibrosis of the bile ducts with strictures, development of liver fibrosis and cirrhosis, and eventually a need for liver transplantation, after which primary sclerosing cholangitis can reoccur. Primary sclerosing cholangitis is diagnosed mostly at the asymptomatic stage but, as the disease advances, people often have itching, fatigue, upper right abdominal pain, recurrent cholangitis, or complications related to portal hypertension. There are few treatment options and its exact cause and pathogenesis remain unclear. It is widely believed that both genetic and environmental factors are important, with the intestinal microbiome increasingly recognised as crucial to disease development, progression, and outcomes. This Seminar explores the clinical features of primary sclerosing cholangitis, summarises the current understanding of its pathogenesis, and gives insights into the challenges and opportunities in managing the disease.

#3

Distinct phenotype of primary sclerosing cholangitis-associated inflammatory bowel disease.

Journal of gastroenterology2026 Mar 19

Primary sclerosing cholangitis (PSC) is frequently accompanied by colitis with clinicopathologic features that differ from conventional inflammatory bowel disease (IBD). Accumulating evidence indicates that PSC-associated IBD (PSC-IBD) often presents as extensive colitis with a right-sided predominant distribution and characteristic endoscopic features, such as rectal sparing and backwash ileitis. Beyond endoscopic appearance, PSC-IBD also appears to have distinct biological underpinnings, including shared genetic susceptibility, dysregulated bile acid signaling, altered gut microbial communities, and immune crosstalk along the gut-liver axis. These mechanisms may contribute not only to intestinal inflammation but also to PSC-related clinical outcomes. From a long-term perspective, patients with PSC and colitis are consistently classified as a high-risk group for colorectal neoplasia, warranting early and intensive colonoscopic surveillance. In parallel, surveillance for hepatobiliary malignancies remains central in PSC care, although risk stratification continues to evolve. Therapeutic management generally follows established IBD algorithms, yet the extent to which colitis-directed therapies modify PSC outcomes remains uncertain, with heterogeneous findings across cohorts and endpoints. In this review, we summarize current knowledge on the clinical phenotype, mechanistic framework, and outcome-driven management of PSC-IBD, and highlight future directions toward precision surveillance and mechanism-based interventions targeting bile acid-microbiota-immune interactions.

#4

Colitis-Associated Carcinoma: The Quintessential Epithelial Neoplasia Driven by Chronic Inflammation.

Cells2026 Mar 06

Colitis-associated carcinoma (CAC) represents ~1% of colorectal carcinomas and has important differences from sporadic colorectal carcinoma (sCRC). The precursors and carcinomas that arise in the setting of IBD are uniquely challenging to visualize by endoscopy and diagnose via histology, and the rising prevalence of IBD amplifies the challenges of surveillance to informed management. Although in broad strokes, CAC and sCRC share molecular features (~85% chromosomal instability pathway 15% microsatellite instability high (MSI-H)), CAC has a distinct distribution of molecular abnormalities, including lower frequencies of APC and KRAS mutations, greater prevalence of IDH1R132H, and more frequent copy number alterations (e.g., MYC amplifications), and functional data indicate that most CACs show far less dependence on Wnt signaling than sCRC, suggesting a distinct pathogenesis from the earliest stages. Although there are significant gaps in our knowledge of the pathogenesis of CAC, our understanding is growing. This review summarizes how chronic colitis reshapes epithelial homeostasis and somatic evolution, resulting in the distinctive pathogenesis of CAC, and highlights knowledge gaps that could be addressed by applying multimodal technologies to well-annotated clinical material. The review is structured in two sections, the first introducing the IBDs and the homeostatic mechanisms that preserve integrity and prevent colorectal neoplasia. The second section compares failure modes in sporadic and colitic settings and describes the differences in the resulting neoplasms.

#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.

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2026

Cholestatic liver disease in an adolescent: don't forget progressive familial intrahepatic Cholestasis-8.

Oxford medical case reports
2026

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

Frontiers in immunology
2026

Dysbiotic microbiota trigger colitis-associated colorectal cancer and imprint a distinctive bile acid profile in a PSC-IBD model.

Gut
2026

Primary sclerosing cholangitis.

Lancet (London, England)
2026

Microscopic colitis and primary sclerosing cholangitis are bidirectionally associated: A nationwide matched cohort and case-control study.

Journal of internal medicine
2026

Distinct phenotype of primary sclerosing cholangitis-associated inflammatory bowel disease.

Journal of gastroenterology
2026

Beyond alkaline phopshatase: can anti-integrin αvβ6 autoantibodies serve as biomarkers in primary sclerosing cholangitis trials?

Journal of hepatology
2026

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

Journal of advanced research
2026

Colitis-Associated Carcinoma: The Quintessential Epithelial Neoplasia Driven by Chronic Inflammation.

Cells
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
2026

Prevalence of Comorbidities in Inflammatory Bowel Disease: An Umbrella Review of 18 Systematic Reviews.

Journal of clinical medicine
2026

Evaluation and management of primary sclerosing cholangitis patients awaiting liver transplantation.

JHEP reports : innovation in hepatology
2026

Prevalence, interreader agreement, and prognostic value of high-grade and relevant strictures in individuals with primary sclerosing cholangitis.

European radiology
2025

Endometriosis presenting as a rectal stricture in a patient with ulcerative colitis and primary sclerosing cholangitis: a case report.

Frontiers in gastroenterology (Lausanne, Switzerland)
2026

Defining the Contribution of Genetic Variants in MRGPRX4 With Pruritus in Paediatric Cholestasis: Evidence From Case-Control Study.

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

Graft and Patient Survival After Liver Transplantation for Primary Sclerosing Cholangitis: A French National Cohort Study.

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

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

The role and possible mechanism of intestinal fungi in the progression of chronic liver diseases.

NPJ biofilms and microbiomes
2026

Duct-to-Duct Versus Roux-en-Y in Liver Transplantation for Primary Sclerosing Cholangitis: When Evidence Leaves Room for Pragmatism.

Transplantation
2026

[A new paradigm of the pathogenesis of primary sclerosing cholangitis associated with ulcerative colitis: microbiota and system intestine-liver. A review].

Terapevticheskii arkhiv
2026

Recurrent ileocolic variceal hemorrhage treated with superselective Onyx embolization: A case report.

Radiology case reports
2026

Primary sclerosing cholangitis pathogenesis revisited: EBV-idence of immune dysregulation.

JHEP reports : innovation in hepatology
2026

Clinico-pathological and immunohistochemical profile of plasma cell rich variants of chronic cholecystitis.

Annals of diagnostic pathology
2026

Cardiovascular risk profiles in patients with primary sclerosing cholangitis.

European journal of gastroenterology &amp; hepatology
2026

Periductal iron-corrected T1 is a predictor of adverse outcomes in large-duct primary sclerosing cholangitis.

BMC medical imaging
2026

Prognostic value of magnetic resonance imaging in pediatric-onset primary sclerosing cholangitis.

Therapeutic advances in gastroenterology
2026

Bile Acid Sequestrants in Primary Sclerosing Cholangitis and the Risk of Acute Cholangitis: Cause for Concern?

Clinical and translational gastroenterology
2026

Acquired bisalbuminemia associated with hepatobiliary disease: a two-case report and diagnostic implications.

Laboratory medicine
2026

Proctocolectomy with permanent ileostomy is associated with improved transplant-free survival in patients with PSC.

JHEP reports : innovation in hepatology
2026

Proteome-scale autoantibody profiling in PSC: Associations with clinical phenotypes and evidence for neuroendocrine deregulations.

JHEP reports : innovation in hepatology
2026

IgG4-related sclerosing cholangitis: navigating diagnostic dilemmas and the challenge of relapse.

Frontiers in medicine
2026

Differences in immunosuppressive strategies for primary sclerosing cholangitis after liver transplantation.

Journal of hepatology
2026

Statistics and epidemiology of inflammatory bowel disease-associated colorectal neoplasia.

International journal of clinical oncology
2026

Oral vancomycin for primary sclerosing cholangitis and associated inflammatory bowel disease - paving a path forward.

Expert review of gastroenterology &amp; hepatology
2026

The Utility of Elastography in Discriminating Stages of Liver Fibrosis and Risk Stratification in Patients With Primary Sclerosing Cholangitis: A Systematic Review.

Journal of magnetic resonance imaging : JMRI
2026

Incidence and Risk of Colorectal Dysplasia in Patients With Inflammatory Bowel Disease: A Nationwide Cohort Study.

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

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

Journal of gastroenterology and hepatology
2026

A Mendelian randomization study exploring the genetic associations between biliary system disorders and brain structural changes.

Medicine
2026

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

Regenerative therapy
2026

A Rare Finding at the Hepaticojejunostomy in a Postliver Transplant Patient.

ACG case reports journal
2026

Primary Sclerosing Cholangitis: Diagnosis, Management, and Clinical Challenges.

Journal of clinical medicine
2026

Colitis-associated colorectal neoplasia in ulcerative colitis with primary sclerosing cholangitis: a nationwide study.

Intestinal research
2026

A multinational survey assessing the impact and burden of pruritus on patients with primary sclerosing cholangitis.

Hepatology communications
2026

Advanced therapies for inflammatory bowel disease associated with primary sclerosing cholangitis: a systematic review and meta-analysis.

Inflammatory bowel diseases
2026

Primary sclerosing cholangitis in children: a single-center experience and evaluation of prognostic markers.

Translational gastroenterology and hepatology
2026

Liver Transplant in Primary Sclerosing Cholangitis: Results in Our Center.

Transplantation proceedings
2026

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

Medicine
2026

Superior Diagnostic Performance of 99mTc-FAPI-46 SPECT/CT Versus 18FDG PET/CT in Detecting Peritoneal Involvement in Mucinous Adenocarcinoma of the Colon.

Clinical nuclear medicine
2026

Clinical Outcomes in Patients with Primary Sclerosing Cholangitis With and Without Inflammatory Bowel Disease.

Digestive diseases and sciences
2026

Decoding the Role of H19 in Cholestatic Liver Injury Using snRNA-seq, Spatial Transcriptomics, and Machine Learning-Based Disease Prediction.

Research square
2026

Current and Emerging Approaches for Management of Primary Sclerosing Cholangitis.

Gastroenterology &amp; hepatology
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

Association between osteoporosis and waitlist mortality in liver transplant candidates.

Scientific reports
2026

Understanding hepatopancreatobiliary cancer risks in a population-based primary sclerosing cholangitis-inflammatory bowel disease cohort.

Hepatology (Baltimore, Md.)
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

Uncovering the genetic landscape of cholangiocarcinoma and its subtypes via GWAS and integrative analyses.

Hepatology (Baltimore, Md.)
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

Long-term clinical and endoscopic outcomes of ulcerative colitis after liver transplantation: a multicenter cohort study on the use and safety of advanced therapies.

Inflammatory bowel diseases
2026

Prevalence and Risk Factors of Colorectal Cancer in Solid Organ Transplant Recipients: A Systematic Review and Meta Analysis.

Clinical transplantation
2026

Multimodal treatment and tumour biology-driven long-term survival in PSC-associated hilar cholangiocarcinoma: A case report.

Medicine international
2026

Vedolizumab induced acute interstitial nephritis.

Oxford medical case reports
2026

MUC1 as a biomarker in primary sclerosing cholangitis and cholangiocarcinoma.

Annals of gastroenterology
2026

Serum anti-integrin αvβ6 autoantibodies for diagnosis of primary sclerosing cholangitis: a systematic review and meta-analysis.

Annals of gastroenterology
2026

Management of Inflammatory Bowel Disease Associated With Primary Sclerosing Cholangitis Pre- and Post-liver Transplantation.

Transplantation
2025

Bile acids as therapeutic agents.

Frontiers in pharmacology
2025

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

Biomolecules
2026

Identifying biomarkers for diagnosis and disease activity monitoring in PSC-IBD and UC through proteomic profiling: A prospective, biomarker discovery single-center study protocol.

PloS one
2026

[Immunopathogenesis and therapy of primary sclerosing cholangitis].

Zeitschrift fur Gastroenterologie
2026

HERACLIS-PSC: Current characteristics of primary sclerosing cholangitis with or without inflammatory bowel disease in Greece-a national multicenter cohort study.

Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver
2026

Patient-reported Symptoms Are Independent of Extent of Disease in Longstanding Ulcerative Colitis: Magic in Imagine.

Journal of clinical gastroenterology
2026

Updated Systematic Review and Meta-Analysis of Duct-to-duct Versus Hepaticojejunostomy Reconstruction After Liver Transplantation for Primary Sclerosing Cholangitis.

Transplantation
2026

Cholestatic biomarkers and symptoms in primary sclerosing cholangitis: a post-hoc analysis of the DILSTENT.

Scandinavian journal of gastroenterology
2026

Dietary Lauric Acid Suppresses Inflammation, Cholestasis, Hepatocyte Injury, and Senescence in 3,5-Diethoxycarbonyl-1,4-Dihydrocollidine-induced Inflammatory Cholangiopathy.

Cellular and molecular gastroenterology and hepatology
2026

Eosinophilic cholangitis without peripheral eosinophilia presenting as a localized bile duct stricture mimicking perihilar cholangiocarcinoma.

Clinical journal of gastroenterology
2026

Decoding follicular cholangitis: A benign entity with a malignant façade.

Indian journal of pathology &amp; microbiology
2026

Risk of extraintestinal cancer in patients with inflammatory bowel disease: a multicenter retrospective cohort study in Japan.

Journal of gastroenterology
2026

Fecal calprotectin in patients with concomitant primary sclerosing cholangitis and inflammatory bowel disease: a systematic review and meta-analysis.

Clinics and research in hepatology and gastroenterology
2026

FIRST BRAZILIAN CONSENSUS ON CHOLANGIOPANCREATOSCOPY BY THE BRAZILIAN SOCIETY OF DIGESTIVE ENDOSCOPY (SOBED).

Arquivos de gastroenterologia
2025

A multilevel analysis of the genetic association between inflammatory bowel disease and primary sclerosing cholangitis.

European journal of gastroenterology &amp; hepatology
2025

The Scope of Percutaneous Biliary Cholangioscopy: Beyond Choledocholithiasis.

Journal of digestive diseases
2026

Prospective, randomised, placebo-controlled, phase 2 clinical trial assessing the efficacy and safety of oral vancomycin in patients with primary sclerosing cholangitis with/out inflammatory bowel disease in Italy: study protocol of VanC-IT trial.

BMJ open
2025

MAIT Cells in Liver Disease.

Cells
2026

DNA Methylation-mediated BTN3A2 Regulation via CD14+CD16+ Monocytes Protects Against Primary Sclerosing Cholangitis.

Current topics in medicinal chemistry
2026

Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan.

Journal of hepato-biliary-pancreatic sciences
2026

[On familiar terms with the bile and pancreatic ducts : Where is the benefit?].

Innere Medizin (Heidelberg, Germany)
2026

Risk Factors for Primary Sclerosing Cholangitis Recurrence Following Liver Transplantation: A Multicenter Retrospective Analysis.

Clinical transplantation
2026

Validation of Quantitative Magnetic Resonance Cholangiopancreatography Metrics in Prediction of Transplant-free Survival in Primary Sclerosing Cholangitis.

Journal of clinical and experimental hepatology
2026

The Prognostic Utility of Quantitative Magnetic Resonance Cholangiopancreatography in Patients With PSC: A Systematic Review With Structured Evidence Synthesis.

Journal of computer assisted tomography
2025

Drug-induced liver injury in inflammatory bowel disease: Challenges in diagnosis and monitoring.

World journal of hepatology
2025

Protocol for fabricating a vascularized bile duct-on-a-chip.

Biophysics reports
2026

Clinical, Endoscopic, and Upper Gastrointestinal Histological Manifestations in Pediatric Inflammatory Bowel Disease: Insights from a Large Cohort.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
2025

Quality and Quantity? The Clinical Significance of Myosteatosis in Various Liver Diseases: A Narrative Review.

Journal of clinical and translational hepatology
2025

Graves' disease with autoimmune hepatitis and primary Sclerosing cholangitis: an overlap syndrome.

Oxford medical case reports
2026

Single-Cell Genetic Mapping of Gasdermin Expression Across Immune-Mediated Inflammatory Diseases.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology
2026

Hepatocyte Mettl3 Deficiency Drives Primary Sclerosing Cholangitis and Liver Fibrosis via Cholangiocyte-Macrophage Crosstalk.

Advanced science (Weinheim, Baden-Wurttemberg, Germany)
2026

Novel MicroRNA biomarkers revealed in the serum and bile of thioacetamide induced cholangiocarcinoma.

Chemico-biological interactions
2025

Exploring the biliary microbiome in hepatopancreatobiliary disorders: a comprehensive systematic review of microbial signatures and diagnostic potential.

BMC gastroenterology
2025

RIPK1 inhibition reduces biliary injury and fibrosis in primary sclerosing cholangitis.

Science advances
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
2025

Endoscopic retrograde cholangiopancreatography and primary sclerosing cholangitis: a retrospective study of a high-volume program.

Journal of the Canadian Association of Gastroenterology
2026

Viral infection as a trigger for primary sclerosing cholangitis in genetically susceptible individuals.

Journal of hepatology
2026

Burden of primary sclerosing cholangitis in Sweden (2002-2020): Incidence, outcomes, healthcare utilization, and costs.

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

Biliary Reconstruction in Liver Transplantation with Primary Sclerosing Cholangitis: Roux-en-Y Hepaticojejunostomy or Duct-to-Duct Anastomosis?

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

Risk of Incident Immune-Mediated Inflammatory Diseases with Second Tumor Necrosis Factor Inhibitor Versus Alternative Biologic Therapy in Patients with Inflammatory Bowel Disease and First TNFi Exposure: A Real-World Cohort Study.

Digestive diseases and sciences
2025

Radial laser ablation under cholangioscopic guidance for a tight intrahepatic bile duct stricture caused by primary sclerosing cholangitis.

Endoscopy
2025

Pembrolizumab-Induced Secondary Cholangitis: A Case Report.

Cureus
2025

Beyond futility: The history and potential of liver transplantation in cholangiocarcinoma.

World journal of transplantation
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

miR-21-5p dysregulation is associated with gut microbiota dysbiosis and pro-oncogenic markers in primary sclerosing cholangitis with concomitant inflammatory bowel disease.

Experimental and molecular pathology
2025

Pruritus is common in primary sclerosing cholangitis, persists over time, and its intensity is associated with disease severity: a multicentre, prospective observational study.

Hepatology (Baltimore, Md.)
2026

Risk of intestinal complications, extraintestinal morbidity, and mortality in patients with ulcerative colitis and ocular involvement.

Eye (London, England)
2025

Prevalence and Outcomes of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease: A Multinational Study Across Asia.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
2025

Risk of Intestinal and Extraintestinal Malignancies in Inflammatory Bowel Disease With and Without Primary Sclerosing Cholangitis.

Digestive diseases and sciences
2026

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

Hepatology international
2025

A retrospective evaluation of preemptive liver transplantation for bile duct dysplasia in primary sclerosing cholangitis: Balancing risks and benefits.

JHEP reports : innovation in hepatology
2025

Intestinal barrier in chronic gut and liver diseases: Pathogenesis and therapeutic targets.

Acta pharmaceutica Sinica. B
2025

Impact of Metabolic Dysfunction-Associated Steatotic Liver Disease on Fatigue and Pruritus in Primary Sclerosing Cholangitis: A U.S. Single-Center Study.

Journal of clinical medicine
2025

Blood and tissue dysregulated bile acids and short-chain fatty acids in cholangiocarcinoma.

JHEP reports : innovation in hepatology
2026

Differences in Anti-αvβ6 Integrin Antibody Expression between U.S. and Japanese Cohorts in Inflammatory Bowel Disease.

Inflammatory bowel diseases
2025

Systemic PCSK9 elevation characterises autoimmune liver disease across sexes.

Scientific reports
2025

An overview of paediatric autoimmune and genetic cholestatic liver disease for the adult physician.

Clinical medicine (London, England)
2025

Moderate increase of MET in hepatocytes protects against cholestatic liver injury by promoting an effective antioxidant response.

The FEBS journal
2025

Safety and Efficacy of Upadacitinib in Patients with Inflammatory Bowel Disease After Liver Transplantation: A Case Series.

Digestive diseases and sciences
2025

Causal associations between primary sclerosing cholangitis and systemic lupus erythematosus: Evidence from Mendelian randomization and transcriptomic analyses.

Medicine
2026

Hepatobiliary and pancreatic findings on magnetic resonance enterography examinations in children with newly diagnosed inflammatory bowel disease.

Pediatric radiology
2025

Nebokitug, an Anti-chemokine (C-C Motif) Ligand 24 Monoclonal Antibody, in Patients With Primary Sclerosing Cholangitis: A Phase 2 Study.

The American journal of gastroenterology
2025

Selective Inhibitors of Toll-Like Receptor 9 (TLR9) Could Potentially Treat Fibrotic, Autoimmune, and Inflammatory Diseases.

ACS medicinal chemistry letters
2025

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

ACG case reports journal
2026

Clinicogenomic Characterization of Primary Sclerosing Cholangitis-Associated Biliary Tract Cancers.

Clinical cancer research : an official journal of the American Association for Cancer Research
2025

Causal mediation of immune cells in autoimmune liver diseases and oral cancer risk: a mendelian randomization study.

International journal of surgery (London, England)
2025

Regulatory T cell stability determines the efficiency of bile duct regeneration during cholangitis.

Cell reports
2025

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

Scientific reports
2025

Risk stratification of IBD-associated liver disease using routinely collected biomarkers from a large-scale real-world dataset.

BMJ open gastroenterology
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

Gut-Liver Axis, Microbiota, Bile Acids, and Immune Response in Pathogenesis of Primary Sclerosing Cholangitis: An Overview.

Journal of clinical medicine
2025

Association between non-invasive biomarkers and quality of life in Primary Sclerosing Cholangitis.

PloS one
2025

Primary sclerosing cholangitis: a narrative review of diagnostic and prognostic biomarkers.

Translational gastroenterology and hepatology
2025

Chronic Recurrent Multifocal Osteomyelitis Associated in a Child with Inflammatory Bowel Disease and Primary Sclerosing Cholangitis: A Case Report.

JMA journal
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
2026

Phase II clinical trials in primary sclerosing cholangitis: Making the most of every opportunity?

Journal of hepatology
2026

Genetic architecture of primary sclerosing cholangitis: shared pathways with inflammatory bowel disease and gut-liver axis mediation.

International journal of surgery (London, England)
2025

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

World journal of gastroenterology
2025

Dietary and Lifestyle Risk Factors in Primary Sclerosing Cholangitis: In Search of Mechanistic Explanations and Health Improvement.

The Journal of nutrition
2026

Cilofexor in non-cirrhotic primary sclerosing cholangitis (PRIMIS): a randomised, double-blind, multicentre, placebo-controlled, phase 3 trial.

The lancet. Gastroenterology &amp; hepatology
2025

Liver transplantations in Finland over 40 years: evolution and recent trends.

eGastroenterology
2025

Quantitative MRCP as Part of Primary Sclerosing Cholangitis Standard of Care in the National Health Service in England: A Feasibility Assessment Among Hepatologists.

Healthcare (Basel, Switzerland)
2025

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

Biomedicines
2025

Infections in primary sclerosing cholangitis and inflammatory bowel disease: a systematic review and meta-analysis.

Journal of the Canadian Association of Gastroenterology
2025

Apremilast as a novel therapeutic option for psoriasis coexisting primary sclerosing cholangitis and ulcerative colitis: A case report.

Medicine
2025

Primary Sclerosing Cholangitis in a Child: A Rare Case Report.

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
2025

Association of Primary Sclerosing Cholangitis With HLA-C*07:02 in a Japanese Population.

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

Navigating the Hepatic Immune Landscape With Fine Needle Aspiration of the Liver-An Emerging Technique.

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

Cultured bacteria isolated from primary sclerosing cholangitis patient bile induce inflammation and cell death.

mSphere
2025

Ulcerative colitis and colorectal cancer: Pathogenic insights and precision strategies for prevention and treatment.

World journal of gastrointestinal oncology
2025

Innate immune cell LXR-β deficiency exacerbates hepatic injury and fibrosis in murine models of primary sclerosing cholangitis.

Liver research (Beijing, China)
2025

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

Clinics in liver disease
2025

Cholangiocarcinoma - Morphology, Immunohistochemistry, and Genetics.

Ceskoslovenska patologie
2025

Spatial heterogeneity in liver stiffness does not predict clinical outcomes in patients with primary sclerosing cholangitis.

Hepatology communications
2025

Epidemiology, comorbidities, treatments and outcomes of autoimmune liver diseases: A French nationwide study.

JHEP reports : innovation in hepatology
2025

Point-of-Care Testing and Biomarkers in Biliary Diseases: Current Evidence and Future Directions.

Journal of clinical medicine
2025

Localization of the therapeutic targets for endothelin receptor antagonists and sodium-glucose co-transporter 2 inhibitors in the chronic liver disease, primary sclerosing cholangitis.

Frontiers in pharmacology
2025

Autoimmune Hepatitis Associated with Other Autoimmune Diseases: A Critical Review.

Journal of clinical and translational hepatology
2025

Coexisting inflammatory bowel disease in primary sclerosing cholangitis is associated with higher colorectal cancer and transplant risk.

Journal of internal medicine
2025

EGFR activation in cholangiocytes promotes extrahepatic bile duct regeneration after injury.

Hepatology communications
2025

Disruption of Bile Acid Metabolism in the Gut-Liver Axis Predisposes Mice to Inflammatory Bowel Disease.

MedComm
2025

Large duct primary sclerosing cholangitis with or without inflammatory bowel disease in Saudi patients.

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

Relationship between depression and chronic liver disease: Potential role of antidepressants in modulating liver fibrosis.

The American journal of the medical sciences
2025

From inflammation to carcinogenesis: Distinct pathways and clinical implications of IBD-associated colorectal cancer compared with sporadic CRC.

Pathology, research and practice
2025

Identification of MMEL1 as a novel therapeutic target for primary sclerosing cholangitis: Integrating Mendelian randomization and transcriptome landscapes.

Medicine
2025

Immune Activation in Primary Sclerosing Cholangitis: A Systematic Review and Comparative Analysis With Inflammatory Bowel Diseases.

United European gastroenterology journal
2026

Phase II INTEGRIS-PSC trial of bexotegrast, an αvβ6/αvβ1 integrin inhibitor, in primary sclerosing cholangitis.

Journal of hepatology
2025

Radiomics-Based Prognostication in Primary Sclerosing Cholangitis: A Proof-of-Concept Study.

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

Microbial Landscapes of the Gut-Biliary Axis: Implications for Benign and Malignant Biliary Tract Diseases.

Microorganisms
2025

Chronic Liver Disease Associated Cholangiocarcinoma: Genomic Insights and Precision Therapeutic Strategies.

Cancers
2025

Antineutrophil Cytoplasmic Autoantibodies Specific to Bactericidal/Permeability-Increasing Protein: A Cross-Road Between Prolonged Gram-Negative Bacterial Infections and Ulcerative Colitis/Primary Sclerosing Cholangitis.

Diagnostics (Basel, Switzerland)
2025

Long-Term Clinical and Endoscopic Outcomes of Crohn's Disease Following Liver Transplantation: A Multicenter Cohort Study.

Biomedicines
2025

Clinical and Prognostic Factors Associated With Inflammatory Bowel Disease in a Highly Admixed Population With Primary Sclerosing Cholangitis.

Journal of clinical gastroenterology
2025

Molecular mechanisms and genetic features of cholangiocarcinoma: implications for targeted therapeutic strategies.

Precision clinical medicine
2025

Organoids in biliary research: insights into developmental signaling and applications in disease modeling.

Frontiers in cell and developmental biology
2025

68Ga-FAPI-46 PET/CT in Primary Sclerosing Cholangitis and Suspected Cholangiocarcinoma.

Journal of nuclear medicine : official publication, Society of Nuclear Medicine
2025

Human T-Lymphotropic Virus Type 1-Associated Myelopathy With Autoimmune Cholangiopathy: An Unusual Immune Conundrum in a Young Patient.

Case reports in infectious diseases
2025

Chance of pouch surgery after colectomy for ulcerative colitis based on pelvic pouch volumes at the colectomy hospital, a Swedish national cohort study.

Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland
2025

The regulatory roles of pparα on hepatic drug-metabolizing enzymes in primary sclerosing cholangitis Syrian hamsters.

Biochemical pharmacology
2025

Advances in Construction and Application of Biliary Organoids.

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2025

Chronic abdominal pain secondary to portosystemic collateral vessels: A case report and review of etiologies.

International journal of surgery case reports
2025

Establishing the Clinical Utility of Glucagon-Enhanced MRCP for Improved Hepatopancreatobiliary Assessment.

Journal of clinical medicine
2025

Longitudinal Comparison of Currently Used Risk Scores for Prognostication of Primary Sclerosing Cholangitis (PSC) in a Hungarian Bicenter PSC Cohort.

Diagnostics (Basel, Switzerland)
2025

Development and Validation of a Scaffold-Free Human Multilineage Spheroid Model for Early Stage Cholangiopathies Driven by Cholangiocyte Senescence.

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

Sarcopenia-A Valuable Imaging Biomarker for Disease Progression in Patients With Primary Sclerosing Cholangitis (PSC)?

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

Causal relationship of body mass index with autoimmune liver diseases mediated by circulating cytokines: A Mendelian randomization study.

Medicine
2025

Revealing the enigmatic interplay between helicobacter pylori infection and biliary system diseases using Mendelian randomization analysis.

Medicine
2025

Primary Sclerosing Cholangitis Worsens Prognosis in Patients With Inflammatory Bowel Disease: A Propensity-Matched Cohort Study.

United European gastroenterology journal
Ver todos os 3.373 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Doença com base genética

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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

Respostas geradas por IA a partir das fontes citadas

É uma doença rara caracterizada pela inflamação e destruição progressiva dos canais biliares intra e extra-hepáticos. Essa lesão contínua gera acúmulo de bile, fibrose no tecido do fígado e pode avançar para cirrose e insuficiência hepática.

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:171
    Orphanet
  2. GARD:1280
    GARD (NIH)
  3. Q1058608
    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 esclerosante primária. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/colangite-esclerosante-primaria

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

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Colangite esclerosante primária
Compêndio · Raras BR

Colangite esclerosante primária

ORPHA:171 · MONDO:0013433
Prevalência
1-9 / 100 000
Herança
Multigenic/multifactorial
CID-10
K83.0 · Colangite
CID-11
Ensaios
41 ativos
Início
Adolescent, Adult, Childhood, Elderly
Prevalência
7.84 (Europe)
MedGen
UMLS
C0566602
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
Genética mendeliana
fonte: OMIM
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Rara