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.
Introdução
O que você precisa saber de cara
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.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 20 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 41 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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.
Transcription factor that plays a critical role in innate immunity by activating expression of type I interferon (IFN) IFNA and INFB and inflammatory cytokines downstream of endolysosomal toll-like receptors TLR7, TLR8 and TLR9 (PubMed:11303025, PubMed:15695821, PubMed:22412986, PubMed:25326418, PubMed:32433612). Regulates the transcription of type I IFN genes (IFN-alpha and IFN-beta) and IFN-stimulated genes (ISG) by binding to an interferon-stimulated response element (ISRE) in their promoters
CytoplasmNucleus
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.
Functions as an interleukin receptor which binds interleukin-12 with low affinity and is involved in IL12 transduction. Associated with IL12RB2 it forms a functional, high affinity receptor for IL12. Also associates with IL23R to form the interleukin-23 receptor which functions in IL23 signal transduction probably through activation of the Jak-Stat signaling cascade
Membrane
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.
Heterodimerizes with IL12B to form the IL-12 cytokine or with EBI3/IL27B to form the IL-35 cytokine (PubMed:8605935, PubMed:8943050). IL-12 is primarily produced by professional antigen-presenting cells (APCs) such as B-cells and dendritic cells (DCs) as well as macrophages and granulocytes and regulates T-cell and natural killer-cell responses, induces the production of interferon-gamma (IFN-gamma), favors the differentiation of T-helper 1 (Th1) cells and is an important link between innate res
Secreted
Importin, which transports target proteins into the nucleus (PubMed:10366588, PubMed:10713112, PubMed:11517331, PubMed:12628928, PubMed:24449914). Specifically mediates the nuclear import of splicing factor serine/arginine (SR) proteins, such as RBM4, SFRS1 and SFRS2, by recognizing phosphorylated SR domains (PubMed:10366588, PubMed:10713112, PubMed:11517331, PubMed:12628928, PubMed:24449914). Also mediates the nuclear import of serine/arginine (SR) protein CPSF6, independently of CPSF6 phosphor
Nucleus envelopeCytoplasm
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.
Sequence specific transcriptional activator which binds to the PU-box, a purine-rich DNA sequence (5'-GAGGAA-3') that can act as a lymphoid-specific enhancer. Promotes development of plasmacytoid dendritic cells (pDCs), also known as type 2 DC precursors (pre-DC2) or natural interferon (IFN)-producing cells. These cells have the capacity to produce large amounts of interferon and block viral replication. May be required for B-cell receptor (BCR) signaling, which is necessary for normal B-cell de
NucleusCytoplasm
Transcriptional coactivator that specifically associates with either POU2F1/OCT1 or POU2F2/OCT2 (PubMed:7859290). It boosts the POU2F1/OCT1 mediated promoter activity and to a lesser extent, that of POU2F2/OCT2 (PubMed:7779176). It recognizes the POU domains of POU2F1/OCT1 and POU2F2/OCT2 (PubMed:7779176). It is essential for the response of B-cells to antigens and required for the formation of germinal centers (PubMed:7623806, PubMed:7859290). Regulates IL6 expression in B cells as POU2F2/OCT2
Nucleus
Metalloprotease involved in sperm function, possibly by modulating the processes of fertilization and early embryonic development. Degrades a broad variety of small peptides with a preference for peptides shorter than 3 kDa containing neutral bulky aliphatic or aromatic amino acid residues. Shares the same substrate specificity with MME and cleaves peptides at the same amide bond (By similarity)
MembraneSecreted
Receptor for TNFRSF25 and TNFRSF6B. Mediates activation of NF-kappa-B. Inhibits vascular endothelial growth and angiogenesis (in vitro). Promotes activation of caspases and apoptosis
MembraneSecreted
Medicamentos aprovados (FDA)
2 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
162 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 1 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
9 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Colangite biliar primária
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 2.942
Anti-MRPS27 Antibody: A Novel Diagnostic Biomarker for Autoimmune Hepatitis Identified via a Human Proteome Microarray.
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.
Geographic distribution and demographic patterns of primary biliary cholangitis and autoimmune hepatitis in Colombia: a national population-based administrative study (2018-2024).
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.
Consensus statements of the Hellenic Autoimmune Liver Diseases Study Group on the diagnosis and current management of primary biliary cholangitis.
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.
Liver transplant outcomes in primary biliary cholangitis and metabolic dysfunction-associated steatotic liver disease overlap.
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.
Mast Cells at the Crossroad of Gut-Derived Signals Through Aryl Hydrocarbon Receptor Activation: A Microbial-Immune Dialogue in Liver Inflammation with Therapeutic Perspectives.
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
From unmet needs to new possibilities: PPAR-targeted therapies in the journey of people living with Primary Biliary Cholangitis.
When to Consider AIH-PBC OverlapSyndrome in the Pathology Report.
Medical Costs Increase Over Time for Primary Biliary Cholangitis Patients: A 20-Year Population-Based Study.
A Nationwide Assessment of Anticholestatic Therapy Uptake in Patients With Primary Biliary Cholangitis: Opportunities for Optimisation.
📚 EuropePMC1.798 artigos no totalmostrando 196
Research progress on the role of gut microbiota dysbiosis in the pathogenesis of immune-mediated liver diseases.
Frontiers in immunologyAnti-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 HepatologyMeta-Analysis: PPAR Agonists for Pruritus and Quality of Life in Primary Biliary Cholangitis.
Alimentary pharmacology & therapeuticsGeographic distribution and demographic patterns of primary biliary cholangitis and autoimmune hepatitis in Colombia: a national population-based administrative study (2018-2024).
Annals of gastroenterologyConsensus statements of the Hellenic Autoimmune Liver Diseases Study Group on the diagnosis and current management of primary biliary cholangitis.
Annals of gastroenterologyZoledronic 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 DensitometryPrimary Biliary Cholangitis and Prognostic Models-Reply.
JAMAPrimary Biliary Cholangitis and Prognostic Models.
JAMALetter: The association between liver biochemistry, biliary and fibrosis in primary biliary cholangitis.
Alimentary pharmacology & therapeuticsPrimary Biliary Cholangitis Increases Mortality Irrespective of Presence or Absence of Cirrhosis.
Liver international : official journal of the International Association for the Study of the LiverProbiotic Lactobacillus rhamnosus mitigates PBC-like features in Mcpip1-deficient mice via modulation of gut-liver crosstalk.
Biochimica et biophysica acta. Molecular basis of diseaseDual Positivity for AMA/AMA-M2 and Anti-gp210/sp100 Shows Highest Diagnostic Value for Primary Biliary Cholangitis.
Alimentary pharmacology & therapeutics[Bullous pemphigoid and primary biliary cholangitis, a rare association with therapeutic challenge].
Orvosi hetilapLiver transplant outcomes in primary biliary cholangitis and metabolic dysfunction-associated steatotic liver disease overlap.
Annals of hepatologyConjugated bile acids facilitate cholangiocyte senescence to promote cholestatic liver diseases via STING signaling.
Journal of advanced researchMast Cells at the Crossroad of Gut-Derived Signals Through Aryl Hydrocarbon Receptor Activation: A Microbial-Immune Dialogue in Liver Inflammation with Therapeutic Perspectives.
CellsRisk factors for mortality in patients with primary biliary cholangitis: a nomogram to predict 5-year survival.
Frontiers in gastroenterology (Lausanne, Switzerland)Integrative Mendelian randomization and experimental validation unveil novel druggable targets in primary biliary cholangitis.
European journal of pharmacologyMetabolic syndrome and its individual components but not MASLD are associated with advanced fibrosis in primary biliary cholangitis.
Therapeutic advances in gastroenterologyConstructing a protein-protein interaction network for autoimmune liver diseases by integrating pQTL, rQTL, and mediation analyses.
Naunyn-Schmiedeberg's archives of pharmacologyOptimising Primary thErapy in pRimAry biliary cholangitis (OPERA): protocol for a randomised, double-blind, placebo-controlled trial of enhanced primary therapy with obeticholic acid.
BMJ openDual cut-offs and beyond: Expanding the role of transient elastography in primary biliary cholangitis.
World journal of gastroenterologyFatigue and circadian rhythm in non-cirrhotic primary biliary cholangitis: An exploratory comparison with primary sclerosing cholangitis and healthy controls.
World journal of hepatologyAdipokine profiles reflect metabolic dysfunction but not fibrosis in patients with primary biliary cholangitis.
World journal of hepatologyHigh-intensity hydrophilic versus lipophilic statins in patients with primary biliary cholangitis: a multi-institutional analysis.
BMC gastroenterologyCan liver biopsy be spared for the diagnosis of autoimmune hepatitis in selected children? A multicenter retrospective study.
Annals of hepatology[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 hepatologyThe PBC Ireland patient registry: study protocol for a national platform on primary biliary cholangitis.
Therapeutic advances in rare diseaseAustralian best practice recommendations for transjugular intrahepatic portosystemic shunt (TIPS) in portal hypertension: a consensus statement.
Hepatology internationalImpact of Alkaline Phosphatase Normalization on Complication-Free Survival in Primary Biliary Cholangitis.
The American journal of gastroenterologyPyroptosis Plays a Key Role Through Macrophages in Primary Biliary Cholangitis of Mice.
BioMed research internationalReconciling the role of ileal bile acid transporter inhibitors as symptom-directed therapy in primary biliary cholangitis: a focused meta-analysis.
European journal of gastroenterology & hepatologySomatostatin Effect on Growth Factors in Hepatocellular Carcinoma.
Current issues in molecular biologyHow do oral pathogenic bacteria potentially contribute to inflammatory processes in autoimmune liver disease? Results from a systematic review.
Clinics and research in hepatology and gastroenterologyVisualization analysis of the use of traditional Chinese medicine in the diagnosis and treatment of rare diseases in mainland China based on CiteSpace.
Intractable & rare diseases researchComments on: Pre-Diagnosis Alkaline Phosphatase and Antimitochondrial Antibody Positivity Vary by Race/Ethnicity Among Patients With Primary Biliary Cholangitis.
Journal of gastroenterology and hepatologyThe ethics of unlicensed therapy use in primary biliary cholangitis: a counter view.
Nature reviews. Gastroenterology & hepatologyMechanistic Rationale of Seladelpar's Clinical Efficacy through the Selective PPAR-δ Activation in Primary Biliary Cholangitis.
Pharmacological researchStains in primary biliary cholangitis: Untangling promise from bias.
Hepatology (Baltimore, Md.)Rheumatologic Manifestations of Patients With Type B Insulin Resistance.
Arthritis care & researchLong-Term Use of Fenofibrate as Second-Line Therapy in Primary Biliary Cholangitis: A Retrospective Study.
Alimentary pharmacology & therapeuticsHuman leukocyte antigen variants and clinical features of primary biliary cholangitis: Cumulative contributions.
World journal of clinical casesCost of hepatic decompensation and liver transplantation events in primary biliary cholangitis: a retrospective observational study.
Journal of comparative effectiveness researchPlasma Biomarkers of Senescence in Cholestatic Liver Disease: A Signature of Risk Stratification and Progression.
Journal of gastroenterology and hepatologyMesenchymal stromal cell administration promotes macrophage-mediated bile duct regeneration.
Regenerative therapyPrimary Biliary Cholangitis Pathogenesis: A Pathophysiology-Based Narrative Review.
International journal of molecular sciencesA Review on Farnesoid X Receptor (FXR) Modulators Focusing on Benzimidazole Scaffold.
Molecules (Basel, Switzerland)The Involvement of Apoptosis Inhibitor of Macrophage in the Disease Severity of Primary Biliary Cholangitis.
Journal of clinical medicinePrimary biliary cholangitis with inflammation involving zone 3 of the liver has a poor response to ursodeoxycholic acid treatment: a retrospective cohort study.
BMC gastroenterologyClinicopathological features of hepatobiliary cells in primary biliary cholangitis patients with incomplete response to ursodeoxycholic acid.
BMC gastroenterologyExploration and discovery of treatment targets for primary biliary cholangitis based on plasma and cerebrospinal fluid proteomics: A multicenter mendelian randomization study.
PloS oneDirect bilirubin was superior to total bilirubin in predicting the long-term outcomes of patients with primary biliary cholangitis.
BMC gastroenterologyEvaluation 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 GastroenterologyElevated 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 GastroenterologyLongitudinal multi-omics pilot study: Small sample size human model of gut microbiota-mitochondrial metabolic dysregulation in primary biliary cholangitis.
Microbiological researchPrimary Biliary Cholangitis-associated Osteoporosis: Contemporary Review of Pathogenesis and Management.
Journal of clinical and translational hepatologyAcute Graft-Versus-Host Disease After Deceased-Donor Liver Transplantation: A Case Report.
The American journal of case reportsA multifaceted analysis of OTUD5 integrated MAVS in innate immunity of Primary Biliary Cholangitis.
PloS oneAssociation between type 2 diabetes and autoimmune liver disease: An integrated analysis of Mendelian randomization and clinical samples.
MedicineIleal 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 LiverAutoimmune hepatitis overlap syndrome: Clinical presentation, treatment response, and disease evolution: Tertiary centre experience.
Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology AssociationLipidome remodeling in primary biliary cholangitis.
Lipids in health and diseaseUse of Ursodeoxycholic Acid and Cancer Risk for Patients With Primary Biliary Cholangitis.
JAMA network openAssociation between statin use and hepatic decompensation in patients with primary biliary cholangitis: A target trial emulation study.
Hepatology (Baltimore, Md.)Primary Biliary Cholangitis: Novel and Emerging Therapies.
Gastroenterology & hepatologyExploring Current and Emerging Therapies for Patients With Primary Biliary Cholangitis.
Gastroenterology & hepatologyDiagnostic potential of salivary biomarkers for primary biliary cholangitis: a systematic review.
Frontiers in medicineQuality 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 LiverAlkaline 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 AssociationIGFBP4 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 LiverBile acids as therapeutic agents.
Frontiers in pharmacologyAwareness of Primary Biliary Cholangitis Among Turkish Physicians: A Cross-Sectional, Multicenter, Web-Based Survey.
Journal of clinical medicineIdentification of Differentially Expressed Genes and Molecular Pathways Involved in Primary Biliary Cholangitis Using RNA-Seq.
GenesElevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker.
BiomoleculesKupffer cell autophagy emerges as a central regulator of immune dysregulation in primary biliary cholangitis.
GutSeladelpar 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 AssociationOrgan-specific autoimmunity in primary Sjögren's disease.
Scandinavian journal of rheumatologyThe role of gut microbiota in the pathogenesis of primary biliary cholangitis: an immunological perspective.
Expert review of clinical immunologyMetabolic Dysfunction-Associated Steatohepatitis With Positive Antimitochondrial M2 Antibodies: Diagnostic Pitfall and Importance of Follow-Up.
CureusHigher sun exposure is associated with a reduced risk of primary biliary cholangitis.
International journal of epidemiologyATF3 Mediates the Immunoregulatory Role of the Tim-3 Pathway in Primary Biliary Cholangitis.
Journal of gastroenterology and hepatologyFactors associated with recompensation and criteria for etiological suppression in patients with primary biliary cholangitis.
Journal of gastroenterologyFCRL3 as a potential link between Benzo[a]pyrene exposure and primary biliary cholangitis: insights from comparative toxicogenomics and multi-omics analysis.
BMC gastroenterologyImpact of Type 2 Diabetes Mellitus on Liver Fibrosis and Hepatic Steatosis in Patients with Primary Biliary Cholangitis: A Longitudinal Study.
Digestive diseases and sciencesDysgeusia-Driven Nutritional Decline in Cirrhosis: A Case of Autoimmune Hepatitis and Primary Biliary Cholangitis.
CureusInferior Vena Cava-Atrial Anastomosis in Liver Transplant Recipient with Inferior Vena Cava Occlusion: A Case Report and Literature Review.
Journal of clinical medicineRedox Modulation in Hepatic Fibrosis: Translating NOX1/4 Inhibition to Therapy.
International journal of molecular sciencesEvaluating 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[A case of recurrent hypoglycemic coma episodes associated with primary biliary cholangitis and literature review].
Zhonghua nei ke za zhiGenetic evidence supports primary biliary cholangitis as a risk factor for lacunar stroke.
iScienceVariation in microbiome and metabolites is associated with advantageous effects of cholestyramine on primary biliary cholangitis with pruritus.
Microbiology spectrumAnti-mitochondrial M2 antibody links to cardiac involvement and immune-mediated inflammatory myopathy-like pathology in myositis.
Rheumatology (Oxford, England)Cell-specific epigenome-wide DNA methylation in peripheral CD4(+) lymphocytes from patients with primary biliary cholangitis.
Journal of translational autoimmunityPrimary Biliary Cholangitis With Incomplete Response to Conventional Therapies But Had Complete Response to Baricitinib: A Case Report.
Case reports in hepatologyDecoding the Gut Microbiome in Primary Sjögren's Syndrome and Primary Biliary Cholangitis: Shared Dysbiosis, Distinct Patterns, and Associations with Clinical Features.
MicroorganismsLiver Innervation in Health and Disease: Neuroimmune-Neurovascular Interface and Future Therapeutic Implications.
BiomedicinesClinical characteristics and factors associated with liver stiffness in patients with autoimmune liver disease: a cross-sectional study from the Kashgar region.
BMC gastroenterologyThe Personalized Management of Primary Biliary Cholangitis in the Era of Precision Medicine: Current Challenges and Future Perspectives.
Journal of personalized medicineGastric Squamous Cell Carcinoma as a Cause of Clinically Unstable Gastrointestinal Bleeding.
ACG case reports journalVitamin D3 attenuates hepatic inflammation in primary biliary cholangitis by inhibiting TLR4/NF-κB mediated M1 macrophage polarization.
Scientific reportsPBC-AIH variant syndrome: emerging new terminology and a new approach to diagnosis and management.
Current opinion in gastroenterologyThe causal relationship between inflammatory bowel disease and primary biliary cholangitis: A bidirectional two-sample Mendelian randomization study.
MedicineAutoimmune thyroid disease and human health: a systematic review of Mendelian randomization studies.
Frontiers in immunologySystemic inflammatory response index: a novel predictor for cardiovascular disease risk in patients with primary biliary cholangitis.
European journal of medical researchClinical outcomes and early- prognostic biomarkers of primary biliary cholangitis with ductopenia.
Frontiers in immunologyAssociation between quality of life and inflammation and fibrosis markers in primary biliary cholangitis over three-year follow-up.
Scandinavian journal of gastroenterologyPleiotropic gene mapping reveals shared immune regulatory hubs across 15 autoimmune diseases.
Human immunologyMechanistic roles and therapeutic potential of bacteriophages in inflammatory gastrointestinal diseases.
Microbiome research reportsUbiquitin D Correlates with Disease Severity and T Cell Infiltration in Cholestasis: Evidence from Integrated Bioinformatics and Experimental Analyses.
International journal of medical sciencesPrimary biliary cholangitis (PBC): evolving approaches and expert perspectives.
Expert review of gastroenterology & hepatologyAssessing the concordance between centromere AC-3 immunofluorescence pattern and anti-centromere protein-B antibody, and analyzing clinical correlates of dual positivity: A retrospective study.
MedicineTransjugular intrahepatic portosystemic shunt for hepatic sinusoidal obstruction syndrome with primary biliary cholangitis and alcoholic liver disease: a case report.
Frontiers in medicineAutoimmune liver diseases in Latin America: Current landscape and challenges.
Hepatology communicationsA novel cell-permeable LOXL2 inhibitor PAT-1251 potently suppresses biliary liver fibrosis via collagen crosslinking-dependent and -independent mechanisms.
Hepatology communicationsPruritus and health-related quality of life in chronic liver disease: a longitudinal, survey-based cohort study.
BMJ open gastroenterologyRandomized Controlled Trial Evidence on Peroxisome Proliferator-Activated Receptor (PPAR) Agonists in Primary Biliary Cholangitis: A Systematic Review and Meta-Analysis.
International journal of hepatologyAutoantibodies in Primary Biliary Cholangitis: From Classical Markers to Emerging Targets.
Journal of clinical medicineFrom 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)Autophagy of Kupffer cells modulates CD8+ T cell activation in primary biliary cholangitis.
GutNecrotizing Fasciitis Caused by Serotype 7C Streptococcus pneumoniae in Overwhelming Post-splenectomy Infection.
Internal medicine (Tokyo, Japan)Beyond bones: Revisiting the role of vitamin D in chronic liver disease.
World journal of hepatologyUnveiling the Spectrum of Liver Endurance in Systemic Lupus Erythematosus: A Single-Center Experience From Kashmir, India.
CureusClinical 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 GastroenterologieAssociation between elevations in alkaline phosphatase and healthcare utilization and costs among individuals with primary biliary cholangitis in the United States.
Journal of medical economicsEffectiveness and tolerability of bezafibrate in primary biliary cholangitis - a nationwide real-world study.
The American journal of gastroenterologyDiagnostic Pitfalls in Primary Biliary Cholangitis: Delta Bilirubin and Lipoprotein-X Interferences.
Clinical laboratoryQuantitative detection of anti-centromere antibodies in primary biliary cholangitis: value of chemiluminescence immunoassay.
Scandinavian journal of clinical and laboratory investigationTumor necrosis factor alpha-induced protein 3: Biomarker discovery and therapeutic advancement in primary biliary cholangitis.
World journal of hepatologyRisk factors for autoimmune liver disease recurrence after liver transplantation.
World journal of transplantationTriple autoimmune overlap syndrome of the liver: a rare case of concomitant PBC, PSC, and AIH.
Future science OABile Acid Metabolites Control Th17/Treg Balance in Primary Biliary Cholangitis: Mechanisms of Disease Progression and Therapeutic Targets.
Journal of gastroenterology and hepatologyEconomic Burden of Primary Biliary Cholangitis by Line of Therapy in the United States.
Advances in therapySmoking and liver diseases: an updated review of pathogenesis, progression, and therapeutic implications.
Clinical and experimental medicineAssociation between ABO blood group system and autoimmune liver disease.
Frontiers in medicineAssociation of Individual or Family History of Autoimmune Disease With Future Development of Type 1 Diabetes.
Diabetes/metabolism research and reviewsAutoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.
Hepatology internationalFracture events in patients with primary biliary cholangitis during treatment with seladelpar in the phase III RESPONSE trial.
Hepatology communicationsMendelian randomization study of the causal relationship between autoimmune diseases and anemia using proteomic and genetic data.
Journal of translational autoimmunityAMA-Negative Primary Biliary Cholangitis and Autoimmune Hepatitis Overlap Syndrome Presenting With Bullous Pemphigoid: A Case Report.
Clinical case reportsAltered sphingolipid profile in primary biliary cholangitis: associations with fibrosis and inflammation.
Scientific reportsGolexanolone Attenuates Neuroinflammation, Fatigue, and Cognitive and Motor Impairment in Diverse Neuroinflammatory Disorders.
Pharmaceuticals (Basel, Switzerland)Profile of Patients with Primary Biliary Cholangitis and Evaluation of Response to Ursodeoxycholic Acid in a Romanian Center-Retrospective Study.
Journal of clinical medicineProteomics identifies complement protein signatures in patients with primary biliary cholangitis.
European journal of medical researchSystemic PCSK9 elevation characterises autoimmune liver disease across sexes.
Scientific reportsOptimizing Care in Primary Biliary Cholangitis: Current Treatments and the Second-Line Decision.
Digestive diseases and sciencesReal-world performance of transient elastography in assessing advanced fibrosis in Chinese patients with primary biliary cholangitis.
World journal of gastroenterologyPrimary biliary cholangitis. Treatment options in 2025. A narrative review.
Frontiers in immunologyLipoprotein X-Induced Hyperlipidemia Following Liver Transplantation in the Setting of Persistent Cholestasis and Antibody-Mediated Rejection.
ACG case reports journalExploring Maralixibat for Treatment-Resistant Pruritus in Intrahepatic Cholestasis of Pregnancy and Primary Biliary Cholangitis: A Case Report.
The American journal of case reportsAssociation 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 researchWhat the European Reference Network Registry for Rare Liver Diseases Tells Us About Primary Biliary Cholangitis in European Practice.
United European gastroenterology journalThe risk and prevalence of hepatic diseases in psoriasis: A systematic review and meta-analysis.
Journal of the European Academy of Dermatology and Venereology : JEADVCase Report: Novel IRF2BP2 variant in a Japanese patient with impaired B-cell differentiation, Th1 polarization, and systemic immune dysregulation.
Frontiers in immunologyHSC-derived exosomal miR-122-5p inhibits EMT and fibrosis of intrahepatic biliary epithelial cells to alleviate primary biliary cholangitis.
Frontiers in immunologySimultaneous degenerative changes in 2 hepatic cavernous hemangiomas observed over 23 years of follow-up: A case report and review of the literature.
Radiology case reportsHepatocyte iron suppresses liver fibrosis via fibrolytic neutrophil recruitment in cholestasis.
JHEP reports : innovation in hepatologyNumb-exon3 and full length Numb equivalently alleviate cholestatic liver fibrosis by inhibiting ductular reaction.
Scientific reportsOptimizing clinic consultations in primary biliary cholangitis: International consensus recommendations.
Hepatology communicationsSpanish 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 hepatologiaPPARevolution. First PPARδ Agonist and a Dual PPARα/PPARδ Activator Approved for the Treatment of Primary Biliary Cholangitis.
Journal of cellular and molecular medicineTepotinib-Induced Cholangitis in a Patient With Non-Small Cell Lung Cancer: A Case Report.
Respirology case reportsLetter on 'Non-Response to Obeticholic Acid Is Associated With Heightened Risks of Developing Clinical Events in Primary Biliary Cholangitis'.
Alimentary pharmacology & therapeuticsCausal effects of immune cell phenotypes on the risk of autoimmune liver diseases: a bidirectional two-sample Mendelian randomization study.
Translational gastroenterology and hepatologyProgress and prospects of gut microbiota-targeted therapy for primary biliary cholangitis.
Gut pathogensPrimary Biliary Cholangitis.
JAMAPercutaneous Transvenous Retrograde Embolization of Adhesion-Related Small Bowel Varices: A Case Report.
CureusBeyond Ursodeoxycholic Acid: A Comprehensive Review of Second-Line Agents in Primary Biliary Cholangitis.
CureusFXR-targeted drug discovery: Recent advances and therapeutic perspectives.
European journal of medicinal chemistryReevaluating the clinical course of AMA-positive patients with normal liver enzymes: A large retrospective cohort study.
Annals of hepatologyTreatment of Refractory Pruritic Dermatitis in the Setting of Primary Biliary Cholangitis and CREST Syndrome With Upadacitinib.
Journal of drugs in dermatology : JDDMicrobiome and gut-liver interactions: From mechanisms to therapies.
World journal of gastroenterologySingle-Nuclei RNA Sequencing Shows the Engagement of PPAR-Delta Target Genes Primarily in Hepatocytes and Cholangiocytes by the Selective PPAR-Delta Agonist Seladelpar.
PPAR researchLetter: Optimizing Risk Stratification in Primary Biliary Cholangitis-The Role of Biochemical Response as a Dynamic Predictor.
Alimentary pharmacology & therapeuticsLinerixibat in patients with primary biliary cholangitis and cholestatic pruritus (GLISTEN): a randomised, multicentre, double-blind, placebo-controlled, phase 3 trial.
The lancet. Gastroenterology & hepatologyIleal bile acid transporter inhibitors for cholestatic pruritus in primary biliary cholangitis.
The lancet. Gastroenterology & hepatologyTreatment Persistence, Normal Alkaline Phosphatase and Clinical Outcomes in Primary Biliary Cholangitis.
Alimentary pharmacology & therapeuticsResearch Communication: Real-World Clinical Experience With Seladelpar in Primary Biliary Cholangitis.
Alimentary pharmacology & therapeuticsMacrophages in Autoimmune Liver Diseases: From Immune Homeostasis to Precision-Targeted Therapy.
BiomedicinesSecond-Line Therapies in Primary Biliary Cholangitis: A Comparative Review of Obeticholic Acid, Fibrates, Seladelpar, and Elafibranor.
BiomedicinesNoninvasive elastography-based assessment of liver fibrosis in primary biliary cholangitis.
Annals of hepatologyThe 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 & immunologyMulti-omics profiling uncovers paradoxical Epstein-Barr virus involvement in autoimmune liver disease pathogenesis.
AMB ExpressAutophagy Sustained the Activation and Survival of Liver-infiltrating CD8+ T Cells in Primary Biliary Cholangitis.
Immunological investigationsThe role of mitochondria-related key genes in primary biliary cholangitis was analyzed based on transcriptome sequencing data.
Frontiers in immunologyIdentification of galangin as a therapeutic candidate for primary biliary cholangitis via systematic druggable genome-wide Mendelian randomization analysis and experimental validation.
Frontiers in pharmacologyRecurrent 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 SurgeonsHistological characteristics of primary biliary cholangitis in patients with incomplete responses to ursodeoxycholic acid.
Human pathologyAssessment 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 gastroenterologyTrends in Primary Biliary Cholangitis: Prospective Cohort Study From the European Reference Network Registry (R-LIVER).
United European gastroenterology journalMixed Multiple Autoimmune Syndrome Type 3 With Coexistence of Primary Biliary Cholangitis, Inflammatory Myopathy, and Chronic Thyroiditis: A Case Report.
CureusAdvancing the management of primary biliary cholangitis: From pathogenesis to emerging therapies.
World journal of clinical casesInnate immunity of bile and cholangiocytes in primary biliary cholangitis.
Frontiers in immunologyFuture Treatment Options for Managing Primary Sclerosing Cholangitis and Cholestatic Pruritus.
Clinics in liver diseaseRecent Advances in Primary Biliary Cholangitis Treatment.
Clinics in liver diseaseCauses, associated exposures, and outcomes of cirrhosis and hepatocellular carcinoma in Malawi: an observational cohort and case-control study.
The Lancet. Global healthThe effects of lifestyle factors on primary biliary cholangitis ascertained by two-sample Mendelian randomization.
The American journal of the medical sciencesAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- 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· PMID 41871135mais citado
- Geographic distribution and demographic patterns of primary biliary cholangitis and autoimmune hepatitis in Colombia: a national population-based administrative study (2018-2024).
- Consensus statements of the Hellenic Autoimmune Liver Diseases Study Group on the diagnosis and current management of primary biliary cholangitis.
- Liver transplant outcomes in primary biliary cholangitis and metabolic dysfunction-associated steatotic liver disease overlap.
- Mast Cells at the Crossroad of Gut-Derived Signals Through Aryl Hydrocarbon Receptor Activation: A Microbial-Immune Dialogue in Liver Inflammation with Therapeutic Perspectives.
- From unmet needs to new possibilities: PPAR-targeted therapies in the journey of people living with Primary Biliary Cholangitis.
- When to Consider AIH-PBC OverlapSyndrome in the Pathology Report.
- Correction to "Characterisation and Clinical Relevance of Tertiary Lymphoid Structures in Primary Biliary Cholangitis".
- Medical Costs Increase Over Time for Primary Biliary Cholangitis Patients: A 20-Year Population-Based Study.
- A Nationwide Assessment of Anticholestatic Therapy Uptake in Patients With Primary Biliary Cholangitis: Opportunities for Optimisation.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:186(Orphanet)
- MONDO:0005388(MONDO)
- GARD:7459(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- 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.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
