É uma doença crônica e inflamatória do fígado, de origem autoimune (o sistema de defesa do corpo ataca o próprio órgão por engano). Ela é caracterizada pelo estreitamento e pelo endurecimento da parte interna dos canais que transportam a bile. É comum ser encontrada em pessoas que têm retocolite ulcerativa. Os sinais e sintomas incluem: icterícia (pele e olhos amarelados), cansaço intenso e má absorção de nutrientes (o corpo tem dificuldade para absorver o que é comido). A doença pode levar à cirrose e à falência (parada de funcionamento) do fígado.
Introdução
O que você precisa saber de cara
A colangite esclerosante primária é uma condição que afeta os ductos biliares. Esses ductos transportam a bile (um fluido que ajuda a digerir gorduras) do fígado, onde a bile é produzida, para a vesícula biliar, onde é armazenada, e para o intestino delgado, onde auxilia na digestão. A colangite esclerosante primária ocorre devido à inflamação nos ductos biliares (colangite) que leva à formação de cicatrizes (esclerose) e ao estreitamento dos ductos. Como resultado, a bile não pode ser liberada para a vesícula biliar e o intestino delgado, e acumula‑se no fígado.
A colangite esclerosante primária costuma ser diagnosticada por volta dos 40 anos de idade e, por razões desconhecidas, afeta os homens duas vezes mais frequentemente que as mulheres. Muitas pessoas não apresentam sinais ou sintomas da condição quando são diagnosticadas, mas exames de sangue de rotina revelam problemas hepáticos. Quando são perceptíveis, os primeiros sinais e sintomas da colangite esclerosante primária incluem cansaço extremo (fadiga), desconforto abdominal e coceira intensa (prurido). À medida que a condição piora, as pessoas com a condição podem desenvolver amarelamento da pele e da parte branca dos olhos (icterícia) e aumento do baço (esplenomegalia). Eventualmente, o acúmulo de bile danifica as células hepáticas, causando doença hepática crônica (cirrose) e insuficiência hepática. Sem bile disponível para digeri‑las, as gorduras passam pelo corpo. Como consequência, podem ocorrer perda de peso e deficiências de vitaminas que são absorvidas juntamente com as gorduras e armazenadas nelas (vitaminas lipossolúveis). Uma vitamina lipossolúvel chamada vitamina D ajuda a absorver cálcio e a endurecer os ossos, e a falta dessa vitamina pode causar afinamento dos ossos (osteoporose) em pessoas com colangite esclerosante primária.
A colangite esclerosante primária costuma estar associada a outra condição chamada doença inflamatória intestinal, que se caracteriza pela inflamação dos intestinos que provoca úlceras (feridas abertas) nos intestinos e dor abdominal. No entanto, a razão desse vínculo não é clara. Aproximadamente 70 % das pessoas com colangite esclerosante primária têm doença inflamatória intestinal, mais comumente uma forma da condição conhecida como colite ulcerativa. Além disso, pessoas com colangite esclerosante primária têm maior probabilidade de ter um distúrbio autoimune, como diabetes tipo 1, doença celíaca ou doença da tireoide, do que pessoas sem a condição. Distúrbios autoimunes ocorrem quando o sistema imunológico funciona de forma inadequada e ataca os tecidos e órgãos do corpo. Pessoas com colangite esclerosante primária também apresentam risco aumentado de desenvolver câncer, particularmente câncer dos ductos biliares (colangiocarcinoma).
Fonte: MedlinePlus Genetics (NLM/NIH), traduzidoÉ uma doença crônica e inflamatória do fígado, de origem autoimune (o sistema de defesa do corpo ataca o próprio órgão por engano). Ela é caracterizada pelo estreitamento e pelo endurecimento da parte interna dos canais que transportam a bile. É comum ser encontrada em pessoas que têm retocolite ulcerativa. Os sinais e sintomas incluem: icterícia (pele e olhos amarelados), cansaço intenso e má absorção de nutrientes (o corpo tem dificuldade para absorver o que é comido). A doença pode levar à cirrose e à falência (parada de funcionamento) do fígado.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 32 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 91 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
6 genes identificados com associação a esta condição.
Dyslexia 2
A relatively common, complex cognitive disorder characterized by an impairment of reading performance despite adequate motivational, educational and intellectual opportunities. It is a multifactorial trait, with evidence for familial clustering and heritability.
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.
Ichthyosis-sclerosing cholangitis neonatal syndrome
A rare autosomal recessive complex ichthyosis syndrome characterized by scalp hypotrichosis, scarring alopecia, mild diffuse ichthyosis, sclerosing cholangitis and leukocyte vacuolization.
Variantes genéticas (ClinVar)
190 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 232 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
11 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
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 3.991
Dysbiotic microbiota trigger colitis-associated colorectal cancer and imprint a distinctive bile acid profile in a PSC-IBD model.
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.
Primary sclerosing cholangitis.
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.
Distinct phenotype of primary sclerosing cholangitis-associated inflammatory bowel disease.
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.
Colitis-Associated Carcinoma: The Quintessential Epithelial Neoplasia Driven by Chronic Inflammation.
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.
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
[Pathological anatomy of the liver in patients who died from COVID-19 in the acute period and in the late stages after the new coronavirus infection].
Differentiating IgG4-related Sclerosing Cholangitis from Primary Sclerosing Cholangitis: A Comprehensive Systematic Review and Meta-analysis of Diagnostic Features.
Compositional and functional differences of gut microbiome and metabolome inform pathogenesis of cholestatic liver disease.
Cholangiocyte Biology in Primary Sclerosing Cholangitis and Other Cholangiopathies: Pathogenesis, Clinical Insights, and Experimental Tools.
Post-inflammatory polyps and risk of dysplasia in inflammatory bowel disease: Wolves in sheep's clothing?
📚 EuropePMC4.342 artigos no totalmostrando 195
Cholestatic liver disease in an adolescent: don't forget progressive familial intrahepatic Cholestasis-8.
Oxford medical case reportsResearch progress on the role of gut microbiota dysbiosis in the pathogenesis of immune-mediated liver diseases.
Frontiers in immunologyDysbiotic microbiota trigger colitis-associated colorectal cancer and imprint a distinctive bile acid profile in a PSC-IBD model.
GutPrimary sclerosing cholangitis.
Lancet (London, England)Microscopic colitis and primary sclerosing cholangitis are bidirectionally associated: A nationwide matched cohort and case-control study.
Journal of internal medicineDistinct phenotype of primary sclerosing cholangitis-associated inflammatory bowel disease.
Journal of gastroenterologyBeyond alkaline phopshatase: can anti-integrin αvβ6 autoantibodies serve as biomarkers in primary sclerosing cholangitis trials?
Journal of hepatologyConjugated bile acids facilitate cholangiocyte senescence to promote cholestatic liver diseases via STING signaling.
Journal of advanced researchColitis-Associated Carcinoma: The Quintessential Epithelial Neoplasia Driven by Chronic Inflammation.
CellsMast Cells at the Crossroad of Gut-Derived Signals Through Aryl Hydrocarbon Receptor Activation: A Microbial-Immune Dialogue in Liver Inflammation with Therapeutic Perspectives.
CellsPrevalence of Comorbidities in Inflammatory Bowel Disease: An Umbrella Review of 18 Systematic Reviews.
Journal of clinical medicineEvaluation and management of primary sclerosing cholangitis patients awaiting liver transplantation.
JHEP reports : innovation in hepatologyPrevalence, interreader agreement, and prognostic value of high-grade and relevant strictures in individuals with primary sclerosing cholangitis.
European radiologyEndometriosis presenting as a rectal stricture in a patient with ulcerative colitis and primary sclerosing cholangitis: a case report.
Frontiers in gastroenterology (Lausanne, Switzerland)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 LiverGraft 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 LiverConstructing a protein-protein interaction network for autoimmune liver diseases by integrating pQTL, rQTL, and mediation analyses.
Naunyn-Schmiedeberg's archives of pharmacologyFatigue and circadian rhythm in non-cirrhotic primary biliary cholangitis: An exploratory comparison with primary sclerosing cholangitis and healthy controls.
World journal of hepatologyThe role and possible mechanism of intestinal fungi in the progression of chronic liver diseases.
NPJ biofilms and microbiomesDuct-to-Duct Versus Roux-en-Y in Liver Transplantation for Primary Sclerosing Cholangitis: When Evidence Leaves Room for Pragmatism.
Transplantation[A new paradigm of the pathogenesis of primary sclerosing cholangitis associated with ulcerative colitis: microbiota and system intestine-liver. A review].
Terapevticheskii arkhivRecurrent ileocolic variceal hemorrhage treated with superselective Onyx embolization: A case report.
Radiology case reportsCan liver biopsy be spared for the diagnosis of autoimmune hepatitis in selected children? A multicenter retrospective study.
Annals of hepatologyPrimary sclerosing cholangitis pathogenesis revisited: EBV-idence of immune dysregulation.
JHEP reports : innovation in hepatologyClinico-pathological and immunohistochemical profile of plasma cell rich variants of chronic cholecystitis.
Annals of diagnostic pathologyCardiovascular risk profiles in patients with primary sclerosing cholangitis.
European journal of gastroenterology & hepatologyPeriductal iron-corrected T1 is a predictor of adverse outcomes in large-duct primary sclerosing cholangitis.
BMC medical imagingPrognostic value of magnetic resonance imaging in pediatric-onset primary sclerosing cholangitis.
Therapeutic advances in gastroenterologyBile Acid Sequestrants in Primary Sclerosing Cholangitis and the Risk of Acute Cholangitis: Cause for Concern?
Clinical and translational gastroenterologyAcquired bisalbuminemia associated with hepatobiliary disease: a two-case report and diagnostic implications.
Laboratory medicineProctocolectomy with permanent ileostomy is associated with improved transplant-free survival in patients with PSC.
JHEP reports : innovation in hepatologyProteome-scale autoantibody profiling in PSC: Associations with clinical phenotypes and evidence for neuroendocrine deregulations.
JHEP reports : innovation in hepatologyIgG4-related sclerosing cholangitis: navigating diagnostic dilemmas and the challenge of relapse.
Frontiers in medicineDifferences in immunosuppressive strategies for primary sclerosing cholangitis after liver transplantation.
Journal of hepatologyStatistics and epidemiology of inflammatory bowel disease-associated colorectal neoplasia.
International journal of clinical oncologyOral vancomycin for primary sclerosing cholangitis and associated inflammatory bowel disease - paving a path forward.
Expert review of gastroenterology & hepatologyThe 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 : JMRIIncidence 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 AssociationBronchiectasis in a child with a homozygous DCDC2 gene mutation: A case report.
Journal of Taibah University Medical SciencesDOCK8 deficiency presenting with sclerosing cholangitis, raised immunoglobulin E, and bronchiectasis: A fatal pediatric case report.
JPGN reportsPlasma Biomarkers of Senescence in Cholestatic Liver Disease: A Signature of Risk Stratification and Progression.
Journal of gastroenterology and hepatologyA Mendelian randomization study exploring the genetic associations between biliary system disorders and brain structural changes.
MedicineMesenchymal stromal cell administration promotes macrophage-mediated bile duct regeneration.
Regenerative therapyA Rare Finding at the Hepaticojejunostomy in a Postliver Transplant Patient.
ACG case reports journalPrimary Sclerosing Cholangitis: Diagnosis, Management, and Clinical Challenges.
Journal of clinical medicineColitis-associated colorectal neoplasia in ulcerative colitis with primary sclerosing cholangitis: a nationwide study.
Intestinal researchLong-term MAPK inhibition of childhood refractory-Langerhans cell histiocytosis: an observational study on 288 patients.
Blood advancesA multinational survey assessing the impact and burden of pruritus on patients with primary sclerosing cholangitis.
Hepatology communicationsAdvanced therapies for inflammatory bowel disease associated with primary sclerosing cholangitis: a systematic review and meta-analysis.
Inflammatory bowel diseasesPrimary sclerosing cholangitis in children: a single-center experience and evaluation of prognostic markers.
Translational gastroenterology and hepatologyLiver Transplant in Primary Sclerosing Cholangitis: Results in Our Center.
Transplantation proceedingsAssociation between type 2 diabetes and autoimmune liver disease: An integrated analysis of Mendelian randomization and clinical samples.
MedicineSuperior Diagnostic Performance of 99mTc-FAPI-46 SPECT/CT Versus 18FDG PET/CT in Detecting Peritoneal Involvement in Mucinous Adenocarcinoma of the Colon.
Clinical nuclear medicineClinical Outcomes in Patients with Primary Sclerosing Cholangitis With and Without Inflammatory Bowel Disease.
Digestive diseases and sciencesDecoding the Role of H19 in Cholestatic Liver Injury Using snRNA-seq, Spatial Transcriptomics, and Machine Learning-Based Disease Prediction.
Research squareCurrent and Emerging Approaches for Management of Primary Sclerosing Cholangitis.
Gastroenterology & hepatologyIleal 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 LiverAssociation between osteoporosis and waitlist mortality in liver transplant candidates.
Scientific reportsClinical spectrum and genotype-phenotype correlation of ABCB4 mutations in children: Insights from a North Indian cohort.
World journal of hepatologyUnderstanding hepatopancreatobiliary cancer risks in a population-based primary sclerosing cholangitis-inflammatory bowel disease cohort.
Hepatology (Baltimore, Md.)Autoimmune hepatitis overlap syndrome: Clinical presentation, treatment response, and disease evolution: Tertiary centre experience.
Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology AssociationHCMV infection disrupts barrier functions and promotes epithelial-mesenchymal transition in a cholangiocyte organoid model.
Nature communicationsUncovering the genetic landscape of cholangiocarcinoma and its subtypes via GWAS and integrative analyses.
Hepatology (Baltimore, Md.)Complementary use of autoantibody detection methods facilitates diagnosis of juvenile autoimmune hepatitis and autoimmune sclerosing cholangitis.
JHEP reports : innovation in hepatologyQuality 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 LiverLong-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 diseasesPrevalence and Risk Factors of Colorectal Cancer in Solid Organ Transplant Recipients: A Systematic Review and Meta Analysis.
Clinical transplantationPrimary (AL) Amyloidosis Following COVID-19 Infection: A Case Report.
Clinical case reportsMultimodal treatment and tumour biology-driven long-term survival in PSC-associated hilar cholangiocarcinoma: A case report.
Medicine internationalVedolizumab induced acute interstitial nephritis.
Oxford medical case reportsMUC1 as a biomarker in primary sclerosing cholangitis and cholangiocarcinoma.
Annals of gastroenterologySerum anti-integrin αvβ6 autoantibodies for diagnosis of primary sclerosing cholangitis: a systematic review and meta-analysis.
Annals of gastroenterologyManagement of Inflammatory Bowel Disease Associated With Primary Sclerosing Cholangitis Pre- and Post-liver Transplantation.
TransplantationBile acids as therapeutic agents.
Frontiers in pharmacologySump syndrome following hepaticojejunostomy: a case report of IgG4-related sclerosing cholangitis.
Frontiers in medicineElevated Epithelial Splicing Regulatory Protein 1 Expression in Biliary Atresia Indicates Its Potential as a Molecular Marker.
BiomoleculesIdentifying 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[Immunopathogenesis and therapy of primary sclerosing cholangitis].
Zeitschrift fur GastroenterologieHERACLIS-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 LiverPatient-reported Symptoms Are Independent of Extent of Disease in Longstanding Ulcerative Colitis: Magic in Imagine.
Journal of clinical gastroenterologyDrug-Induced Sclerosing Cholangitis Leading to Discontinuation of Treatment in a Patient with Metastatic Breast Cancer: A Case Report.
Case reports in oncologyUpdated Systematic Review and Meta-Analysis of Duct-to-duct Versus Hepaticojejunostomy Reconstruction After Liver Transplantation for Primary Sclerosing Cholangitis.
TransplantationCholestatic biomarkers and symptoms in primary sclerosing cholangitis: a post-hoc analysis of the DILSTENT.
Scandinavian journal of gastroenterologyDietary Lauric Acid Suppresses Inflammation, Cholestasis, Hepatocyte Injury, and Senescence in 3,5-Diethoxycarbonyl-1,4-Dihydrocollidine-induced Inflammatory Cholangiopathy.
Cellular and molecular gastroenterology and hepatologyEosinophilic cholangitis without peripheral eosinophilia presenting as a localized bile duct stricture mimicking perihilar cholangiocarcinoma.
Clinical journal of gastroenterologyImmunoglobulin G4-related disease with multi-organ involvement: A case report.
MedicineDecoding follicular cholangitis: A benign entity with a malignant façade.
Indian journal of pathology & microbiologyRisk of extraintestinal cancer in patients with inflammatory bowel disease: a multicenter retrospective cohort study in Japan.
Journal of gastroenterologyFecal calprotectin in patients with concomitant primary sclerosing cholangitis and inflammatory bowel disease: a systematic review and meta-analysis.
Clinics and research in hepatology and gastroenterologyFIRST BRAZILIAN CONSENSUS ON CHOLANGIOPANCREATOSCOPY BY THE BRAZILIAN SOCIETY OF DIGESTIVE ENDOSCOPY (SOBED).
Arquivos de gastroenterologiaSteroid-Resistant Relapse of Autoimmune Pancreatitis with Marked Main Pancreatic Duct Dilatation Mimicking Multifocal Pancreatic Cancer.
Internal medicine (Tokyo, Japan)[Acute cholecystitis associated with IgG4-related sclerosing cholangitis: a case report].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyA multilevel analysis of the genetic association between inflammatory bowel disease and primary sclerosing cholangitis.
European journal of gastroenterology & hepatologyThe Scope of Percutaneous Biliary Cholangioscopy: Beyond Choledocholithiasis.
Journal of digestive diseasesProspective, 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 openMAIT Cells in Liver Disease.
Cells[Rational diagnostics and differential diagnosis in IgG4-related disease].
Zeitschrift fur RheumatologieDNA Methylation-mediated BTN3A2 Regulation via CD14+CD16+ Monocytes Protects Against Primary Sclerosing Cholangitis.
Current topics in medicinal chemistryMulticenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan.
Journal of hepato-biliary-pancreatic sciences[On familiar terms with the bile and pancreatic ducts : Where is the benefit?].
Innere Medizin (Heidelberg, Germany)Risk Factors for Primary Sclerosing Cholangitis Recurrence Following Liver Transplantation: A Multicenter Retrospective Analysis.
Clinical transplantationValidation of Quantitative Magnetic Resonance Cholangiopancreatography Metrics in Prediction of Transplant-free Survival in Primary Sclerosing Cholangitis.
Journal of clinical and experimental hepatologyThe Prognostic Utility of Quantitative Magnetic Resonance Cholangiopancreatography in Patients With PSC: A Systematic Review With Structured Evidence Synthesis.
Journal of computer assisted tomographyDrug-induced liver injury in inflammatory bowel disease: Challenges in diagnosis and monitoring.
World journal of hepatologyProtocol for fabricating a vascularized bile duct-on-a-chip.
Biophysics reportsClinical, 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 SocietyQuality and Quantity? The Clinical Significance of Myosteatosis in Various Liver Diseases: A Narrative Review.
Journal of clinical and translational hepatologyGraves' disease with autoimmune hepatitis and primary Sclerosing cholangitis: an overlap syndrome.
Oxford medical case reportsSingle-Cell Genetic Mapping of Gasdermin Expression Across Immune-Mediated Inflammatory Diseases.
FASEB journal : official publication of the Federation of American Societies for Experimental BiologyHepatocyte Mettl3 Deficiency Drives Primary Sclerosing Cholangitis and Liver Fibrosis via Cholangiocyte-Macrophage Crosstalk.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)Novel MicroRNA biomarkers revealed in the serum and bile of thioacetamide induced cholangiocarcinoma.
Chemico-biological interactionsExploring the biliary microbiome in hepatopancreatobiliary disorders: a comprehensive systematic review of microbial signatures and diagnostic potential.
BMC gastroenterologyEfficacy, safety, and relapse outcomes of MAPK inhibitors in pediatric Langerhans cell histiocytosis: A real-world study.
International journal of cancerRIPK1 inhibition reduces biliary injury and fibrosis in primary sclerosing cholangitis.
Science advancesMechanistic roles and therapeutic potential of bacteriophages in inflammatory gastrointestinal diseases.
Microbiome research reportsA rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma.
Journal of liver cancerUbiquitin D Correlates with Disease Severity and T Cell Infiltration in Cholestasis: Evidence from Integrated Bioinformatics and Experimental Analyses.
International journal of medical sciencesEndoscopic retrograde cholangiopancreatography and primary sclerosing cholangitis: a retrospective study of a high-volume program.
Journal of the Canadian Association of GastroenterologyViral infection as a trigger for primary sclerosing cholangitis in genetically susceptible individuals.
Journal of hepatologyBurden of primary sclerosing cholangitis in Sweden (2002-2020): Incidence, outcomes, healthcare utilization, and costs.
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 gastroenterologyBiliary Reconstruction in Liver Transplantation with Primary Sclerosing Cholangitis: Roux-en-Y Hepaticojejunostomy or Duct-to-Duct Anastomosis?
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)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 sciencesRadial laser ablation under cholangioscopic guidance for a tight intrahepatic bile duct stricture caused by primary sclerosing cholangitis.
EndoscopyPembrolizumab-Induced Secondary Cholangitis: A Case Report.
CureusBeyond futility: The history and potential of liver transplantation in cholangiocarcinoma.
World journal of transplantationRisk 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 OAmiR-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 pathologyPruritus 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.)Risk of intestinal complications, extraintestinal morbidity, and mortality in patients with ulcerative colitis and ocular involvement.
Eye (London, England)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 AssociationRisk of Intestinal and Extraintestinal Malignancies in Inflammatory Bowel Disease With and Without Primary Sclerosing Cholangitis.
Digestive diseases and sciencesSecondary Sclerosing Cholangitis in a Critically Ill Burn Patient: A Case Report and Review of the Literature.
Clinical case reportsAutoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.
Hepatology internationalA retrospective evaluation of preemptive liver transplantation for bile duct dysplasia in primary sclerosing cholangitis: Balancing risks and benefits.
JHEP reports : innovation in hepatologyIntestinal barrier in chronic gut and liver diseases: Pathogenesis and therapeutic targets.
Acta pharmaceutica Sinica. BImpact of Metabolic Dysfunction-Associated Steatotic Liver Disease on Fatigue and Pruritus in Primary Sclerosing Cholangitis: A U.S. Single-Center Study.
Journal of clinical medicineA Pharmacovigilance Study of Immune Checkpoint Inhibitor-Associated Cholangitis: Insights From the FDA Adverse Event Reporting System.
Hepatology research : the official journal of the Japan Society of HepatologyBlood and tissue dysregulated bile acids and short-chain fatty acids in cholangiocarcinoma.
JHEP reports : innovation in hepatologyDifferences in Anti-αvβ6 Integrin Antibody Expression between U.S. and Japanese Cohorts in Inflammatory Bowel Disease.
Inflammatory bowel diseasesSystemic PCSK9 elevation characterises autoimmune liver disease across sexes.
Scientific reportsAn overview of paediatric autoimmune and genetic cholestatic liver disease for the adult physician.
Clinical medicine (London, England)Clinicopathological features of hepatic Langerhans cell histiocytosis: report of ten cases and review of the literature.
Annals of diagnostic pathologyModerate increase of MET in hepatocytes protects against cholestatic liver injury by promoting an effective antioxidant response.
The FEBS journalAutoimmune hepatitis in children: Guidelines of the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ISPGHAN).
Hepatology internationalSafety and Efficacy of Upadacitinib in Patients with Inflammatory Bowel Disease After Liver Transplantation: A Case Series.
Digestive diseases and sciencesCausal associations between primary sclerosing cholangitis and systemic lupus erythematosus: Evidence from Mendelian randomization and transcriptomic analyses.
MedicineHepatobiliary and pancreatic findings on magnetic resonance enterography examinations in children with newly diagnosed inflammatory bowel disease.
Pediatric radiologyNebokitug, an Anti-chemokine (C-C Motif) Ligand 24 Monoclonal Antibody, in Patients With Primary Sclerosing Cholangitis: A Phase 2 Study.
The American journal of gastroenterologySelective Inhibitors of Toll-Like Receptor 9 (TLR9) Could Potentially Treat Fibrotic, Autoimmune, and Inflammatory Diseases.
ACS medicinal chemistry lettersLipoprotein X-Induced Hyperlipidemia Following Liver Transplantation in the Setting of Persistent Cholestasis and Antibody-Mediated Rejection.
ACG case reports journalClinicogenomic Characterization of Primary Sclerosing Cholangitis-Associated Biliary Tract Cancers.
Clinical cancer research : an official journal of the American Association for Cancer ResearchCausal mediation of immune cells in autoimmune liver diseases and oral cancer risk: a mendelian randomization study.
International journal of surgery (London, England)Sclerosing Cholangitis and Multiple Liver Abscesses in a Patient with Thymoma, Myasthenia Gravis, and Immune Deficiency (Good's Syndrome).
The American journal of case reportsRegulatory T cell stability determines the efficiency of bile duct regeneration during cholangitis.
Cell reportsNumb-exon3 and full length Numb equivalently alleviate cholestatic liver fibrosis by inhibiting ductular reaction.
Scientific reportsRisk stratification of IBD-associated liver disease using routinely collected biomarkers from a large-scale real-world dataset.
BMJ open gastroenterologySpanish 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 hepatologiaTepotinib-Induced Cholangitis in a Patient With Non-Small Cell Lung Cancer: A Case Report.
Respirology case reportsGut-Liver Axis, Microbiota, Bile Acids, and Immune Response in Pathogenesis of Primary Sclerosing Cholangitis: An Overview.
Journal of clinical medicineAssociation between non-invasive biomarkers and quality of life in Primary Sclerosing Cholangitis.
PloS onePrimary sclerosing cholangitis: a narrative review of diagnostic and prognostic biomarkers.
Translational gastroenterology and hepatologyChronic Recurrent Multifocal Osteomyelitis Associated in a Child with Inflammatory Bowel Disease and Primary Sclerosing Cholangitis: A Case Report.
JMA journalCausal effects of immune cell phenotypes on the risk of autoimmune liver diseases: a bidirectional two-sample Mendelian randomization study.
Translational gastroenterology and hepatologyPhase II clinical trials in primary sclerosing cholangitis: Making the most of every opportunity?
Journal of hepatologyGenetic architecture of primary sclerosing cholangitis: shared pathways with inflammatory bowel disease and gut-liver axis mediation.
International journal of surgery (London, England)Microbiome and gut-liver interactions: From mechanisms to therapies.
World journal of gastroenterologyDietary and Lifestyle Risk Factors in Primary Sclerosing Cholangitis: In Search of Mechanistic Explanations and Health Improvement.
The Journal of nutritionCilofexor in non-cirrhotic primary sclerosing cholangitis (PRIMIS): a randomised, double-blind, multicentre, placebo-controlled, phase 3 trial.
The lancet. Gastroenterology & hepatologyBile Duct Targeting or Preservation: Contrasting Liver Histology in Langerhans Cell Histiocytosis and Disseminated Juvenile Xanthogranuloma.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyPatient radiation safety in imaging in Crohn's disease.
The British journal of radiologyLiver transplantations in Finland over 40 years: evolution and recent trends.
eGastroenterologyQuantitative 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)Macrophages in Autoimmune Liver Diseases: From Immune Homeostasis to Precision-Targeted Therapy.
BiomedicinesInfections in primary sclerosing cholangitis and inflammatory bowel disease: a systematic review and meta-analysis.
Journal of the Canadian Association of GastroenterologyApremilast as a novel therapeutic option for psoriasis coexisting primary sclerosing cholangitis and ulcerative colitis: A case report.
MedicinePrimary Sclerosing Cholangitis in a Child: A Rare Case Report.
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chiAssociation 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 LiverIsolated IgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma: A case report and review.
Hepatology forumNavigating 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 LiverCultured bacteria isolated from primary sclerosing cholangitis patient bile induce inflammation and cell death.
mSphereUlcerative colitis and colorectal cancer: Pathogenic insights and precision strategies for prevention and treatment.
World journal of gastrointestinal oncologyInnate immune cell LXR-β deficiency exacerbates hepatic injury and fibrosis in murine models of primary sclerosing cholangitis.
Liver research (Beijing, China)Immunotherapy-Induced Hepatitis Mimicking Sclerosing Cholangitis in a Patient With Metastatic Triple-Negative Breast Cancer.
CureusFuture Treatment Options for Managing Primary Sclerosing Cholangitis and Cholestatic Pruritus.
Clinics in liver diseaseClinical Profile and Predictors of Repeated Relapse in Type 1 Autoimmune Pancreatitis: A Multicenter Observational Study.
Journal of gastroenterology and hepatologyDistal Bile Duct Metastasis From Rectal Cancer: The Diagnostic Contribution of Intraductal Ultrasonography.
DEN openCholangiocarcinoma - Morphology, Immunohistochemistry, and Genetics.
Ceskoslovenska patologieSpatial heterogeneity in liver stiffness does not predict clinical outcomes in patients with primary sclerosing cholangitis.
Hepatology communicationsEpidemiology, comorbidities, treatments and outcomes of autoimmune liver diseases: A French nationwide study.
JHEP reports : innovation in hepatologyPoint-of-Care Testing and Biomarkers in Biliary Diseases: Current Evidence and Future Directions.
Journal of clinical medicineLocalization 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 pharmacologyAssociações
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Colangite esclerosante e no aconselhamento genético da família.
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
A doença é causada por uma inflamação crônica de natureza autoimune, na qual o sistema de defesa afeta os ductos biliares do fígado. Fatores genéticos também estão sob investigação, incluindo variantes em genes como DCDC2 e GPR35.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/colangite-esclerosante
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Colangite esclerosante
📋 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
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Ensaios clínicos
- fonte: ClinicalTrials.gov
