Colangite esclerosante secundária é uma doença hepática rara caracterizada por inflamação e cicatrização progressiva dos ductos biliares, geralmente secundária a outras condições como colangite bacteriana recorrente ou cirurgia biliar. Leva à obstrução do fluxo biliar, podendo resultar em cirrose e insuficiência hepática.
Introdução
O que você precisa saber de cara
Visão geral
A colangite esclerosante secundária (conhecida internacionalmente como Secondary sclerosing cholangitis) é uma condição clínica rara codificada como MONDO:0018647 e classificada sob o código CID-10 K83.0.[1]
Diagnóstico
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
A colangite esclerosante secundária é uma doença rara que acomete os ductos biliares após agressões conhecidas ao organismo. Ela pode se desenvolver em contextos de internações graves em terapia intensiva ou após outras condições clínicas e medicamentosas prévias. A enfermidade não consta na lista oficial de PCDT de doenças raras do Ministério da Saúde nem tem remédios catalogados para entrega no SUS. O cuidado do paciente é direcionado pelas equipes médicas nos serviços especializados de saúde.
Colangite esclerosante secundária é uma doença hepática rara caracterizada por inflamação e cicatrização progressiva dos ductos biliares, geralmente secundária a outras condições como colangite bacteriana recorrente ou cirurgia biliar. Leva à obstrução do fluxo biliar, podendo resultar em cirrose e insuficiência hepática.
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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 esclerosante secundária
Selecione um estado ou use sua localização para ver resultados.
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Com ensaio aberto em 2 países. O ponto verde marca onde há vaga agora.
Pesquisa e ensaios clínicos
4 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
An Unusual Cause of Secondary Sclerosing Cholangitis: A Case Report.
Correction to "Secondary Sclerosing Cholangitis in a Critically Ill Burn Patient: A Case Report and Review of the Literature".
[This corrects the article DOI: 10.1002/ccr3.71549.].
Bile Duct Targeting or Preservation: Contrasting Liver Histology in Langerhans Cell Histiocytosis and Disseminated Juvenile Xanthogranuloma.
Liver involvement by histiocytic and dendritic cell neoplasms signals high-risk disease, often necessitating closer monitoring and aggressive management. Severe cases may progress to liver failure, requiring transplantation. Liver involvement occurs in about one-third of patients with systemic juvenile xanthogranuloma (JXG) and 20% to 60% of pediatric patients with Langerhans cell histiocytosis (LCH), particularly in multiorgan disease. Tyrosine kinase inhibitors show promise in LCH treatment, but optimal timing for treatment cessation remains uncertain. We present 2 pediatric cases, 1 with LCH, and the other with disseminated JXG, along with a literature review emphasizing liver histopathology and transplant considerations. These cases highlight distinct histological patterns. In LCH, progressive bile duct destruction led to ductopenic cholestatic cirrhosis and secondary sclerosing cholangitis. In contrast, in the case of JXG, bile ducts remained intact despite being surrounded by histiocytes. In both, disease localization to larger, segmental portal tracts may reduce liver biopsy sensitivity. In LCH, BRAF inhibitor therapy triggered a granulomatous reaction that could mimic disease recurrence in the liver graft. Other histiocytoses typically spare the bile ducts and do not cause biliary cirrhosis. Recognizing these distinct infiltration patterns can aid diagnosis and management.
Drug-induced liver injury. Part II: Late complications and hepatotoxicity monitoring.
The picture of drug-induced liver injury (DILI) is polymorphic, with variable intensity of clinical symptoms and prognosis. Most cases of DILI are acute, although the incidence of chronic hepatopathy has been reported to range from 3.4% to 39.0% in the period 6-12 months after discontinuation of the drug. The long-term chronic consequences of DILI in terms of morbidity and mortality are unclear. The rare obstructive bile duct syndrome is associated with an unfavorable prognosis - a higher risk of chronic liver failure and the need for liver transplantation. Other long-term forms of hepatopathy following DILI include progressive liver fibrosis, autoimmune hepatitis, hepatic steatosis, secondary sclerosing cholangitis, vascular lesions, and liver tumors. Recently, immune checkpoint inhibitors, which can cause an autoimmune-like phenotype, have also been shown to cause sclerosing cholangitis with infiltration of cytotoxic T cells in the biliary tract. Autoimmune pancreatitis (AIP), also referred to as nonalcoholic destructive pancreatitis and sclerosing pancreatitis, is a rare condition characterized histologically by chronic inflammation of the pancreas and clinically by various symptoms related to biliary and pancreatic pathologies. Recurrent acute pancreatitis or painless jaundice should prompt an evaluation for autoimmune pancreatitis. AIP can be a primary pancreatic disorder or a systemic autoimmune disease associated with other autoimmune conditions, such as immunoglobulin G subclass 4 (IgG4)–related diseases. Autoimmune pancreatic disease is the only pancreatic condition treated effectively with corticosteroids. Three types of AIP are clinically recognized: Type 1: IgG4-related pancreatitis, associated with a serum IgG4 concentration greater than twice the normal reference range in most affected individuals. Diagnostic findings include infiltration with greater than 10 IgG4-positive plasma cells per high-power field (HPF), a cartwheel pattern of fibrosis, lymphocytic and plasma cell inflammation of venules, and a histologic description of lymphoplasmacytic sclerosing pancreatitis. The most common clinical presentation of type 1 AIP is painless jaundice with laboratory studies suggesting biliary obstruction and hyperglycemia. Other cases present with abdominal pain or are discovered in asymptomatic individuals with a pancreatic mass, pancreatic duct stricturing, or an enlarged pancreas. Acute pancreatitis is an unusual presentation in type 1 AIP, but it can result in exocrine and endocrine pancreatic insufficiency. The pancreas may be the only organ affected, or there may also be other organs involved with corresponding clinical presentations, including: Bile ducts (secondary sclerosing cholangitis). Retroperitoneal fibrosis. Kidneys (tubulointerstitial nephritis). Lungs. Submandibular and parotid glands (Sjögren disease). Sublingual glands (sclerosing sialadenitis). Orbits and lacrimal glands (IgG4-related dacryoadenitis and sialoadenitis, formerly Mikulicz disease) . Joints (rheumatoid arthritis). Type 2: Histologically, this is idiopathic duct-centric pancreatitis with granulocytic epithelial lesions within the pancreatic duct, small numbers of IgG4–positive plasma cells (fewer than 10/HPF), and no extrapancreatic involvement. Type 2 AIP is often associated with inflammatory bowel disease and is twice as common in patients with ulcerative colitis or proctitis as in those with Crohn disease. Unlike patients with type 1 AIP, about half of those with type 2 AIP present with abdominal pain or acute pancreatitis. Type 3: Immune checkpoint inhibitor-induced pancreatic injury is a type of progressive immune-mediated pancreatitis, an adverse effect of cancer treatment with immune checkpoint inhibitor drugs. The risk of this immune response is heightened when multiple immune checkpoint inhibitors are used. Most patients with type 3 AIP are asymptomatic.
Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series.
To describe the occurrence of secondary sclerosing cholangitis in critically ill patients (SC-CIP) with febrile infection-related epilepsy syndrome (FIRES). Monocentric retrospective analysis of all adult patients with FIRES admitted from January 2020 to December 2024. Four patients (3 males) with a mean age of 24 years (range: 18-40 years) and no significant medical history presented with cryptogenic FIRES. They required treatment with antiseizure medications (mean: 9; range: 8-10), anesthetics (propofol, midazolam and ketamine in all cases), and immunotherapies. The average duration of status epilepticus (SE) was 57 days (range: 34-90 days), while the mean duration of intensive care unit (ICU) stay was 82 days (range: 58-117 days). All patients developed cholestatic liver disease during their ICU stay, reversible in one case. In the three cases with persistent injury (75%), SC-CIP was diagnosed with MR-colangiography after a mean of 106 days from SE onset. The high incidence of SC-CIP in our cohort of patients with FIRES suggests a link between these two rare conditions, likely related to prolonged intensive care, hyperinflammation and polytherapy, including ketamine use. Vigilant monitoring of liver disease progression in critically ill patients with FIRES and similar predisposing factors may allow early recognition of SC-CIP and improved patient outcomes.
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].
Secondary changes in the peripheral liver due to kaposiform haemangioendothelioma in the hepatic hilum: a report of two transplant cases.
Demystifying the imaging diagnoses of sclerosing cholangitis.
Secondary Sclerosing Cholangitis in a Critically Ill Burn Patient: A Case Report and Review of the Literature.
📚 EuropePMC133 artigos no totalmostrando 170
Secondary Sclerosing Cholangitis in a Critically Ill Burn Patient: A Case Report and Review of the Literature.
Clinical case reportsBile 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 SocietyDrug-induced liver injury. Part II: Late complications and hepatotoxicity monitoring.
Clinical and experimental hepatologyAn Unusual Cause of Secondary Sclerosing Cholangitis: A Case Report.
GastroenterologySecondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series.
Frontiers in neurologySecondary sclerosing cholangitis in patients suffering cardiogenic shock.
ESC heart failureEtiology and Clinical Features of Secondary Sclerosing Cholangitis: A Single-Center Retrospective Study From 2016 to 2024.
JGH open : an open access journal of gastroenterology and hepatologySecondary sclerosing cholangitis: an unusual presentation of leptospirosis.
Tropical medicine and healthPerihilar and Intrahepatic Cholangiocarcinoma after Resection: Clinicopathological Characteristics, Outcomes, and Implications for Addition of Chemoradiotherapy.
Euroasian journal of hepato-gastroenterologyPrognostic factors for transplant-free survival in patients with secondary sclerosing cholangitis associated with critical illness.
BMJ open gastroenterologySecondary sclerosing cholangitis due to drug-induced liver injury: a retrospective cohort study.
European journal of gastroenterology & hepatologySecondary Sclerosing Cholangitis due to Drugs With a Special Emphasis on Checkpoint Inhibitors.
Liver international : official journal of the International Association for the Study of the LiverOutcome of a 'step-up approach' for recurrent cholangitis in patients with a non-stenotic hepaticojejunostomy after hepato-pancreato-biliary surgery: single center series.
HPB : the official journal of the International Hepato Pancreato Biliary AssociationSecondary Sclerosing Cholangitis (SSC): An Underrecognized Complication of Severe COVID-19 Pneumonitis.
CureusIntegrin αVβ6: Autoantigen and Driver of Epithelial Remodeling in Colon and Bile Ducts in Primary Sclerosing Cholangitis and Inflammatory Bowel Disease.
Journal of Crohn's & colitisCOVID-19-Related Cholangiopathy: Histological Findings.
Diagnostics (Basel, Switzerland)Drug-induced cholestatic liver diseases.
Hepatology (Baltimore, Md.)Imaging of Biliary Tree Abnormalities.
Radiographics : a review publication of the Radiological Society of North America, IncHistopathological impact of SARS-CoV-2 on the liver: Cellular damage and long-term complications.
World journal of gastroenterologyEndoscopic Characterization and Outcome of COVID-19 Patients with Secondary Sclerosing Cholangitis: A Case Series of a Tertiary Center.
Journal of gastrointestinal and liver diseases : JGLDHepatitis and Vasodilatory Shock due to an Unsuspected Culprit: A Rare Presentation of Multisystem Langerhans Cell Histiocytosis.
Case reports in gastroenterologyPart 2: Current Concepts in Radiologic Imaging & Intervention in Acute Biliary Tract Diseases.
Journal of intensive care medicineBiliary Cast Syndrome and Secondary Sclerosing Cholangitis in Critically Ill Patient after Long-Term Treatment in the Intensive Care Unit.
Case reports in gastroenterologyvon Meyenburg complexes are more frequently associated with cholangiocarcinoma.
Journal of clinical pathologyIncidence of Secondary Sclerosing Cholangitis in Hospitalized Long COVID-19 Patients: A Retrospective Single Center Study.
Diagnostics (Basel, Switzerland)COVID-19-associated secondary sclerosing cholangitis with liver transplantation.
Virchows Archiv : an international journal of pathologyA Review of IgG4-related Sclerosing Cholangitis (IgG4-SC).
Journal of clinical gastroenterologyPrimary Sclerosing Cholangitis: Diagnostic Criteria.
Tomography (Ann Arbor, Mich.)Biliary cast syndrome developed after treatment for acute cholangitis: progression to secondary sclerosing cholangitis.
Gastrointestinal endoscopyPhenotypes of Primary Sclerosing Cholangitis and Differential Diagnosis.
Clinics in liver diseaseRole of intravenous ketamine in the pathogenesis of secondary sclerosing cholangitis in critically ill patients: perpetrator or innocent bystander? Answers provided by forensic toxicology.
Intensive care medicineEndoscopic Features of Post-COVID-19 Cholangiopathy and Its Management Using ERCP.
The American journal of gastroenterologyPost-COVID-19 cholangiopathy: Systematic review.
World journal of methodologyMetallothionein: a game changer in histopathological diagnosis of Wilson disease.
HistopathologySecondary Sclerosing Cholangitis After SARS-CoV2: ICU Ketamine Use or Virus-Specific Biliary Tropism and Injury in the Context of Biliary Ischemia in Critically Ill Patients?
Hepatic medicine : evidence and researchSystemic Disease and the Liver-Part 1: Systemic Lupus Erythematosus, Celiac Disease, Rheumatoid Arthritis, and COVID-19.
Surgical pathology clinicsUnraveling the Molecular and Cellular Pathogenesis of COVID-19-Associated Liver Injury.
VirusesPost-COVID-19 cholangiopathy: Current understanding and management options.
World journal of gastrointestinal surgery[Drug-induced bile duct injury: progress and challenges].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologySecondary sclerosing cholangitis induced by systemic chemotherapy.
Revista espanola de enfermedades digestivasCOVID-19 related biliary injury: A review of recent literature.
World journal of gastroenterologyHepatobiliary long-term consequences of COVID-19: dramatically increased rate of secondary sclerosing cholangitis in critically ill COVID-19 patients.
Hepatology internationalBISCIT: Biliary interventions in critically ill patients with secondary sclerosing cholangitis-a study protocol for a multicenter, randomized, controlled parallel group trial.
TrialsSecondary Sclerosing Cholangitis After Emphysematous Cholecystitis.
ACG case reports journalImplications of anaemia and response to anaemia treatment on outcomes in patients with cirrhosis.
JHEP reports : innovation in hepatologySecondary Sclerosing Cholangitis due to Severe COVID-19: An Emerging Disease Entity?
Digestion[A case of secondary sclerosing cholangitis following endoscopic submucosal dissection ulcer bleeding].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyProfile of liver cholestatic biomarkers following prolonged ketamine administration in patients with COVID-19.
BMC anesthesiologyCOVID-19 and the liver: Are footprints still there?
World journal of gastroenterologyA Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis.
ACG case reports journalRisk of New-Onset Liver Injuries Due to COVID-19 in Preexisting Hepatic Conditions-Review of the Literature.
Medicina (Kaunas, Lithuania)Secondary sclerosing cholangitis in a young COVID-19 patient resulting in death: A case report.
World journal of gastrointestinal surgerySecondary sclerosing cholangitis and IgG4-sclerosing cholangitis - A review of cholangiographic and ultrasound imaging.
Endoscopic ultrasoundOptimal timing of liver transplantation for liver cirrhosis caused by sclerosing cholangitis in a patient with Langerhans cell histiocytosis: a case report.
International journal of hematologyPractical Guide for Radiological Diagnosis of Primary and Secondary Sclerosing Cholangitis.
Seminars in ultrasound, CT, and MRBiliary Tract Injury in Patients With COVID-19: A Review of the Current Literature.
Gastroenterology & hepatologyCOVID-19-associated secondary sclerosing cholangitis - A case series of 4 patients.
Clinics and research in hepatology and gastroenterologyEndosonographic and ERCP findings in COVID-19 critical illness cholangiopathy.
Revista espanola de enfermedades digestivasEarly detection of COVID-19 cholangiopathy using cholangioscopy-a case report of two critically ill patients.
European surgery : ACA : Acta chirurgica AustriacaDelayed immune-related sclerosing cholangitis after discontinuation of pembrolizumab: A case report.
World journal of gastroenterologyAn Extremely Rare Cause of an Obstructive Jaundice in Adults: Limited Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct.
Visceral medicineSecondary sclerosing cholangitis after critical COVID-19: Three case reports.
World journal of hepatologyCurrent perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review.
World journal of gastroenterologyForms of cholangitis to be considered after SARS-CoV-2 infection.
Clinical and molecular hepatologyPost-COVID-19 Secondary Sclerosing Cholangitis: A Rare but Severe Condition with no Treatment Besides Liver Transplantation.
The American journal of case reportsSecondary sclerosing cholangitis after COVID-19 pneumonia: a report of two cases and review of the literature.
Clinical journal of gastroenterologyImaging features of COVID-19-associated secondary sclerosing cholangitis on magnetic resonance cholangiopancreatography: a retrospective analysis.
Insights into imagingEffect of endoscopic treatment in patients with secondary sclerosing cholangitis.
Journal of gastroenterology and hepatologyGamma-glutamyltransferase is a strong predictor of secondary sclerosing cholangitis after lung transplantation for COVID-19 ARDS.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationSecondary Sclerosing Cholangitis Following Coronavirus Disease 2019 (COVID-19): A Multicenter Retrospective Study.
Clinical infectious diseases : an official publication of the Infectious Diseases Society of AmericaEASL Clinical Practice Guidelines on sclerosing cholangitis.
Journal of hepatologyPersistent Cholestatic Injury and Secondary Sclerosing Cholangitis in COVID-19 Patients.
Archives of pathology & laboratory medicineProgressive cholestasis and associated sclerosing cholangitis are frequent complications of COVID-19 in patients with chronic liver disease.
Hepatology (Baltimore, Md.)Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis.
Abdominal radiology (New York)Secondary sclerosing cholangitis: A complication after severe COVID-19 infection.
Gastroenterologia y hepatologiaSecondary Sclerosing Cholangitis in a Critically Ill Patient with Severe SARS-CoV-2 Infection: A Possibly Emergent Entity during the Current Global Pandemic.
GE Portuguese journal of gastroenterologyMeta-analysis and systematic review of liver transplantation as an ultimate treatment option for secondary sclerosing cholangitis.
Przeglad gastroenterologicznySevere acute respiratory syndrome coronavirus-2-associated cholangiopathies.
Current opinion in gastroenterologyTechnical Note: Novel Use of CytoSorb™ Haemadsorption to Provide Wound Healing Support in Case of Severe Burn Trauma via Reduction of Hyperbilirubinaemia.
Frontiers in surgeryIgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease.
Canadian journal of gastroenterology & hepatologyKinetics of Bilirubin and Ammonia Elimination during Hemadsorption Therapy in Secondary Sclerosing Cholangitis Following ECMO Therapy and Severe COVID-19.
BiomedicinesDisseminated cerebral fusariosis in a liver-transplant patient: A case report and review of the literature.
Zeitschrift fur GastroenterologieDiagnosis of Secondary Sclerosing Cholangitis by Port Site Metastasis.
CureusLong-term sequelae of drug-induced liver injury.
Journal of hepatologyCorona again? SSC after a severe COVID-disease.
Zeitschrift fur Gastroenterologie[Severe secondary sclerosing cholangitis as manifestation of a very rare underlying disease].
Der InternistHistopathological examination of the impact of sodium hypochlorite on the hepatobiliary system. An experimental study.
Annali italiani di chirurgiaA case of secondary sclerosing cholangitis due to COVID-19.
Clinical imagingSecondary sclerosing cholangitis: an emerging complication in critically ill COVID-19 patients.
Intensive care medicineSecondary sclerosing cholangitis as a complication of severe COVID-19: A case report and review of the literature.
Clinical case reportsComparison of clinical features between immune-related sclerosing cholangitis and hepatitis.
Investigational new drugsSecondary sclerosing cholangitis as cause of persistent jaundice in patients with severe COVID-19.
Liver international : official journal of the International Association for the Study of the LiverCase report: Secondary sclerosing cholangitis induced by lapatinib and vinorelbine in a metastasis breast cancer patient.
Thoracic cancerHigh Rate of Gastrointestinal Bleeding in Patients with Secondary Sclerosing Cholangitis in Critically Ill Patients (SC-CIP).
Journal of clinical medicineEndogenous Opioid Levels Do Not Correlate With Itch Intensity and Therapeutic Interventions in Hepatic Pruritus.
Frontiers in medicineCOVID-19-Induced Bile Duct Casts and Cholangitis: A Case Report.
CureusPost-Covid-19 Cholangiopathy-A New Indication for Liver Transplantation: A Case Report.
Transplantation proceedingsThe Gut-Liver Axis in Cholestatic Liver Diseases.
NutrientsLiver Transplantation for Langerhans Cell Histiocytosis: A US Population-Based Analysis and Systematic Review of the Literature.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation SocietyPost-COVID-19 Cholangiopathy: A Novel Entity.
The American journal of gastroenterologyAtezolizumab-induced Sclerosing Cholangitis in a patient with lung cancer: A case report.
Cancer treatment and research communicationsA case with life-threatening secondary sclerosing cholangitis caused by nivolumab.
Clinical journal of gastroenterologySecondary sclerosing cholangitis in critically ill patients: a rare disease precipitated by severe SARS-CoV-2 infection.
BMJ case reportsFibrates for Itch (FITCH) in Fibrosing Cholangiopathies: A Double-Blind, Randomized, Placebo-Controlled Trial.
GastroenterologyAn Interprofessional Approach in Caring for a Patient on Maintenance Hemodialysis with COVID-19 in Toronto, Canada: An Educational Case Report.
Canadian journal of kidney health and diseaseSecondary Sclerosing Cholangitis in Critically Ill Patients Alters the Gut-Liver Axis: A Case Control Study.
Nutrients[Hepatic dysfunction in sepsis].
Medizinische Klinik, Intensivmedizin und NotfallmedizinChallenging biliary strictures: pathophysiological features, differential diagnosis, diagnostic algorithms, and new clinically relevant biomarkers - part 1.
Therapeutic advances in gastroenterologySecondary sclerosing cholangitis in critically ill patients has a poor outcome but lower tumour incidence than primary sclerosing cholangitis.
United European gastroenterology journalPathologic Manifestations of Gastrointestinal and Hepatobiliary Injury in Immune Checkpoint Inhibitor Therapy.
Archives of pathology & laboratory medicineLiver Dysfunction in Sepsis.
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chiPembrolizumab-induced secondary sclerosing cholangitis in a non-small cell lung cancer patient.
Respirology case reportsSecondary Sclerosing Cholangitis in Critically Ill Patients: An Underdiagnosed Entity.
GE Portuguese journal of gastroenterologyExtrahepatic biliary duplication with heterotopic gastric mucosa in a 46,XX male patient.
Clinics and research in hepatology and gastroenterologySecondary sclerosing cholangitis from percutaneous transhepatic biliary drainage in a patient with gallbladder cancer after surgery: A case report.
Journal of interventional medicineIntrahepatic biliary strictures after liver transplantation are morphologically similar to primary sclerosing cholangitis but immunologically distinct.
European journal of gastroenterology & hepatologyChemotherapy-Induced Sclerosing Cholangitis Caused by Systemic Chemotherapy.
ACG case reports journal[Hepatic dysfunction in patients with cardiogenic shock].
Medizinische Klinik, Intensivmedizin und NotfallmedizinNivolumab-Induced Secondary Sclerosing Cholangitis with Deterioration Despite Immunosuppression.
Journal of thoracic oncology : official publication of the International Association for the Study of Lung CancerA Case of Secondary Sclerosing Cholangitis in the setting of Non-Hodgkin's Lymphoma.
CureusContributing factors and outcomes of burn-associated cholestasis.
Journal of hepatologySclerosing Cholangitis Related to IgG4: Not Always a Curable Entity.
Annals of hepatologySecondary sclerosing cholangitis in critically ill patients.
Cirugia y cirujanosLow-Phospholipid Associated Cholelithiasis (LPAC) syndrome: A synthetic review.
Journal of visceral surgerySecondary sclerosing cholangitis with hemobilia induced by pembrolizumab: Case report and review of published work.
Hepatology research : the official journal of the Japan Society of HepatologyRadiological patterns of secondary sclerosing cholangitis in patients after lung transplantation.
Abdominal radiology (New York)Secondary sclerosing cholangitis following extracorporeal membrane oxygenation for acute respiratory distress in polytrauma.
Clinical case reportsInfant onset severe complement-mediated hemolytic uremic syndrome complicated by secondary sclerosing cholangitis.
Journal of clinical apheresisAntiprogrammed Cell Death-1 Immunotherapy-Related Secondary Sclerosing Cholangitis.
Hepatology (Baltimore, Md.)[IgG4-associated cholangitis - clinical presentation of an overlooked disease entity].
Der Internist[Secondary sclerosing cholangitis in critically ill patients].
Cirugia y cirujanosSecondary sclerosing cholangitis in critically ill patients after a traffic accident-a new entity that should be considered in death classification.
International journal of legal medicineDrug-induced cholestasis.
Hepatology communicationsPregnant Lady With Undiagnosed Hodgkin's Disease Presenting As Secondary Sclerosing Cholangitis.
Journal of Ayub Medical College, Abbottabad : JAMCSecondary sclerosing cholangitis in localized hepatobiliary tuberculosis simulating cholangiocarcinoma: a rare case report.
BMC gastroenterologyIgG4-Related Cholangiopathy and Its Mimickers: A Case Report and Review Highlighting the Importance of Early Diagnosis.
International journal of surgical pathologyNOD2 gene variants confer risk for secondary sclerosing cholangitis in critically ill patients.
Scientific reportsSecondary Sclerosing Cholangitis: a Review of Recent Literature.
Current gastroenterology reportsSecondary sclerosing cholangitis in critically ill patients: current perspectives.
Clinical and experimental gastroenterologyVery Severe Secondary Sclerosing Cholangitis as a Sequela of Influenza.
Deutsches Arzteblatt internationalSecondary sclerosing cholangitis following cardiac surgery: An uncommon but deadly gastrointestinal complication.
The Journal of thoracic and cardiovascular surgeryResolution of Diffuse Intrahepatic Biliary Strictures after Chemotherapy for Metastatic Ovarian Cancer.
ACG case reports journalSecondary sclerosing cholangitis in cardiac surgical patients: A complication with a dismal prognosis.
The Journal of thoracic and cardiovascular surgeryFibrates for the treatment of cholestatic itch (FITCH): study protocol for a randomized controlled trial.
Trials[A contribution to the differential diagnostics of sclerosing cholangitides].
Vnitrni lekarstviProphylaxis of post-ERC infectious complications in patients with biliary obstruction by adding antimicrobial agents into ERC contrast media- a single center retrospective study.
BMC gastroenterologyLiver - guardian, modifier and target of sepsis.
Nature reviews. Gastroenterology & hepatology[Elevated liver enzymes].
Deutsche medizinische Wochenschrift (1946)Secondary Sclerosing Cholangitis From Spontaneous Choledochoduodenal Fistula.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationSecondary Sclerosing Cholangitis During Pregnancy.
ACG case reports journalMacrophage Depletion Attenuates Extracellular Matrix Deposition and Ductular Reaction in a Mouse Model of Chronic Cholangiopathies.
PloS oneAnti-GP2 IgA autoantibodies are associated with poor survival and cholangiocarcinoma in primary sclerosing cholangitis.
GutExtrarenal Manifestations in Shigatoxin-associated Haemolytic Uremic Syndrome.
Klinische PadiatrieSecondary sclerosing cholangitis in a critically ill patient.
Quantitative imaging in medicine and surgerySclerosing Cholangitis: Clinicopathologic Features, Imaging Spectrum, and Systemic Approach to Differential Diagnosis.
Korean journal of radiologySecondary Sclerosing Cholangitis in Critically Ill Patients: Clinical Presentation, Cholangiographic Features, Natural History, and Outcome: A Series of 16 Cases.
MedicineHow to Diagnose Immunoglobulin G4-Associated Cholangitis: The Jack-of-All-Trades in the Biliary Tract.
ViszeralmedizinUpdate on Sclerosing Cholangitis in Critically Ill Patients.
ViszeralmedizinThe Onset of de novo Hepatocellular Carcinoma after Liver Transplantation can be both of Donor and Recipient origin. A Case Report.
Journal of gastrointestinal and liver diseases : JGLDSecondary Sclerosing Cholangitis and Hodgkin's Lymphoma.
Clinical medicine insights. Case reportsSecondary sclerosing cholangitis following major burn.
Annals of hepatologySecondary sclerosing cholangitis rapidly leading to liver cirrhosis: a possible post-ICU treatment sequel.
QJM : monthly journal of the Association of PhysiciansLiver transplantation for critically Ill patients with secondary sclerosing cholangitis: Outcome and complications.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society[A case of secondary sclerosing cholangitis caused by chemotherapy with nab-paclitaxel].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyExtensive multifocal branch duct IPMN of the pancreas after liver transplantation: is surgery justified?
European journal of medical researchTrigger mechanisms of secondary sclerosing cholangitis in critically ill patients.
Critical care (London, England)Secondary sclerosing cholangitis in patients with drug-induced liver injury.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverIgG4-associated cholangitis.
Journal of the Mississippi State Medical AssociationMicrobiological analysis of bile and its impact in critically ill patients with secondary sclerosing cholangitis.
The Journal of infectionIgG4-related sclerosing cholangitis in the absence of autoimmune pancreatitis mimicking extrahepatic cholangiocarcinoma.
Scandinavian journal of gastroenterologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Colangite esclerosante secundária.
É de uma associação que acompanha esta doença? Fale com a gente →
Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Colangite esclerosante secundária e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
A condição é relacionada na literatura científica a insultos secundários, como internações em estado crítico e lesões biliares induzidas por drogas. Não há genes causais catalogados nos registros oficiais para esta afecção.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
3 fontes citadas no texto · 9 publicações do grafo RarasNet (PubMed) · 4 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. (2026). Colangite esclerosante secundária. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/colangite-esclerosante-secundaria
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Colangite esclerosante secundária
📋 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
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata
- Ensaios clínicos
- fonte: ClinicalTrials.gov
