Introdução
O que você precisa saber de cara
A doença relacionada a IgG4 (DR-IgG4), anteriormente conhecida como doença sistêmica relacionada a IgG4, é uma condição inflamatória crônica caracterizada por infiltração tecidual de linfócitos e plasmócitos secretores de IgG4, vários graus de fibrose (cicatrização) e uma resposta geralmente pronta a esteroides orais. Em aproximadamente 51–70% das pessoas com esta doença, as concentrações séricas de IgG4 estão elevadas durante uma fase aguda.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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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 IgG4-relacionada
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Ensaios clínicos abertos e novidades científicas recentes
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Pesquisa e ensaios clínicos
6 ensaios clínicos encontrados.
Publicações mais relevantes
Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan.
The diagnostic performance of clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020 (IgG4-SC2020) has not been fully validated since its proposal as a revision of the 2012 criteria (IgG4-SC2012). We conducted a multicenter validation study to evaluate the diagnostic performance of IgG4-SC2020 using clinical data collected from 1034 patients with IgG4-SC and 447 patients with mimickers, including 143 with pancreatic cancer, 157 with primary sclerosing cholangitis, and 147 with cholangiocarcinoma in Japan. The sensitivity of IgG4-SC2020 was significantly higher than that of IgG4-SC2012 (99.0% vs. 89.1%; p < 0.001). The specificities of both IgG4-SC2020 and IgG4-SC2012 were 100% for pancreatic cancer and cholangiocarcinoma. For primary sclerosing cholangitis, the specificities of IgG4-SC2020 and IgG4-SC2012 were 97.5% and 100%, respectively, with no significant difference (p = 0.123). A total of 113 patients who could not be diagnosed according to the IgG4-SC2012 were successfully diagnosed using IgG4-SC2020. These diagnostic improvements were attributed to the inclusion of MRCP findings (n = 97), the absence of neoplastic cells on histology (n = 15), and the presence of IgG4-related kidney lesions (n = 1). This Japanese multicenter validation study demonstrated that the diagnostic performance of IgG4-SC2020 was superior to that of IgG4-SC2012. UMIN Clinical Trials Registry (UMIN-CTR), UMIN000052984.
Autoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.
Autoimmune liver diseases (AILD) have historically been considered uncommon in the Asia-Pacific region, where viral hepatitis has predominated as the leading cause of chronic liver disease. However, their recognition and clinical relevance have increased substantially in recent years, mirroring global epidemiologic trends. This proceedings report summarizes the educational course on AILD held during the 34th Annual Meeting of the Asian Pacific Association for the Study of the Liver (APASL) in 2025, highlighting contemporary insights into disease burden, diagnosis, and management across diverse populations. International experts presented updated evidence and region-specific considerations concerning autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and IgG4-related sclerosing cholangitis. Key advances in risk stratification, imaging, serology, patient-reported outcome measures (PROMs), and emerging therapeutics were synthesized. Important updates included improved understanding of acute presentations of AIH, evolving risk stratification and second-line pharmacologic approaches in PBC, and advances in PSC diagnostics incorporating novel imaging modalities and emerging autoantibodies. The unique clinical phenotype and steroid responsiveness of IgG4-related sclerosing cholangitis were also emphasized. Increasing incorporation of PROMs into clinical practice and trials, as well as progress in targeted therapies-such as peroxisome proliferator-activated receptor agonists, farnesoid X receptor agonists, and ileal bile acid transporter inhibitors-reflects a shift toward precision medicine and improved quality of life. AILD represents a growing clinical challenge in the Asia-Pacific region. Earlier recognition, individualized treatment strategies, and strengthened multinational collaboration are essential to address unmet diagnostic and therapeutic needs and to improve long-term outcomes.
Metachronic development of cholangiocarcinoma during treatment for IgG4-related sclerosing cholangitis.
We report a case of obstructive jaundice due to recurrent distal biliary stricture during 3 years of treatment for immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) associated with autoimmune pancreatitis. Although a relapse of IgG4-SC was initially suspected, imaging findings, laboratory tests, and histopathological examinations led to the diagnosis of metachronous cholangiocarcinoma. The patient underwent pancreaticoduodenectomy, and no cancer recurrence was noted 6 months postoperatively. Distal cholangiocarcinoma and IgG4-SC remission were observed in the resected specimen. In patients with recurrent biliary strictures during IgG4-SC treatment, comprehensive evaluations are essential because of the risk of disease relapse and development of metachronous cholangiocarcinoma.
Reply to the Comment on "Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan".
Comment on "Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan".
Publicações recentes
Differentiating IgG4-related Sclerosing Cholangitis from Primary Sclerosing Cholangitis: A Comprehensive Systematic Review and Meta-analysis of Diagnostic Features.
Eosinophilic cholangitis mimicking IgG4-related sclerosing cholangitis in an older female patient.
IgG4-related sclerosing cholangitis: navigating diagnostic dilemmas and the challenge of relapse.
📚 EuropePMC137 artigos no totalmostrando 187
IgG4-related sclerosing cholangitis: navigating diagnostic dilemmas and the challenge of relapse.
Frontiers in medicineSump syndrome following hepaticojejunostomy: a case report of IgG4-related sclerosing cholangitis.
Frontiers in medicineDecoding follicular cholangitis: A benign entity with a malignant façade.
Indian journal of pathology & microbiologySteroid-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-enterologyMulticenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan.
Journal of hepato-biliary-pancreatic sciencesA rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma.
Journal of liver cancerAutoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.
Hepatology internationalIsolated IgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma: A case report and review.
Hepatology forumClinicopathological Pearls and Diagnostic Pitfalls in IgG4-Related Disease: Challenging Case Series and Literature Review.
Diagnostics (Basel, Switzerland)Metachronic development of cholangiocarcinoma during treatment for IgG4-related sclerosing cholangitis.
Clinical journal of gastroenterologyEndoscopic Diagnostics for IgG4-Related Pancreatobiliary Diseases: Current Modalities and Clinical Perspectives.
Diagnostics (Basel, Switzerland)Hepatic Decompensation Caused by IgG4-positive Plasma Cell Infiltration Mimicking Sclerosing Cholangitis, Who Achieved a Four-year Survival by Glucocorticoid Treatment.
Internal medicine (Tokyo, Japan)Endoscopic diagnosis of immunoglobulin G4-related sclerosing cholangitis.
Digestive endoscopy : official journal of the Japan Gastroenterological Endoscopy SocietyUsefulness and Safety of Endoscopic Nasobiliary Drainage for Type 1 Autoimmune Pancreatitis.
PancreasMultiple intrahepatic artery aneurysms during the treatment for IgG4-related sclerosing cholangitis: A case report.
World journal of hepatologyJourney to diagnosis: An unfinished exploration of IgG4-related sclerosing cholangitis.
World journal of clinical cases[Clinical characteristics and prognosis analysis of patients with IgG4-related hepatobiliary-pancreatic disease].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyAutoimmune Biliary Diseases: A Review of Primary Biliary Cholangitis, Primary Sclerosing Cholangitis, Immunoglobulin G4-Related Sclerosing Cholangitis, and Autoimmune Hepatitis.
The Surgical clinics of North AmericaA typical case of IgG4 related sclerosing cholangitis.
Indian journal of gastroenterology : official journal of the Indian Society of GastroenterologyImaging of Benign Biliary Tract Disease.
The Indian journal of radiology & imagingUnexpected diagnosis: IgG4-related sclerosing cholangitis with normal serum IgG4 levels - Unraveling diagnostic and therapeutic challenges.
Revista espanola de enfermedades digestivasIgG4-related sclerosing cholangitis associated with essential thrombocythemia: A case report.
World journal of clinical casesEndoscopic Retrograde Cholangiopancreatography-Related Procedures for the Differential Diagnosis of Isolated Immunoglobin G4-Related Sclerosing Cholangitis and Perihilar Cholangiocarcinoma.
Diagnostics (Basel, Switzerland)Diagnosis of isolated hilar-/extrahepatic-type IgG-4-related sclerosing cholangitis can be increased by improved recognition of this condition-A Japanese multicenter analysis.
Journal of hepato-biliary-pancreatic sciencesA Rare Cause of Hepatic Mass With High Metabolic Activity and Jaundice.
GastroenterologyPerinatal diagnosis of isolated IgG4-related sclerosing cholangitis: A case report.
Asian journal of surgeryA Duodenal Ulcer and Biliopancreatic Lesions: What Is the Culprit?
GastroenterologyFungemia With Wickerhamomyces anomalus: A Case Report and Literature Review.
CureusA Review of IgG4-related Sclerosing Cholangitis (IgG4-SC).
Journal of clinical gastroenterologyMimickers of immunoglobulin G4-related hepatobiliary disease on biopsy.
Seminars in diagnostic pathology[IgG4 related sclerosing cholangitis:up to date].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyAn atypical case of isolated immunoglobulin G4-related sclerosing cholangitis with a cholangiogram resembling primary sclerosing cholangitis.
Clinical journal of gastroenterologyIgG4-related pancreatobiliary diseases could be associated with onset of pancreatobiliary cancer: A multicenter cohort study.
Journal of hepato-biliary-pancreatic sciencesIgG4-related sclerosing cholangitis mimicking cholangiocarcinoma.
Journal of surgical case reportsA Unique Adverse Event of Radiotherapy in a Patient With IgG4-related Disease: A Case Report.
In vivo (Athens, Greece)An unusual cause of extrahepatic cholestasis associated with solid liver lesions: a case report.
Acta gastro-enterologica BelgicaCholangioscopy in IgG4-related sclerosing cholangitis using texture and color enhancement imaging and red dichromatic imaging.
EndoscopyPrimary Sclerosing Cholangitis, Small Duct Primary Sclerosing Cholangitis, IgG4-Related Sclerosing Cholangitis, and Ischemic Cholangiopathy: Diagnostic Challenges on Biopsy.
Surgical pathology clinicsUsefulness of contrast-enhanced ultrasonography for biliary tract disease.
Journal of medical ultrasonics (2001)Xanthogranulomatous cholecystitis with histologic features suggestive of IgG4 related cholecystitis - A morphologic overlap with IgG4 related disease.
Annals of diagnostic pathology[State of recurrent IgG4-related sclerosing cholangitis during the course of hormone therapy: a case report].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyPrimary sclerosing cholangitis with partial steroid responsiveness: a case report.
Fujita medical journalCholangiocarcinoma Resembling IgG4-related Sclerosing Cholangitis.
Internal medicine (Tokyo, Japan)Mass-forming immunoglobulin G4-related cholangitis with atypical pancreatic lesions: a case report of difficult diagnosis.
American journal of translational researchInflammasome and pyroptosis in autoimmune liver diseases.
Frontiers in immunologyIgG4-related sclerosing cholangitis, a mimicker of the cholangiocarcinoma: A case report.
Clinical case reportsIgG4-related sclerosing cholangitis: A case report.
Asian journal of surgeryComparison of the long-term outcomes between proximal and distal IgG4-related sclerosing cholangitis: A multicenter cohort study.
Journal of gastroenterology and hepatologySecondary sclerosing cholangitis and IgG4-sclerosing cholangitis - A review of cholangiographic and ultrasound imaging.
Endoscopic ultrasoundBudesonide with Low-Dose 6-Mercaptopurine as a Possible New Treatment for IgG4-Related Sclerosing Cholangitis and Systemic IgG4-Related Disease: A Case Report.
The American journal of case reportsReducing relapse through maintenance steroid treatment can decrease the cancer risk in patients with IgG4-sclerosing cholangitis: Based on a Japanese nationwide study.
Journal of gastroenterology and hepatologyIntestinal homeostasis in autoimmune liver diseases.
Chinese medical journalAutoimmune Pancreatitis and Immunoglobulin G4-related Sclerosing Cholangitis: Past, Present, and Future.
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chiNavigating the Challenges Associated With a Diagnosis of Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis.
Archives of pathology & laboratory medicineRequest for biliary drainage for IgG4-SC could be waived before steroid administration?
Journal of hepato-biliary-pancreatic sciencesCase Series of Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis.
CureusIgG4-Related Disease with Hypophysitis and Cholangitis as the Main Manifestations.
Digestive diseases and sciencesClinical Utility of Personalized Serum IgG Subclass Ratios for the Differentiation of IgG4-Related Sclerosing Cholangitis (IgG4-SC) from Primary Sclerosing Cholangitis (PSC) and Cholangiocarcinoma (CCA).
Journal of personalized medicineIgG4-Related Sclerosing Cholangitis with Cholecystitis Mimicking Cholangiocarcinoma: A Case Report.
Journal of clinical and experimental hepatologySecondary sclerosing cholangitis: mimics of primary sclerosing cholangitis.
Abdominal radiology (New York)Sclerosing cholangitis may mimic radiological pattern of cholangiocarcinoma: Differential diagnosis and review of literature.
The American journal of the medical sciencesSteroid therapy still plays a crucial role and could serve as a bridge to the next promising treatments in patients with IgG4-related sclerosing cholangitis: Results of a Japanese nationwide study.
Journal of hepato-biliary-pancreatic sciencesIgG4-Related Disease With Gastrointestinal Involvement: Case Reports and Literature Review.
Frontiers in immunology[Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021)].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyPrimary Sclerosing Cholangitis Complicated with Aphthoid Colitis Difficult to Diagnose from IgG4-Related Sclerosing Cholangitis.
Case reports in gastroenterologyIgG4-related sclerosing cholangitis involving the gallbladder mimicking a hilar cholangiocarcinoma.
EndoscopyIsolated IgG4-related cholecystitis with localized gallbladder wall thickening mimicking gallbladder cancer: a case report and literature review.
BMC gastroenterologyA novel serum metabolomic panel distinguishes IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.
Liver international : official journal of the International Association for the Study of the LiverLiver-side of inflammatory bowel diseases: Hepatobiliary and drug-induced disorders.
World journal of hepatologyIgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease.
Canadian journal of gastroenterology & hepatologyAutoimmune Diseases of Digestive Organs-A Multidisciplinary Challenge: A Focus on Hepatopancreatobiliary Manifestation.
Journal of clinical medicineIsolated mass-forming IgG4-related sclerosing cholangitis masquerading as extrahepatic cholangiocarcinoma: A case report.
World journal of clinical casesHistological features of autoimmune pancreatitis and IgG4-related sclerosing cholangitis with a correlation with imaging findings.
Journal of medical ultrasonics (2001)Hilar cholangiocarcinoma with extensive immunoglobulin G4 reaction.
International journal of clinical and experimental pathologyIgG4-related hepatic inflammatory pseudotumor mimicking cholangiolocellular carcinoma.
Clinical journal of gastroenterologyGallbladder wall thickening in patients with IgG4-related diseases, with special emphasis on IgG4-related cholecystitis.
Scandinavian journal of gastroenterologyClassification and Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis.
Gut and liver[The immune-modulation therapy of autoimmune liver diseases].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyEndoscopic retrograde cholangiopancreatography and intraductal ultrasonography in the diagnosis of autoimmune pancreatitis and IgG4-related sclerosing cholangitis.
Journal of medical ultrasonics (2001)Intrahepatic bile duct rupture associated with IgG4-related sclerosing cholangitis presenting hepatic inflammatory pseudotumor.
Clinical journal of gastroenterologyIgG4-related sclerosing cholangitis: not always an obvious entity.
Annals of gastroenterologyEfficacy and safety of rituximab for IgG4-related pancreato-biliary disease: A systematic review and meta-analysis.
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]Diagnosing Biliary Strictures: Distinguishing IgG4-Related Sclerosing Cholangitis From Cholangiocarcinoma and Primary Sclerosing Cholangitis.
Mayo Clinic proceedings. Innovations, quality & outcomesAutoimmune pancreatitis: Current perspectives.
Indian journal of pathology & microbiologySystemic IgG4-Related Disease Masquerading as Cholangiocarcinoma: A Case Report Underscoring the Importance of Medical History.
The American journal of case reportsAltered faecal microbiome and metabolome in IgG4-related sclerosing cholangitis and primary sclerosing cholangitis.
GutIgG4-related sclerosing cholangitis may be a risk factor for cancer.
Journal of hepato-biliary-pancreatic sciencesClinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort.
Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the LiverClinical diagnostic criteria for IgG4-related sclerosing cholangitis 2020: (Revision of the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012).
Journal of hepato-biliary-pancreatic sciencesFalse-positive PET/CT finding of hepatic inflammatory pseudotumor in IgG4-related sclerosing cholangitis.
Clinics and research in hepatology and gastroenterologyRole of Endoscopic Procedures in the Diagnosis of IgG4-Related Pancreatobiliary Disease.
Chonnam medical journalImmunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report.
World journal of clinical casesUsefulness of ultrasonography to assess the response to steroidal therapy for the rare case of type 2b immunoglobulin G4-related sclerosing cholangitis without pancreatitis: A case report.
World journal of clinical casesIgG4-related autoimmune liver disease.
Minerva gastroenterologyThe Diagnosis of Autoimmune Pancreatitis Using Endoscopic Ultrasonography.
Diagnostics (Basel, Switzerland)Isolated IgG4-related sclerosing cholangitis with normal serum IgG4 levels-A case report.
Clinical case reportsIgG4-related sclerosing cholangitis- A great mimicker.
Indian journal of gastroenterology : official journal of the Indian Society of GastroenterologyIgG4-related Sclerosing Cholangitis Complicated with Cholangiocarcinoma and Detected by Forkhead Box P3 Immunohistochemical Staining.
Internal medicine (Tokyo, Japan)Cholangioscopy findings in IgG4-related sclerosing cholangitis.
EndoscopyImmunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review.
The Journal of international medical researchIgG4-related sclerosing cholangitis presenting as an isolated intrahepatic stenosis: a rare presentation of a rare disease.
EndoscopyKlatskin tumor diagnosed concurrently with IgG4 related sclerosing cholangitis: A case report.
MedicineUsefulness of peroral cholangioscopy in the differential diagnosis of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma: a single-center retrospective study.
BMC gastroenterologyDifferential Diagnosis of IgG4-Related Sclerosing Cholangitis.
AJR. American journal of roentgenologyReview of primary sclerosing cholangitis with increased IgG4 levels.
World journal of gastroenterologyChallenging biliary strictures: pathophysiological features, differential diagnosis, diagnostic algorithms, and new clinically relevant biomarkers - part 1.
Therapeutic advances in gastroenterologyRisks and characteristics of pancreatic cancer and pancreatic relapse in autoimmune pancreatitis patients.
Journal of gastroenterology and hepatology[Recent Updates of Immunoglobulin G4-related Pancreatobiliary Disease].
The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chiRole of Peroral Cholangioscopy in the Diagnosis of Primary Sclerosing Cholangitis.
Diagnostics (Basel, Switzerland)Management of biliary stricture in patients with IgG4-related sclerosing cholangitis.
PloS oneA man in his fifties with abdominal pain, itching and weight loss.
Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekkeAnimal Models of Autoimmune Liver Diseases: a Comprehensive Review.
Clinical reviews in allergy & immunologyIgG4-Producing MALT Lymphoma in the Renal Hilum.
Case reports in oncologyIgG4-related Sclerosing Cholangitis Mimicking Cholangiocarcinoma Diagnosed by Endoscopic Ultrasound-guided Fine-needle Aspiration.
Internal medicine (Tokyo, Japan)Peribiliary glands: development, dysfunction, related conditions and imaging findings.
Abdominal radiology (New York)Validity and safety of endoscopic biliary stenting for biliary stricture associated with IgG4-related pancreatobiliary disease during steroid therapy.
Endoscopy international openIgG4-Related Sclerosing Cholangitis: A Clinical and Imaging Review.
AJR. American journal of roentgenology[Clinical guidelines for IgG4-related sclerosing cholangitis].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyBenign hilar bile duct strictures resected as perihilar cholangiocarcinoma.
The British journal of surgeryDiagnosis and management of IgG4-related disease.
Frontline gastroenterology[Paying attention to the diagnosis and treatment of IgG4-related sclerosing cholangitis].
Zhonghua wai ke za zhi [Chinese journal of surgery]A rare case of localized IgG4-related sclerosing cholecystitis mimicking gallbladder cancer.
Journal of rural medicine : JRM[Intraductal papillary neoplasm of bile duct developed in a patient with IgG4-related sclerosing cholangitis, autoimmune pancreatitis, and myasthenia gravis].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyA Curious Case of Autoimmune Pancreatitis with IgG4-related Sclerosing Cholangitis.
CureusActivated T-Follicular Helper 2 Cells Are Associated With Disease Activity in IgG4-Related Sclerosing Cholangitis and Pancreatitis.
Clinical and translational gastroenterology[Diagnosis of extra pancreatic lesions (focused on IgG4-related sclerosing cholangitis)].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyAutoimmune Diseases of the Biliary Tract: A Review.
The Surgical clinics of North AmericaRelapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.
Journal of digestive diseasesClinical practice guidelines for IgG4-related sclerosing cholangitis.
Journal of hepato-biliary-pancreatic sciencesClinical features of isolated proximal-type immunoglobulin G4-related sclerosing cholangitis.
Digestive endoscopy : official journal of the Japan Gastroenterological Endoscopy SocietyA good response to steroid therapy in IgG4-related sclerosing cholangitis: a case report.
Clinical and experimental hepatology[Diagnosis and differential diagnosis of immunoglobulin G4-related hepatobiliary disease].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology[Histopathological features and pathological diagnosis of IgG4-related hepatobiliary diseases].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology[Research status and challenges in immunoglobulin G4-related hepatobiliary disease].
Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatologyIgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed with Liver Biopsy.
Case reports in pathologyImmunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice-Case Report and Literature Review.
Case reports in rheumatologyIgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis.
Gut and liverDifficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis.
Case reports in gastroenterologyAdvances in IgG4-related pancreatobiliary diseases.
The lancet. Gastroenterology & hepatologyImmunoglobulin G4-related sclerosing cholangitis.
The Korean journal of internal medicineDifferential diagnostic roles of the serum CA19-9, total bilirubin (TBIL) and the ratio of CA19-9 to TBIL for benign and malignant.
Journal of CancerIgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma (Klatskin tumor): a case report of a challenging disease and review of the literature.
Innovative surgical sciencesThe Immunobiology of Receptor Activator for Nuclear Factor Kappa B Ligand and Myeloid-Derived Suppressor Cell Activation in Immunoglobulin G4-Related Sclerosing Cholangitis.
Hepatology (Baltimore, Md.)Hilar cholangiocarcinoma associated with immunoglobulin G4-positive plasma cells and elevated serum immunoglobulin G4 levels.
Hepatology communicationsDifferential diagnosis of cholangiocarcinoma and IgG4-related sclerosing cholangitis by fluorescence in situ hybridization using transpapillary forceps biopsy specimens.
Journal of hepato-biliary-pancreatic sciencesLocalized intrahepatic IgG4-related sclerosing cholangitis (IgG4-SC) as an additional type of IgG4-SC: a systematic analysis of 12 cases.
Scandinavian journal of gastroenterologyThe significance of serum IgG4 and CA19-9, autoantibodies in diagnosis and differential diagnosis of IgG4-related sclerosing cholangitis.
Scandinavian journal of gastroenterologyAutoimmune Hemolytic Anemia Obscured by the Obstructive Jaundice Associated with IgG4-related Sclerosing Cholangitis in a Patient with Type 1 Autoimmune Pancreatitis: A Case Report and Review of the Literature.
Internal medicine (Tokyo, Japan)IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis.
Virchows Archiv : an international journal of pathologyCholangiocarcinoma and its mimickers in primary sclerosing cholangitis.
Abdominal radiology (New York)IgG4-related sclerosing cholangitis and chronic sclerosing sialadenitis mimicking cholangiocarcinoma and neck malignancy.
Hepatobiliary & pancreatic diseases international : HBPD INTIgG4-related sclerosing cholangitis.
Clinical liver diseaseIgG4-related Disease and the Liver.
Gastroenterology clinics of North AmericaCase report: A female case of isolated IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma.
MedicinePoor response of initial steroid therapy for IgG4-related sclerosing cholangitis with multiple organs affected.
Medicine[Immunoglobulin G4-related sclerosing cholangitis diagnosed by liver biopsy: a case report].
Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterologyIgG4-related sclerosing cholangitis overlapping with autoimmune hepatitis: Report of a case.
Pathology, research and practiceIsolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report.
BMC surgeryClinical Features, Response to Treatment, and Outcomes of IgG4-Related Sclerosing Cholangitis.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationNo negative impact of serum IgG4 levels on clinical outcome in 435 patients with primary sclerosing cholangitis from Japan.
Journal of hepato-biliary-pancreatic sciencesIgG4-related hepatobiliary disease: an overview.
Nature reviews. Gastroenterology & hepatologyDevelopment of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.
Journal of gastroenterologyHistopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease.
Seminars in liver diseaseAdult bile duct strictures: differentiating benign biliary stenosis from cholangiocarcinoma.
Medical molecular morphologyIgG4-related Sclerosing Cholangitis with No Biliary Stricture but Severe Thickening of the Bile Duct Wall.
Internal medicine (Tokyo, Japan)[Isolated IgG4-related sclerosing cholangitis].
Zhonghua yi xue za zhiTwo cases of immunoglobulin G4-related sclerosing cholangitis in which transabdominal ultrasonography was useful in diagnosis and follow-up observation.
Journal of medical ultrasonics (2001)"Biliary Diseases with Pancreatic Counterparts": Cross-sectional Imaging Findings.
Radiographics : a review publication of the Radiological Society of North America, IncIgG4-related sclerosing cholangitis: all we need to know.
Journal of gastroenterologyComparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma.
Clinical radiologyEndoscopic retrograde cholangiopancreatography-related adverse events in patients with type 1 autoimmune pancreatitis.
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]Primary Sclerosing Cholangitis: Multiple Phenotypes, Multiple Approaches.
Clinics in liver diseaseNew Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis.
Clinics in liver diseaseExtensive Metastatic Cholangiocarcinoma Associated With IgG4-Related Sclerosing Cholangitis Misdiagnosed as Isolated IgG4-Related Sclerosing Cholangitis: A Case Report and Literature Review.
MedicineEvaluation of indeterminate biliary strictures.
Nature reviews. Gastroenterology & hepatology[112th Scientific Meeting of the Japanese Society of Internal Medicine: Symposium: Recent Progress in IgG4-related Disease: Autoimmune Pancreatitis・IgG4-related Sclerosing Cholangitis].
Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal MedicineMagnetic resonance cholangiography: Current and future perspectives.
Clinics and research in hepatology and gastroenterologyPericardiobiliary Fistulation: A Rare Complication of Therapeutic ERCP in a Patient With IgG4-Related Sclerosing Cholangitis.
ACG case reports journalOrgan Correlation in IgG4-Related Diseases.
Journal of Korean medical scienceAssessment in steroid trial for IgG4-related sclerosing cholangitis.
Advances in medical sciencesHepatobiliary Iminodiacetic Acid Scanning Detects High-Grade Biliary Obstruction Secondary to IgG4-Related Sclerosing Cholangitis.
Journal of nuclear medicine technologyImmunoglobulin G4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma diagnosed with following bile duct resection: report of a case.
International surgeryDifference from bile duct cancer and relationship between bile duct wall thickness and serum IgG/IgG4 levels in IgG4-related sclerosing cholangitis.
Hepato-gastroenterologyIgG4-related sclerosing cholangitis in the absence of autoimmune pancreatitis mimicking extrahepatic cholangiocarcinoma.
Scandinavian journal of gastroenterologyIsolated intrapancreatic IgG4-related sclerosing cholangitis.
World journal of gastroenterologyComparison of intraductal ultrasonography findings between primary sclerosing cholangitis and IgG4-related sclerosing cholangitis.
Journal of gastroenterology and hepatologyAssociações
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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.
- Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan.
- Autoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.
- Metachronic development of cholangiocarcinoma during treatment for IgG4-related sclerosing cholangitis.
- Reply to the Comment on "Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan".
- Comment on "Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan".
- Differentiating IgG4-related Sclerosing Cholangitis from Primary Sclerosing Cholangitis: A Comprehensive Systematic Review and Meta-analysis of Diagnostic Features.
- Eosinophilic cholangitis mimicking IgG4-related sclerosing cholangitis in an older female patient.
- IgG4-related sclerosing cholangitis: navigating diagnostic dilemmas and the challenge of relapse.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:447764(Orphanet)
- MONDO:0018645(MONDO)
- GARD:21867(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55788238(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
