Raras
Buscar doenças, sintomas, genes...
Colangite esclerosante IgG4-relacionada
Mantido por Agente Raras·Colaborar como especialista →

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.

Publicações científicas
269 artigos
Último publicado: 2026 Apr 16
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: K83.0
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico269PubMed
Últimos 10 anos195publicações
Pico202127 papers
Linha do tempo
2026Hoje · 2026🧪 2012Primeiro ensaio clínico📈 2021Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
1Fase 11
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 4 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Colangite esclerosante IgG4-relacionada

🗺️

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

Pesquisa e ensaios clínicos

6 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
197 papers (10 anos)
#1

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

Journal of hepato-biliary-pancreatic sciences2026 Jan 07

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.

#2

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

Hepatology international2026 Feb

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.

#3

Metachronic development of cholangiocarcinoma during treatment for IgG4-related sclerosing cholangitis.

Clinical journal of gastroenterology2026 Feb

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.

#4

Reply to the Comment on "Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan".

Journal of hepato-biliary-pancreatic sciences2026 Mar 09
#5

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

Journal of hepato-biliary-pancreatic sciences2026 Mar 06

Publicações recentes

Ver todas no PubMed

📚 EuropePMC137 artigos no totalmostrando 187

2026

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

Frontiers in medicine
2025

Sump syndrome following hepaticojejunostomy: a case report of IgG4-related sclerosing cholangitis.

Frontiers in medicine
2026

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

Indian journal of pathology &amp; microbiology
2026

Steroid-Resistant Relapse of Autoimmune Pancreatitis with Marked Main Pancreatic Duct Dilatation Mimicking Multifocal Pancreatic Cancer.

Internal medicine (Tokyo, Japan)
2026

[Acute cholecystitis associated with IgG4-related sclerosing cholangitis: a case report].

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
2026

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

Journal of hepato-biliary-pancreatic sciences
2025

A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma.

Journal of liver cancer
2026

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

Hepatology international
2025

Isolated IgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma: A case report and review.

Hepatology forum
2025

Clinicopathological Pearls and Diagnostic Pitfalls in IgG4-Related Disease: Challenging Case Series and Literature Review.

Diagnostics (Basel, Switzerland)
2026

Metachronic development of cholangiocarcinoma during treatment for IgG4-related sclerosing cholangitis.

Clinical journal of gastroenterology
2025

Endoscopic Diagnostics for IgG4-Related Pancreatobiliary Diseases: Current Modalities and Clinical Perspectives.

Diagnostics (Basel, Switzerland)
2026

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)
2025

Endoscopic diagnosis of immunoglobulin G4-related sclerosing cholangitis.

Digestive endoscopy : official journal of the Japan Gastroenterological Endoscopy Society
2025

Usefulness and Safety of Endoscopic Nasobiliary Drainage for Type 1 Autoimmune Pancreatitis.

Pancreas
2024

Multiple intrahepatic artery aneurysms during the treatment for IgG4-related sclerosing cholangitis: A case report.

World journal of hepatology
2024

Journey to diagnosis: An unfinished exploration of IgG4-related sclerosing cholangitis.

World journal of clinical cases
2024

[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 hepatology
2024

Autoimmune Biliary Diseases: A Review of Primary Biliary Cholangitis, Primary Sclerosing Cholangitis, Immunoglobulin G4-Related Sclerosing Cholangitis, and Autoimmune Hepatitis.

The Surgical clinics of North America
2024

A typical case of IgG4 related sclerosing cholangitis.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2024

Imaging of Benign Biliary Tract Disease.

The Indian journal of radiology &amp; imaging
2025

Unexpected diagnosis: IgG4-related sclerosing cholangitis with normal serum IgG4 levels - Unraveling diagnostic and therapeutic challenges.

Revista espanola de enfermedades digestivas
2024

IgG4-related sclerosing cholangitis associated with essential thrombocythemia: A case report.

World journal of clinical cases
2024

Endoscopic Retrograde Cholangiopancreatography-Related Procedures for the Differential Diagnosis of Isolated Immunoglobin G4-Related Sclerosing Cholangitis and Perihilar Cholangiocarcinoma.

Diagnostics (Basel, Switzerland)
2024

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 sciences
2024

A Rare Cause of Hepatic Mass With High Metabolic Activity and Jaundice.

Gastroenterology
2024

Perinatal diagnosis of isolated IgG4-related sclerosing cholangitis: A case report.

Asian journal of surgery
2024

A Duodenal Ulcer and Biliopancreatic Lesions: What Is the Culprit?

Gastroenterology
2024

Fungemia With Wickerhamomyces anomalus: A Case Report and Literature Review.

Cureus
2024

A Review of IgG4-related Sclerosing Cholangitis (IgG4-SC).

Journal of clinical gastroenterology
2024

Mimickers of immunoglobulin G4-related hepatobiliary disease on biopsy.

Seminars in diagnostic pathology
2024

[IgG4 related sclerosing cholangitis:up to date].

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
2024

An atypical case of isolated immunoglobulin G4-related sclerosing cholangitis with a cholangiogram resembling primary sclerosing cholangitis.

Clinical journal of gastroenterology
2024

IgG4-related pancreatobiliary diseases could be associated with onset of pancreatobiliary cancer: A multicenter cohort study.

Journal of hepato-biliary-pancreatic sciences
2023

IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma.

Journal of surgical case reports
2023

A Unique Adverse Event of Radiotherapy in a Patient With IgG4-related Disease: A Case Report.

In vivo (Athens, Greece)
2023

An unusual cause of extrahepatic cholestasis associated with solid liver lesions: a case report.

Acta gastro-enterologica Belgica
2023

Cholangioscopy in IgG4-related sclerosing cholangitis using texture and color enhancement imaging and red dichromatic imaging.

Endoscopy
2023

Primary Sclerosing Cholangitis, Small Duct Primary Sclerosing Cholangitis, IgG4-Related Sclerosing Cholangitis, and Ischemic Cholangiopathy: Diagnostic Challenges on Biopsy.

Surgical pathology clinics
2023

Usefulness of contrast-enhanced ultrasonography for biliary tract disease.

Journal of medical ultrasonics (2001)
2023

Xanthogranulomatous cholecystitis with histologic features suggestive of IgG4 related cholecystitis - A morphologic overlap with IgG4 related disease.

Annals of diagnostic pathology
2023

[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 hepatology
2023

Primary sclerosing cholangitis with partial steroid responsiveness: a case report.

Fujita medical journal
2023

Cholangiocarcinoma Resembling IgG4-related Sclerosing Cholangitis.

Internal medicine (Tokyo, Japan)
2023

Mass-forming immunoglobulin G4-related cholangitis with atypical pancreatic lesions: a case report of difficult diagnosis.

American journal of translational research
2023

Inflammasome and pyroptosis in autoimmune liver diseases.

Frontiers in immunology
2023

IgG4-related sclerosing cholangitis, a mimicker of the cholangiocarcinoma: A case report.

Clinical case reports
2023

IgG4-related sclerosing cholangitis: A case report.

Asian journal of surgery
2023

Comparison of the long-term outcomes between proximal and distal IgG4-related sclerosing cholangitis: A multicenter cohort study.

Journal of gastroenterology and hepatology
2023

Secondary sclerosing cholangitis and IgG4-sclerosing cholangitis - A review of cholangiographic and ultrasound imaging.

Endoscopic ultrasound
2022

Budesonide 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 reports
2023

Reducing 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 hepatology
2022

Intestinal homeostasis in autoimmune liver diseases.

Chinese medical journal
2022

Autoimmune Pancreatitis and Immunoglobulin G4-related Sclerosing Cholangitis: Past, Present, and Future.

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
2023

Navigating the Challenges Associated With a Diagnosis of Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis.

Archives of pathology &amp; laboratory medicine
2023

Request for biliary drainage for IgG4-SC could be waived before steroid administration?

Journal of hepato-biliary-pancreatic sciences
2022

Case Series of Autoimmune Pancreatitis and IgG4-Related Sclerosing Cholangitis.

Cureus
2022

IgG4-Related Disease with Hypophysitis and Cholangitis as the Main Manifestations.

Digestive diseases and sciences
2022

Clinical 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 medicine
2022

IgG4-Related Sclerosing Cholangitis with Cholecystitis Mimicking Cholangiocarcinoma: A Case Report.

Journal of clinical and experimental hepatology
2023

Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis.

Abdominal radiology (New York)
2022

Sclerosing cholangitis may mimic radiological pattern of cholangiocarcinoma: Differential diagnosis and review of literature.

The American journal of the medical sciences
2022

Steroid 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 sciences
2022

IgG4-Related Disease With Gastrointestinal Involvement: Case Reports and Literature Review.

Frontiers in immunology
2022

[Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021)].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
2022

Primary Sclerosing Cholangitis Complicated with Aphthoid Colitis Difficult to Diagnose from IgG4-Related Sclerosing Cholangitis.

Case reports in gastroenterology
2022

IgG4-related sclerosing cholangitis involving the gallbladder mimicking a hilar cholangiocarcinoma.

Endoscopy
2022

Isolated IgG4-related cholecystitis with localized gallbladder wall thickening mimicking gallbladder cancer: a case report and literature review.

BMC gastroenterology
2022

A 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 Liver
2021

Liver-side of inflammatory bowel diseases: Hepatobiliary and drug-induced disorders.

World journal of hepatology
2021

IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease.

Canadian journal of gastroenterology &amp; hepatology
2021

Autoimmune Diseases of Digestive Organs-A Multidisciplinary Challenge: A Focus on Hepatopancreatobiliary Manifestation.

Journal of clinical medicine
2021

Isolated mass-forming IgG4-related sclerosing cholangitis masquerading as extrahepatic cholangiocarcinoma: A case report.

World journal of clinical cases
2021

Histological features of autoimmune pancreatitis and IgG4-related sclerosing cholangitis with a correlation with imaging findings.

Journal of medical ultrasonics (2001)
2021

Hilar cholangiocarcinoma with extensive immunoglobulin G4 reaction.

International journal of clinical and experimental pathology
2021

IgG4-related hepatic inflammatory pseudotumor mimicking cholangiolocellular carcinoma.

Clinical journal of gastroenterology
2021

Gallbladder wall thickening in patients with IgG4-related diseases, with special emphasis on IgG4-related cholecystitis.

Scandinavian journal of gastroenterology
2022

Classification and Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis.

Gut and liver
2021

[The immune-modulation therapy of autoimmune liver diseases].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
2021

Endoscopic retrograde cholangiopancreatography and intraductal ultrasonography in the diagnosis of autoimmune pancreatitis and IgG4-related sclerosing cholangitis.

Journal of medical ultrasonics (2001)
2021

Intrahepatic bile duct rupture associated with IgG4-related sclerosing cholangitis presenting hepatic inflammatory pseudotumor.

Clinical journal of gastroenterology
2021

IgG4-related sclerosing cholangitis: not always an obvious entity.

Annals of gastroenterology
2021

Efficacy 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.]
2021

Diagnosing Biliary Strictures: Distinguishing IgG4-Related Sclerosing Cholangitis From Cholangiocarcinoma and Primary Sclerosing Cholangitis.

Mayo Clinic proceedings. Innovations, quality &amp; outcomes
2021

Autoimmune pancreatitis: Current perspectives.

Indian journal of pathology &amp; microbiology
2021

Systemic IgG4-Related Disease Masquerading as Cholangiocarcinoma: A Case Report Underscoring the Importance of Medical History.

The American journal of case reports
2022

Altered faecal microbiome and metabolome in IgG4-related sclerosing cholangitis and primary sclerosing cholangitis.

Gut
2021

IgG4-related sclerosing cholangitis may be a risk factor for cancer.

Journal of hepato-biliary-pancreatic sciences
2021

Clinical 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 Liver
2021

Clinical 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 sciences
2021

False-positive PET/CT finding of hepatic inflammatory pseudotumor in IgG4-related sclerosing cholangitis.

Clinics and research in hepatology and gastroenterology
2021

Role of Endoscopic Procedures in the Diagnosis of IgG4-Related Pancreatobiliary Disease.

Chonnam medical journal
2021

Immunosuppressant treatment for IgG4-related sclerosing cholangitis: A case report.

World journal of clinical cases
2020

Usefulness 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 cases
2023

IgG4-related autoimmune liver disease.

Minerva gastroenterology
2020

The Diagnosis of Autoimmune Pancreatitis Using Endoscopic Ultrasonography.

Diagnostics (Basel, Switzerland)
2020

Isolated IgG4-related sclerosing cholangitis with normal serum IgG4 levels-A case report.

Clinical case reports
2020

IgG4-related sclerosing cholangitis- A great mimicker.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2021

IgG4-related Sclerosing Cholangitis Complicated with Cholangiocarcinoma and Detected by Forkhead Box P3 Immunohistochemical Staining.

Internal medicine (Tokyo, Japan)
2021

Cholangioscopy findings in IgG4-related sclerosing cholangitis.

Endoscopy
2020

Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review.

The Journal of international medical research
2021

IgG4-related sclerosing cholangitis presenting as an isolated intrahepatic stenosis: a rare presentation of a rare disease.

Endoscopy
2020

Klatskin tumor diagnosed concurrently with IgG4 related sclerosing cholangitis: A case report.

Medicine
2020

Usefulness of peroral cholangioscopy in the differential diagnosis of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma: a single-center retrospective study.

BMC gastroenterology
2020

Differential Diagnosis of IgG4-Related Sclerosing Cholangitis.

AJR. American journal of roentgenology
2020

Review of primary sclerosing cholangitis with increased IgG4 levels.

World journal of gastroenterology
2020

Challenging biliary strictures: pathophysiological features, differential diagnosis, diagnostic algorithms, and new clinically relevant biomarkers - part 1.

Therapeutic advances in gastroenterology
2020

Risks and characteristics of pancreatic cancer and pancreatic relapse in autoimmune pancreatitis patients.

Journal of gastroenterology and hepatology
2020

[Recent Updates of Immunoglobulin G4-related Pancreatobiliary Disease].

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
2020

Role of Peroral Cholangioscopy in the Diagnosis of Primary Sclerosing Cholangitis.

Diagnostics (Basel, Switzerland)
2020

Management of biliary stricture in patients with IgG4-related sclerosing cholangitis.

PloS one
2020

A man in his fifties with abdominal pain, itching and weight loss.

Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
2020

Animal Models of Autoimmune Liver Diseases: a Comprehensive Review.

Clinical reviews in allergy &amp; immunology
2019

IgG4-Producing MALT Lymphoma in the Renal Hilum.

Case reports in oncology
2020

IgG4-related Sclerosing Cholangitis Mimicking Cholangiocarcinoma Diagnosed by Endoscopic Ultrasound-guided Fine-needle Aspiration.

Internal medicine (Tokyo, Japan)
2020

Peribiliary glands: development, dysfunction, related conditions and imaging findings.

Abdominal radiology (New York)
2019

Validity and safety of endoscopic biliary stenting for biliary stricture associated with IgG4-related pancreatobiliary disease during steroid therapy.

Endoscopy international open
2019

IgG4-Related Sclerosing Cholangitis: A Clinical and Imaging Review.

AJR. American journal of roentgenology
2019

[Clinical guidelines for IgG4-related sclerosing cholangitis].

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
2019

Benign hilar bile duct strictures resected as perihilar cholangiocarcinoma.

The British journal of surgery
2019

Diagnosis and management of IgG4-related disease.

Frontline gastroenterology
2019

[Paying attention to the diagnosis and treatment of IgG4-related sclerosing cholangitis].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2019

A rare case of localized IgG4-related sclerosing cholecystitis mimicking gallbladder cancer.

Journal of rural medicine : JRM
2019

[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-enterology
2019

A Curious Case of Autoimmune Pancreatitis with IgG4-related Sclerosing Cholangitis.

Cureus
2019

Activated T-Follicular Helper 2 Cells Are Associated With Disease Activity in IgG4-Related Sclerosing Cholangitis and Pancreatitis.

Clinical and translational gastroenterology
2019

[Diagnosis of extra pancreatic lesions (focused on IgG4-related sclerosing cholangitis)].

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
2019

Autoimmune Diseases of the Biliary Tract: A Review.

The Surgical clinics of North America
2019

Relapse patterns and predictors of IgG4-related diseases involved with autoimmune pancreatitis: A single-center retrospective study of 115 patients.

Journal of digestive diseases
2019

Clinical practice guidelines for IgG4-related sclerosing cholangitis.

Journal of hepato-biliary-pancreatic sciences
2019

Clinical features of isolated proximal-type immunoglobulin G4-related sclerosing cholangitis.

Digestive endoscopy : official journal of the Japan Gastroenterological Endoscopy Society
2018

A good response to steroid therapy in IgG4-related sclerosing cholangitis: a case report.

Clinical and experimental hepatology
2018

[Diagnosis and differential diagnosis of immunoglobulin G4-related hepatobiliary disease].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
2018

[Histopathological features and pathological diagnosis of IgG4-related hepatobiliary diseases].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
2018

[Research status and challenges in immunoglobulin G4-related hepatobiliary disease].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology
2018

IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed with Liver Biopsy.

Case reports in pathology
2018

Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice-Case Report and Literature Review.

Case reports in rheumatology
2019

IgG4-Related Sclerosing Cholangitis and Primary Sclerosing Cholangitis.

Gut and liver
2018

Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis.

Case reports in gastroenterology
2018

Advances in IgG4-related pancreatobiliary diseases.

The lancet. Gastroenterology &amp; hepatology
2018

Immunoglobulin G4-related sclerosing cholangitis.

The Korean journal of internal medicine
2018

Differential diagnostic roles of the serum CA19-9, total bilirubin (TBIL) and the ratio of CA19-9 to TBIL for benign and malignant.

Journal of Cancer
2018

IgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma (Klatskin tumor): a case report of a challenging disease and review of the literature.

Innovative surgical sciences
2018

The 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.)
2018

Hilar cholangiocarcinoma associated with immunoglobulin G4-positive plasma cells and elevated serum immunoglobulin G4 levels.

Hepatology communications
2018

Differential diagnosis of cholangiocarcinoma and IgG4-related sclerosing cholangitis by fluorescence in situ hybridization using transpapillary forceps biopsy specimens.

Journal of hepato-biliary-pancreatic sciences
2018

Localized intrahepatic IgG4-related sclerosing cholangitis (IgG4-SC) as an additional type of IgG4-SC: a systematic analysis of 12 cases.

Scandinavian journal of gastroenterology
2018

The significance of serum IgG4 and CA19-9, autoantibodies in diagnosis and differential diagnosis of IgG4-related sclerosing cholangitis.

Scandinavian journal of gastroenterology
2018

Autoimmune 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)
2018

IgG4-related disease: with emphasis on the biopsy diagnosis of autoimmune pancreatitis and sclerosing cholangitis.

Virchows Archiv : an international journal of pathology
2017

Cholangiocarcinoma and its mimickers in primary sclerosing cholangitis.

Abdominal radiology (New York)
2017

IgG4-related sclerosing cholangitis and chronic sclerosing sialadenitis mimicking cholangiocarcinoma and neck malignancy.

Hepatobiliary &amp; pancreatic diseases international : HBPD INT
2017

IgG4-related sclerosing cholangitis.

Clinical liver disease
2017

IgG4-related Disease and the Liver.

Gastroenterology clinics of North America
2017

Case report: A female case of isolated IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma.

Medicine
2017

Poor response of initial steroid therapy for IgG4-related sclerosing cholangitis with multiple organs affected.

Medicine
2017

[Immunoglobulin G4-related sclerosing cholangitis diagnosed by liver biopsy: a case report].

Nihon Shokakibyo Gakkai zasshi = The Japanese journal of gastro-enterology
2017

IgG4-related sclerosing cholangitis overlapping with autoimmune hepatitis: Report of a case.

Pathology, research and practice
2017

Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report.

BMC surgery
2017

Clinical Features, Response to Treatment, and Outcomes of IgG4-Related Sclerosing Cholangitis.

Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
2017

No negative impact of serum IgG4 levels on clinical outcome in 435 patients with primary sclerosing cholangitis from Japan.

Journal of hepato-biliary-pancreatic sciences
2016

IgG4-related hepatobiliary disease: an overview.

Nature reviews. Gastroenterology &amp; hepatology
2017

Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.

Journal of gastroenterology
2016

Histopathology of IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease.

Seminars in liver disease
2016

Adult bile duct strictures: differentiating benign biliary stenosis from cholangiocarcinoma.

Medical molecular morphology
2016

IgG4-related Sclerosing Cholangitis with No Biliary Stricture but Severe Thickening of the Bile Duct Wall.

Internal medicine (Tokyo, Japan)
2016

[Isolated IgG4-related sclerosing cholangitis].

Zhonghua yi xue za zhi
2016

Two cases of immunoglobulin G4-related sclerosing cholangitis in which transabdominal ultrasonography was useful in diagnosis and follow-up observation.

Journal of medical ultrasonics (2001)
2016

"Biliary Diseases with Pancreatic Counterparts": Cross-sectional Imaging Findings.

Radiographics : a review publication of the Radiological Society of North America, Inc
2016

IgG4-related sclerosing cholangitis: all we need to know.

Journal of gastroenterology
2016

Comparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma.

Clinical radiology
2016

Endoscopic retrograde cholangiopancreatography-related adverse events in patients with type 1 autoimmune pancreatitis.

Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
2016

Primary Sclerosing Cholangitis: Multiple Phenotypes, Multiple Approaches.

Clinics in liver disease
2016

New Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis.

Clinics in liver disease
2015

Extensive Metastatic Cholangiocarcinoma Associated With IgG4-Related Sclerosing Cholangitis Misdiagnosed as Isolated IgG4-Related Sclerosing Cholangitis: A Case Report and Literature Review.

Medicine
2016

Evaluation of indeterminate biliary strictures.

Nature reviews. Gastroenterology &amp; hepatology
2015

[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 Medicine
2015

Magnetic resonance cholangiography: Current and future perspectives.

Clinics and research in hepatology and gastroenterology
2015

Pericardiobiliary Fistulation: A Rare Complication of Therapeutic ERCP in a Patient With IgG4-Related Sclerosing Cholangitis.

ACG case reports journal
2015

Organ Correlation in IgG4-Related Diseases.

Journal of Korean medical science
2015

Assessment in steroid trial for IgG4-related sclerosing cholangitis.

Advances in medical sciences
2015

Hepatobiliary Iminodiacetic Acid Scanning Detects High-Grade Biliary Obstruction Secondary to IgG4-Related Sclerosing Cholangitis.

Journal of nuclear medicine technology
2015

Immunoglobulin G4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma diagnosed with following bile duct resection: report of a case.

International surgery
2014

Difference from bile duct cancer and relationship between bile duct wall thickness and serum IgG/IgG4 levels in IgG4-related sclerosing cholangitis.

Hepato-gastroenterology
2015

IgG4-related sclerosing cholangitis in the absence of autoimmune pancreatitis mimicking extrahepatic cholangiocarcinoma.

Scandinavian journal of gastroenterology
2015

Isolated intrapancreatic IgG4-related sclerosing cholangitis.

World journal of gastroenterology
2015

Comparison of intraductal ultrasonography findings between primary sclerosing cholangitis and IgG4-related sclerosing cholangitis.

Journal of gastroenterology and hepatology

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Colangite esclerosante IgG4-relacionada.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Colangite esclerosante IgG4-relacionada

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ainda não achamos doenças com sintomas parecidos o suficiente.

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.

  1. Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan.
    Journal of hepato-biliary-pancreatic sciences· 2026· PMID 41500843mais citado
  2. Autoimmune liver diseases in the Asia Pacific region: proceedings of the autoimmune liver disease course at APASL 2025.
    Hepatology international· 2026· PMID 41326940mais citado
  3. Metachronic development of cholangiocarcinoma during treatment for IgG4-related sclerosing cholangitis.
    Clinical journal of gastroenterology· 2026· PMID 40889100mais citado
  4. Reply to the Comment on "Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan".
    Journal of hepato-biliary-pancreatic sciences· 2026· PMID 41800696mais citado
  5. Comment on "Multicenter Validation Study of the Clinical Diagnostic Criteria for IgG4-Related Sclerosing Cholangitis 2020 in Japan".
    Journal of hepato-biliary-pancreatic sciences· 2026· PMID 41792570mais citado
  6. Differentiating IgG4-related Sclerosing Cholangitis from Primary Sclerosing Cholangitis: A Comprehensive Systematic Review and Meta-analysis of Diagnostic Features.
    Am J Gastroenterol· 2026· PMID 41989037recente
  7. Eosinophilic cholangitis mimicking IgG4-related sclerosing cholangitis in an older female patient.
    Immunol Med· 2026· PMID 41894615recente
  8. IgG4-related sclerosing cholangitis: navigating diagnostic dilemmas and the challenge of relapse.
    Front Med (Lausanne)· 2026· PMID 41728618recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:447764(Orphanet)
  2. MONDO:0018645(MONDO)
  3. GARD:21867(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. 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

Colangite esclerosante IgG4-relacionada
Compêndio · Raras BR

Colangite esclerosante IgG4-relacionada

ORPHA:447764 · MONDO:0018645
CID-10
K83.0 · Colangite
CID-11
MedGen
UMLS
C4302109
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades