European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations
Open accessAbstract
The overall objective of these guidelines is to provide evidence‐based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)‐related digestive disease in adults and children. IgG4‐related digestive disease can be diagnosed only with a comprehensive work‐up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra‐pancreatic digestive organs, including IgG4‐related cholangitis. Treatment with glucocorticoids should be weight‐based and initiated at a dose of 0.6–0.8 mg/kg body weight/day orally (typical starting dose 30‐40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2–4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi‐organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added.
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Sclerosing cholangitis spans several different aetiologies converging on final common pathways leading to fibrosis and strictures of the intra- and extrahepatic bile ducts, often with cholestasis and complications related to bile duct obstruction.
“Sclerosing cholangitis spans several different aetiologies converging on final common pathways leading to a clinicopathologic presentation of fibrosis and strictures of the intraand extrahepatic bile ducts, often with concomitant cholestasis and other complications related to bile duct obstruction.[1][2][3]”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [3]
IgG4-related cholangitis, like PSC, is associated with biliary strictures, elevated serum markers of cholestasis, elevated serum IgG4 and similar clinical signs and symptoms; thus IRC and PSC may be very difficult to distinguish.
“Among these, IRC, like PSC, is associated with biliary strictures, elevated serum markers of cholestasis, elevated serum IgG4 and similar clinical signs and symptoms; thus, IRC and PSC may be very difficult to distinguish.3”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [3]
In studies of IRC, the rate of corticosteroid response ranged from 62 to 100% and the relapse rate during tapering or after withdrawal was 30%.
“the rate of corticosteroid response ranged from 62 to 100% and the relapse rate as defined by biochemical and/or imaging findings during tapering or after withdrawal was 30%. 3”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [3]
Treatment response to corticosteroids is regarded as a major diagnostic criterion for IRC.
“Treatment response to corticosteroids is regarded as a major diagnostic criterion for IRC.3,39”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [3]
The vast majority of patients with IRC are clinical, biochemical and cholangiographic responders.
“The vast majority of patients with IRC are clinical, biochemical and cholangiographic responders.”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [3]
Response failure in IRC is associated with a more fibrotic phenotype, multiple bile duct strictures, and multi-organ involvement.
“Response failure was also associated with a more fibrotic phenotype, multiple bile duct strictures, and multi-organ involvement.3”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [3]
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Type 1 and Type 2 Autoimmune Pancreatitis
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- Authors
- Löhr, J., Beuers, U., Vujasinovic, M., Alvaro, D., Frøkjær, J. B., Buttgereit, F., Capurso, G., Culver, E. L., de‐Madaria, E., Della‐Torre, E., Detlefsen, S., Dominguez‐Muñoz, E., Czubkowski, P., Ewald, N., Frulloni, L., Gubergrits, N., Duman, D. G., Hackert, T., Iglesias‐Garcia, J., Kartalis, N., Laghi, A., Lammert, F., Lindgren, F., Okhlobystin, A., Oracz, G., Parniczky, A., Mucelli, R. M. P., Rebours, V., Rosendahl, J., Schleinitz, N., Schneider, A., van Bommel, E. F., Verbeke, C. S., Vullierme, M. P., Witt, H., & the UEG guideline working group
- Affiliations
- Department of Upper Gastrointestinal Diseases Karolinska University Hospital Stockholm Sweden and Department of Clinical Science Intervention and Technology (CLINTEC) Karolinska Institutet Stockholm Sweden
- Amsterdam UMC Location University of Amsterdam (NL)
- Department of Upper Gastrointestinal Diseases Karolinska University Hospital Stockholm Sweden and Department of Medicine Huddinge Karolinska Institutet Stockholm Sweden
- Sapienza University of Rome (IT)
- Aalborg University Hospital (DK)
- Charité – Universitätsmedizin Berlin (DE)
- IRCCS San Raffaele Roma (IT)
- John Radcliffe Hospital (GB)
- Hospital General Universitario de Alicante Doctor Balmis (ES)
- Vita-Salute San Raffaele University (IT)
- Odense University Hospital (DK)
- Department of Gastroenterology and Hepatology University Hospital of Santiago de Compostela Santiago de Compostela Spain
- Children's Memorial Health Institute (PL)
- Justus-Liebig-Universität Gießen (DE)
- University of Verona (IT)
- Donetsk National Medical University (UA)
- Marmara University (TR)
- Heidelberg University (DE)
- Karolinska University Hospital (SE)
- Azienda Ospedaliera Sant'Andrea (IT)
- Universitätsklinikum des Saarlandes (DE)
- Sechenov University (RU)
- Institute for Translational Medicine Szentágothai Research Centre Medical School University of Pécs Pécs Hungary
- Department of Abdominal Radiology Karolinska University Hospital Stockholm Sweden
- Université Paris Cité (FR)
- Martin Luther University Halle-Wittenberg (DE)
- Aix-Marseille Université (FR)
- Department of Gastroenterology and Hepatology Klinikum Bad Hersfeld Bad Hersfeld Germany
- Department of Internal Medicine Dutch National Center of Expertise Retroperitoneal Fibrosis Albert Schweitzer hospital Dordrecht the Netherlands
- Oslo University Hospital (NO)
- Hôpital Beaujon (FR)
- Else Kröner Fresenius Zentrum für Ernährungsmedizin (DE)
- ORCID
- Löhr, J‐Matthias
- Beuers, Ulrich
- Alvaro, Domenico
- Frøkjær, Jens Brøndum
- Buttgereit, Frank
- Capurso, Gabriele
- Culver, Emma L
- de‐Madaria, Enrique
- Della‐Torre, Emanuel
- Detlefsen, Sönke
- Czubkowski, Piotr
- Frulloni, Luca
- Gubergrits, Natalya
- Duman, Deniz Guney
- Hackert, Thilo
- Kartalis, Nikolaos
- Laghi, Andrea
- Lammert, Frank
- Lindgren, Fredrik
- Okhlobystin, Alexey
- Oracz, Grzegorz
- Rebours, Vinciane
- Rosendahl, Jonas
- Schleinitz, Nicolas
- van Bommel, Eric FH
- Verbeke, Caroline Sophie
- Vullierme, Marie Pierre
- Witt, Heiko
- Venue
- United European Gastroenterology Journal
- Publisher
- Wiley
- Published
- 2020-07
- Volume
- 8
- Issue
- 6
- Pages
- 637-666
- ISSN
- 2050-6406 (ISSN-L)
- 2050-6414
- PMC
- PMC7437085
- OpenAlex
- W3035831639
- Cited by
- 252
- References
- 234
- Citations by year
- 2026: 40 · 2025: 43 · 2024: 46 · 2023: 40 · 2022: 36 · 2021: 38 · 2020: 8 · 2019: 1
- FWCI
- 22.98
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- United European Gastroenterology Journal (publishedVersion)
- PubMed Central (publishedVersion)
- VBN Forskningsportal (Aalborg Universitet) (publishedVersion, other-oa)
- PubMed (publishedVersion)
- SZTE Publicatio Repozitórium (University of Szeged) (acceptedVersion, other-oa)
- Repository of the Academy's Library (Library of the Hungarian Academy of Sciences) (acceptedVersion)
- University of Southern Denmark Research Portal (University of Southern Denmark) (acceptedVersion, other-oa)
- IRIS Research product catalog (Sapienza University of Rome) (submittedVersion, other-oa)
- Pure Amsterdam UMC (submittedVersion, other-oa)
- Data Archiving and Networked Services (DANS) (submittedVersion, other-oa)
- Universitätsbibliographie, Universität Duisburg-Essen (submittedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Archivio Istituzionale della Ricerca - Università Vita-Salute San Raffaele (submittedVersion)
- Dspace Repository (Marmara Üniversitesi) (submittedVersion, other-oa)
- VBN Forskningsportal (Aalborg Universitet) (submittedVersion, other-oa)
- NORA - Norwegian Open Research Archives (submittedVersion, other-oa)
- Sage Journals Data (cc-by)
- Funders
- United European Gastroenterology
- Topics
- Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
- Health Sciences › Medicine › Surgery › Pancreatitis Pathology and Treatment
- Health Sciences › Medicine › Oncology › Pancreatic and Hepatic Oncology Research
- Keywords
- IgG4-related sclerosing cholangitis, IgG4-related pancreatitis, glucocorticoid therapy, diagnosis, treatment guidelines, recurrence, immunosuppressive therapy, obstructive jaundice, abdominal pain, pediatric patients
- MeSH terms
- Immunoglobulin G4-Related Disease, Adult, Body Weight, Child, Digestive System Diseases, Dose-Response Relationship, Drug, Europe, Gastroenterology, Glucocorticoids, Humans, Immunosuppressive Agents, Severity of Illness Index, Treatment Outcome, Evidence-Based Medicine, Drug Dosage Calculations, Maintenance Chemotherapy, Induction Chemotherapy
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