European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations

J-Matthias Löhr¹, Ulrich Beuers², Miroslav Vujasinovic³, Domenico Alvaro⁴, Jens Brøndum Frøkjær⁵, Frank Buttgereit⁶, Gabriele Capurso⁷, Emma L Culver⁸, Enrique de-Madaria⁹, Emanuel Della-Torre¹⁰, Sönke Detlefsen¹¹, Enrique Dominguez-Muñoz¹², Piotr Czubkowski¹³, Nils Ewald¹⁴, Luca Frulloni¹⁵, Natalya Gubergrits¹⁶, Deniz Guney Duman¹⁷, Thilo Hackert¹⁸, Julio Iglesias-Garcia¹², Nikolaos Kartalis¹⁹, Andrea Laghi²⁰, Frank Lammert²¹, Fredrik Lindgren²², Alexey Okhlobystin²³, Grzegorz Oracz¹³, Andrea Parniczky²⁴, Raffaella Maria Pozzi Mucelli¹⁹, Vinciane Rebours²⁵, Jonas Rosendahl²⁶, Nicolas Schleinitz²⁷, Alexander Schneider²⁸, Eric Fh van Bommel²⁹, Caroline Sophie Verbeke³⁰, Marie Pierre Vullierme³¹, Heiko Witt³²; UEG guideline working group³³

Affiliations

¹ Department of Upper Gastrointestinal Diseases, Karolinska University Hospital, Stockholm, Sweden and Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institutet, Stockholm, Sweden.
² Department of Gastroenterology and Hepatology, Amsterdam University Medical Centers, location AMC, Amsterdam, the Netherlands.
³ Department of Upper Gastrointestinal Diseases, Karolinska University Hospital, Stockholm, Sweden and Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden.
⁴ Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.
⁵ Department of Radiology, Aalborg University Hospital, Aalborg, Denmark.
⁶ Department of Rheumatology and Clinical Immunology, Charité University Medicine Berlin, Berlin, Germany.
⁷ PancreatoBiliary Endoscopy and EUS Division Pancreas Translational and Clinical Research Center IRCCS San Raffaele Scientific Institute, Milan, Italy.
⁸ Translational Gastroenterology Unit, John Radcliffe Hospital and Nuffield Department of Medicine, University of Oxford, Oxford, UK.
⁹ Gastroenterology Department, Alicante University General Hospital, ISABIAL, Alicante, Spain.
¹⁰ School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Disease (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy.
¹¹ Department of Pathology, Odense Pancreas Center (OPAC), Odense University Hospital, Odense, Denmark.
¹² Department of Gastroenterology and Hepatology, University Hospital of Santiago de Compostela, Santiago de Compostela, Spain.
¹³ Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.
¹⁴ Institute of Endocrinology, Diabetology and Metabolism, Johannes Wesling University hospital, Minden, Germany and Justus Liebig University Giessen, Giessen, Germany.
¹⁵ Department of Medicine, Pancreas Institute, University of Verona, Verona, Italy.
¹⁶ Department of Internal Medicine, Donetsk National Medical University, Lyman, Ukraine.
¹⁷ Department of Gastroenterology, School of Medicine, Marmara University, Istanbul, Turkey.
¹⁸ Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany.
¹⁹ Department of Abdominal Radiology, Karolinska University Hospital, Stockholm, Sweden.
²⁰ Department of Surgical and Medical Sciences and Translational Medicine, Sapienza University of Rome, Sant'Andrea Hospital, Rome, Italy.
²¹ Department of Medicine II, Saarland University Medical Center, Homburg, Germany.
²² Department of Pediatric Gastroenterology, Hepatology and Nutrition, Karolinska University Hospital, Stockholm, Sweden.
²³ I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
²⁴ Institute for Translational Medicine, Szentágothai Research Centre, Medical School, University of Pécs, Pécs, Hungary; Heim Pál National Insitute of Pediatrics, Budapest, Hungary.
²⁵ Pancreatology Department, Beaujon Hospital, Clichy, Université de Paris, France.
²⁶ Department of Internal Medicine I, Martin Luther University, Halle, Germany.
²⁷ Département de Médicine Interne Timone, Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France.
²⁸ Department of Gastroenterology and Hepatology, Klinikum Bad Hersfeld, Bad Hersfeld, Germany.
²⁹ Department of Internal Medicine, Dutch National Center of Expertise Retroperitoneal Fibrosis, Albert Schweitzer hospital, Dordrecht, the Netherlands.
³⁰ Department of Pathology, Oslo University Hospital, Oslo, and University of Oslo, Norway.
³¹ Radiology Department, Beaujon Hospital, Clichy, France.
³² Else Kröner-Fresenius-Zentrum für Ernährungsmedizin, Paediatric Nutritional Medicine, Technische Universität München, Freising, Germany.
³³ See list at the end of this article.

PMID: 32552502
PMCID: PMC7437085
DOI: 10.1177/2050640620934911

Practice Guideline

European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations

J-Matthias Löhr et al. United European Gastroenterol J. 2020 Jul.

. 2020 Jul;8(6):637-666.

doi: 10.1177/2050640620934911. Epub 2020 Jun 18.

Authors

Affiliations

¹ Department of Upper Gastrointestinal Diseases, Karolinska University Hospital, Stockholm, Sweden and Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institutet, Stockholm, Sweden.
² Department of Gastroenterology and Hepatology, Amsterdam University Medical Centers, location AMC, Amsterdam, the Netherlands.
³ Department of Upper Gastrointestinal Diseases, Karolinska University Hospital, Stockholm, Sweden and Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden.
⁴ Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.
⁵ Department of Radiology, Aalborg University Hospital, Aalborg, Denmark.
⁶ Department of Rheumatology and Clinical Immunology, Charité University Medicine Berlin, Berlin, Germany.
⁷ PancreatoBiliary Endoscopy and EUS Division Pancreas Translational and Clinical Research Center IRCCS San Raffaele Scientific Institute, Milan, Italy.
⁸ Translational Gastroenterology Unit, John Radcliffe Hospital and Nuffield Department of Medicine, University of Oxford, Oxford, UK.
⁹ Gastroenterology Department, Alicante University General Hospital, ISABIAL, Alicante, Spain.
¹⁰ School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Disease (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy.
¹¹ Department of Pathology, Odense Pancreas Center (OPAC), Odense University Hospital, Odense, Denmark.
¹² Department of Gastroenterology and Hepatology, University Hospital of Santiago de Compostela, Santiago de Compostela, Spain.
¹³ Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children's Memorial Health Institute, Warsaw, Poland.
¹⁴ Institute of Endocrinology, Diabetology and Metabolism, Johannes Wesling University hospital, Minden, Germany and Justus Liebig University Giessen, Giessen, Germany.
¹⁵ Department of Medicine, Pancreas Institute, University of Verona, Verona, Italy.
¹⁶ Department of Internal Medicine, Donetsk National Medical University, Lyman, Ukraine.
¹⁷ Department of Gastroenterology, School of Medicine, Marmara University, Istanbul, Turkey.
¹⁸ Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany.
¹⁹ Department of Abdominal Radiology, Karolinska University Hospital, Stockholm, Sweden.
²⁰ Department of Surgical and Medical Sciences and Translational Medicine, Sapienza University of Rome, Sant'Andrea Hospital, Rome, Italy.
²¹ Department of Medicine II, Saarland University Medical Center, Homburg, Germany.
²² Department of Pediatric Gastroenterology, Hepatology and Nutrition, Karolinska University Hospital, Stockholm, Sweden.
²³ I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
²⁴ Institute for Translational Medicine, Szentágothai Research Centre, Medical School, University of Pécs, Pécs, Hungary; Heim Pál National Insitute of Pediatrics, Budapest, Hungary.
²⁵ Pancreatology Department, Beaujon Hospital, Clichy, Université de Paris, France.
²⁶ Department of Internal Medicine I, Martin Luther University, Halle, Germany.
²⁷ Département de Médicine Interne Timone, Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France.
²⁸ Department of Gastroenterology and Hepatology, Klinikum Bad Hersfeld, Bad Hersfeld, Germany.
²⁹ Department of Internal Medicine, Dutch National Center of Expertise Retroperitoneal Fibrosis, Albert Schweitzer hospital, Dordrecht, the Netherlands.
³⁰ Department of Pathology, Oslo University Hospital, Oslo, and University of Oslo, Norway.
³¹ Radiology Department, Beaujon Hospital, Clichy, France.
³² Else Kröner-Fresenius-Zentrum für Ernährungsmedizin, Paediatric Nutritional Medicine, Technische Universität München, Freising, Germany.
³³ See list at the end of this article.

PMID: 32552502
PMCID: PMC7437085
DOI: 10.1177/2050640620934911

Abstract

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.

Keywords: IgG4-related; autoimmune pancreatitis type 1; biomarkers; cancer; diabetes mellitus; digestive; disease; glucocorticoids; immune-related cholangitis; other organ involvement.

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Figures

**Figure 1.**
CT pictures of IgG4-RD in the abdomen. Sausage-like pattern of the pancreatic gland with loss of lobulation (left). Contrast enhancement of the distal bile duct (CBD, arrow) indicating IgG4-related cholangitis (IRC). Note the typical kidney lesions (arrowhead) pathognomonic to IgG4-RD underscoring the diagnosis.

**Figure 2.**
Classification of IgG4-related cholangitis (related to statement 3.2).

See this image and copyright information in PMC

Comment in

United European Gastroenterology guideline: How to manage immunoglobulin G4-related digestive diseases.
Matsubayashi H, Kubota K. Matsubayashi H, et al. United European Gastroenterol J. 2020 Jul;8(6):635-636. doi: 10.1177/2050640620927846. United European Gastroenterol J. 2020. PMID: 32628896 Free PMC article. No abstract available.

References

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1. Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis 2020; 79: 77–87. - PubMed
1. Boonstra K, Culver EL, de Buy Wenniger LM, et al. Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4-associated cholangitis from primary sclerosing cholangitis. Hepatology 2014; 59: 1954–1963. - PMC - PubMed
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European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations

Affiliations

European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations

Authors

Affiliations

Abstract

Figures

Comment in

References

Publication types

MeSH terms

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LinkOut - more resources

Full Text Sources

Medical