Российский журнал гастроэнтерологии, гепатологии, колопроктологии (Apr 2021)
European Guideline on IgG4-related Digestive Disease — UEG and SGF Evidence-based Recommendations
- J.-M. Lohr,
- U. Beuers,
- M. Vujasinovic,
- D. Alvaro,
- J. B. Frøkjær,
- F. Buttgereit,
- G. Capurso,
- E. L. Culver,
- E. de-Madaria,
- E. Della-Torre,
- S. Detlefsen,
- E. Dominguez-Munoz,
- P. Czubkowski,
- N. Ewald,
- L. Frulloni,
- N. Gubergrits,
- D. G. Duman,
- Th. Hackert,
- J. Iglesias-Garcia,
- N. Kartalis,
- A. Laghi,
- F. Lammert,
- F. Lindgren,
- A. Okhlobystin,
- G. Oracz,
- A. Parniczky,
- R. M. P. Mucelli,
- V. Rebours,
- J. Rosendahl,
- N. Schleinitz,
- A. Schneider,
- E. FH van Bommel,
- C. S. Verbeke,
- M. P. Vullierme,
- H. Witt,
- the UEG guideline working group
Affiliations
- J.-M. Lohr
- Department of Upper Gastrointestinal Diseases, Karolinska University Hospital; Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institutet
- U. Beuers
- Department of Gastroenterology and Hepatology, Amsterdam University Medical Centers, location AMC
- M. Vujasinovic
- Department of Upper Gastrointestinal Diseases, Karolinska University Hospital, Sweden and Department of Medicine Huddinge, Karolinska Institutet
- D. Alvaro
- Department of Translational and Precision Medicine, Sapienza University of Rome
- J. B. Frøkjær
- Department of Radiology, Aalborg University Hospital
- F. Buttgereit
- Department of Rheumatology and Clinical Immunology, Charite University Medicine Berlin
- G. Capurso
- PancreatoBiliary Endoscopy and EUS Division Pancreas Translational and Clinical Research Center IRCCS San Raffaele Scientific Institute
- E. L. Culver
- Translational Gastroenterology Unit, John Radcliffe Hospital and Nuffield Department of Medicine, University of Oxford
- E. de-Madaria
- Gastroenterology Department, Alicante University General Hospital, ISABIAL
- E. Della-Torre
- School of Medicine, Vita-Salute San Raffaele University; Unit of Immunology, Rheumatology, Allergy and Rare Disease (UnIRAR), IRCCS San Raffaele Scientific Institute
- S. Detlefsen
- Department of Pathology, Odense Pancreas Center (OPAC), Odense University Hospital
- E. Dominguez-Munoz
- Department of Gastroenterology and Hepatology, University Hospital of Santiago de Compostela
- P. Czubkowski
- Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children’s Memorial Health Institute
- N. Ewald
- Institute of Endocrinology, Diabetology and Metabolism, Johannes Wesling University hospital, Minden, Germany and Justus Liebig University Giessen
- L. Frulloni
- Department of Medicine, Pancreas Institute, University of Verona
- N. Gubergrits
- Department of Internal Medicine, Donetsk National Medical University
- D. G. Duman
- Department of Gastroenterology, School of Medicine, Marmara University
- Th. Hackert
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg
- J. Iglesias-Garcia
- Department of Gastroenterology and Hepatology, University Hospital of Santiago de Compostela
- N. Kartalis
- Department of Abdominal Radiology, Karolinska University Hospital
- A. Laghi
- Department of Surgical and Medical Sciences and Translational Medicine, Sapienza University of Rome, Sant’Andrea Hospital
- F. Lammert
- Department of Medicine II, Saarland University Medical Center
- F. Lindgren
- Department of Pediatric Gastroenterology, Hepatology and Nutrition, Karolinska University Hospital
- A. Okhlobystin
- I.M. Sechenov First Moscow State Medical University
- G. Oracz
- Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children’s Memorial Health Institute
- A. Parniczky
- Institute for Translational Medicine, Szenta'gothai Research Centre, Medical School, University of Pecs; Heim Pál National Insitute of Pediatrics
- R. M. P. Mucelli
- Department of Abdominal Radiology, Karolinska University Hospital
- V. Rebours
- Pancreatology Department, Beaujon Hospital, Clichy, Universite de Paris
- J. Rosendahl
- Department of Internal Medicine I, Martin Luther University
- N. Schleinitz
- Departement de Medicine Interne Timone, Assistance Publique-Hôpitaux de Marseille, Aix-Marseille Universite
- A. Schneider
- Department of Gastroenterology and Hepatology, Klinikum Bad Hersfeld
- E. FH van Bommel
- Department of Internal Medicine, Dutch National Center of Expertise Retroperitoneal Fibrosis, Albert Schweitzer hospital
- C. S. Verbeke
- Department of Pathology, Oslo University Hospital, and University of Oslo
- M. P. Vullierme
- Radiology Department, Beaujon Hospital
- H. Witt
- Else Kröner-Fresenius-Zentrum für Ernährungsmedizin, Paediatric Nutritional Medicine, Technische Universität München
- the UEG guideline working group
- Journal volume & issue
-
Vol. 31,
no. 1
pp. 80 – 114
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.
