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

J. Matthias Löhr*, Ulrich Beuers, Miroslav Vujasinovic, Domenico Alvaro, Jens Brøndum Frøkjær, Frank Buttgereit, Gabriele Capurso, Emma L. Culver, Enrique De-Madaria, Emanuel Della-Torre, Sönke Detlefsen, Enrique Dominguez-Mu~noz, Piotr Czubkowski, Nils Ewald, Luca Frulloni, Natalya Gubergrits, Deniz Guney Duman, Thilo Hackert, Julio Iglesias-Garcia, Nikolaos KartalisAndrea Laghi, Frank Lammert, Fredrik Lindgren, Alexey Okhlobystin, Grzegorz Oracz, Andrea Parniczky, Raffaella Maria Pozzi Mucelli, Vinciane Rebours, Jonas Rosendahl, Nicolas Schleinitz, Alexander Schneider, Eric F.H. van Bommel, Caroline Sophie Verbeke, Marie Pierre Vullierme, Heiko Witt, Marc G. Besselink, Marco J. Bruno, László Czakó, Marco Del Chiaro, Oleksandra Filippova, Akihisa Fukuda, Sebastien Gaujoux, Phil A. Hart, Peter Hegyi, Eduard Jonas, Alisan Kahraman, Alexander Kleger, Olexander Kuryata, Johanna Laukkarinen, Markus M. Lerch, Giovanni Marchegiani, Hanns Ulrich Marschall, Celso Matos, Yair Molad, Dilek Oguz, Aldis Pukitis, Sohei Satoi, John H. Stone, Joanne Verheij, Niek de Vries

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)637-666
Number of pages30
JournalUnited European Gastroenterology Journal
Issue number6
StatePublished - 1 Jul 2020


  • IgG4-related
  • autoimmune pancreatitis type 1
  • biomarkers
  • cancer
  • diabetes mellitus
  • digestive
  • disease
  • glucocorticoids
  • immune-related cholangitis
  • other organ involvement


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