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 Kartalis; Andrea 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

doi:10.1177/2050640620934911

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

Clinical Departments

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Pages (from-to)	637-666
Number of pages	30
Journal	United European Gastroenterology Journal
Volume	8
Issue number	6
DOIs	https://doi.org/10.1177/2050640620934911
State	Published - 1 Jul 2020

Keywords

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

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1177/2050640620934911

Cite this

Löhr, J. M., 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-Mu~noz, E., Czubkowski, P., Ewald, N., Frulloni, L., Gubergrits, N., Duman, D. G., Hackert, T., Iglesias-Garcia, J., ... Vries, N. D. (2020). European Guideline on IgG4-related digestive disease – UEG and SGF evidence-based recommendations. United European Gastroenterology Journal, 8(6), 637-666. https://doi.org/10.1177/2050640620934911

@article{331587f2579c4077b5ee058b553879ad,

title = "European Guideline on IgG4-related digestive disease – UEG and SGF evidence-based recommendations",

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",

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

note = "Publisher Copyright: {\textcopyright} Author(s) 2020.",

year = "2020",

month = jul,

day = "1",

doi = "10.1177/2050640620934911",

language = "אנגלית",

volume = "8",

pages = "637--666",

journal = "United European Gastroenterology Journal",

issn = "2050-6406",

publisher = "SAGE Publications Inc.",

number = "6",

}

Löhr, JM, Beuers, U, Vujasinovic, M, Alvaro, D, Frøkjær, JB, Buttgereit, F, Capurso, G, Culver, EL, De-Madaria, E, Della-Torre, E, Detlefsen, S, Dominguez-Mu~noz, E, Czubkowski, P, Ewald, N, Frulloni, L, Gubergrits, N, Duman, DG, Hackert, T, Iglesias-Garcia, J, Kartalis, N, Laghi, A, Lammert, F, Lindgren, F, Okhlobystin, A, Oracz, G, Parniczky, A, Mucelli, RMP, Rebours, V, Rosendahl, J, Schleinitz, N, Schneider, A, van Bommel, EFH, Verbeke, CS, Vullierme, MP, Witt, H, Besselink, MG, Bruno, MJ, Czakó, L, Chiaro, MD, Filippova, O, Fukuda, A, Gaujoux, S, Hart, PA, Hegyi, P, Jonas, E, Kahraman, A, Kleger, A, Kuryata, O, Laukkarinen, J, Lerch, MM, Marchegiani, G, Marschall, HU, Matos, C, Molad, Y, Oguz, D, Pukitis, A, Satoi, S, Stone, JH, Verheij, J & Vries, ND 2020, 'European Guideline on IgG4-related digestive disease – UEG and SGF evidence-based recommendations', United European Gastroenterology Journal, vol. 8, no. 6, pp. 637-666. https://doi.org/10.1177/2050640620934911

TY - JOUR

T1 - European Guideline on IgG4-related digestive disease – UEG and SGF evidence-based recommendations

AU - Löhr, J. Matthias

AU - Beuers, Ulrich

AU - Vujasinovic, Miroslav

AU - Alvaro, Domenico

AU - Frøkjær, Jens Brøndum

AU - Buttgereit, Frank

AU - Capurso, Gabriele

AU - Culver, Emma L.

AU - De-Madaria, Enrique

AU - Della-Torre, Emanuel

AU - Detlefsen, Sönke

AU - Dominguez-Mu~noz, Enrique

AU - Czubkowski, Piotr

AU - Ewald, Nils

AU - Frulloni, Luca

AU - Gubergrits, Natalya

AU - Duman, Deniz Guney

AU - Hackert, Thilo

AU - Iglesias-Garcia, Julio

AU - Kartalis, Nikolaos

AU - Laghi, Andrea

AU - Lammert, Frank

AU - Lindgren, Fredrik

AU - Okhlobystin, Alexey

AU - Oracz, Grzegorz

AU - Parniczky, Andrea

AU - Mucelli, Raffaella Maria Pozzi

AU - Rebours, Vinciane

AU - Rosendahl, Jonas

AU - Schleinitz, Nicolas

AU - Schneider, Alexander

AU - van Bommel, Eric F.H.

AU - Verbeke, Caroline Sophie

AU - Vullierme, Marie Pierre

AU - Witt, Heiko

AU - Besselink, Marc G.

AU - Bruno, Marco J.

AU - Czakó, László

AU - Chiaro, Marco Del

AU - Filippova, Oleksandra

AU - Fukuda, Akihisa

AU - Gaujoux, Sebastien

AU - Hart, Phil A.

AU - Hegyi, Peter

AU - Jonas, Eduard

AU - Kahraman, Alisan

AU - Kleger, Alexander

AU - Kuryata, Olexander

AU - Laukkarinen, Johanna

AU - Lerch, Markus M.

AU - Marchegiani, Giovanni

AU - Marschall, Hanns Ulrich

AU - Matos, Celso

AU - Molad, Yair

AU - Oguz, Dilek

AU - Pukitis, Aldis

AU - Satoi, Sohei

AU - Stone, John H.

AU - Verheij, Joanne

AU - Vries, Niek de

PY - 2020/7/1

Y1 - 2020/7/1

N2 - 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.

AB - 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.

KW - IgG4-related

KW - autoimmune pancreatitis type 1

KW - biomarkers

KW - cancer

KW - diabetes mellitus

KW - digestive

KW - disease

KW - glucocorticoids

KW - immune-related cholangitis

KW - other organ involvement

UR - http://www.scopus.com/inward/record.url?scp=85086576981&partnerID=8YFLogxK

U2 - 10.1177/2050640620934911

DO - 10.1177/2050640620934911

M3 - ???researchoutput.researchoutputtypes.contributiontojournal.article???

C2 - 32552502

AN - SCOPUS:85086576981

SN - 2050-6406

VL - 8

SP - 637

EP - 666

JO - United European Gastroenterology Journal

JF - United European Gastroenterology Journal

IS - 6

ER -

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

Abstract

Keywords

UN SDGs

Access to Document

Other files and links

Fingerprint

Cite this