European guidelines for the diagnosis and treatment of pancreatic exocrine insufficiency
: UEG, EPC, EDS, ESPEN, ESPGHAN, ESDO, and ESPCG evidence-based recommendations
Dominguez-Muñoz, J Enrique ✉; Vujasinovic, Miroslav; de la Iglesia, Daniel; Cahen, Djuna; Capurso, Gabriele; Gubergrits, Natalya; Hegyi, Peter [Hegyi, Péter (Gasztroenterológia), author] Institute of Pancreatic Diseases
(SU / FM / C); Centre for Translational Medicine (SU / KSZE); Institute for Translational
Medicine (UP / UPMS); University of Szeged; Hungin, Pali; Ockenga, Johann; Paiella, Salvatore; Perkhofer, Lukas; Rebours, Vinciane; Rosendahl, Jonas; Salvia, Roberto; Scheers, Isabelle; Szentesi, Andrea [Szentesi, Andrea Ildikó (Pankreatológia), author] Institute for
Translational Medicine (UP / UPMS); Bonovas, Stefanos; Piovani, Daniele; Löhr, J Matthias; Algül, Hana [Collaborator]; European PEI Multidisciplinary Group [Collaborative Organization]; Archibugi, Livia [Collaborator]; Arvanitakis, Marianna [Collaborator]; Barbu, Sorin [Collaborator]; Beyer, Georg [Collaborator]; Bezmarevic, Mihailo [Collaborator]; Bodewes, Frank [Collaborator]; Boermeester, Marja A [Collaborator]; Bordin, Dmitry [Collaborator]; Bruno, Marco [Collaborator]; Ceyhan, Güralp [Collaborator]; Czako, Laszlo [Czakó, László (gasztroenterológia), Collaborator] First Department
of Internal Medicine (SZTE / ASZMS / DIMedicine); D'Amico, Ferdinando [Collaborator]; De Madaria, Enrique [Collaborator]; De Martino, Julian [Collaborator]; Deprez, Pierre H [Collaborator]; Dervenis, Christos [Collaborator]; Dité, Petr [Collaborator]; Drewes, Asbjørn [Collaborator]; Duggan, Sinead N [Collaborator]; Duman, Deniz [Collaborator]; Engjom, Trond [Collaborator]; Ewald, Nils [Collaborator]; Fabian, Ovidiu [Collaborator]; Fracasso, Pierluigi [Collaborator]; Friess, Helmut [Collaborator]; Frøkjær, Jens Brøndum [Collaborator]; Frulloni, Luca [Collaborator]; Gaujoux, Sebastien [Collaborator]; Gheorghe, Cristian [Collaborator]; Gohy, Sophie [Collaborator]; Hardt, Philip [Collaborator]; Hauge, Truls [Collaborator]; Hopper, Andrew [Collaborator]; Iglesias-Garcia, Julio [Collaborator]; Keller, Jutta [Collaborator]; Kiriukova, Mariia [Collaborator]; Kleeff, Jörg [Collaborator]; Kleger, Alexander [Collaborator]; Laghi, Andrea [Collaborator]; Larino-Noia, José [Collaborator]; Laukkarinen, Johanna [Collaborator]; Leeds, John [Collaborator]; Lindkvist, Björn [Collaborator]; Masip, Etna [Collaborator]; Marchegiani, Giovanni [Collaborator]; Mari, Amir [Collaborator]; Martinez-Moneo, Emma [Collaborator]; Mayerle, Julia [Collaborator]; Molven, Anders [Collaborator]; Okhlobystin, Alexey [Collaborator]; Panic, Nikola [Collaborator]; Parniczky, Andrea [Collaborator]; Pezzilli, Raffaele [Collaborator]; Phillips, Mary [Collaborator]; Poropat, Goran [Collaborator]; Matic, Jelena Rakic [Collaborator]; Roberts, Keith [Collaborator]; Robinson, Stuart [Collaborator]; Sandru, Vasile [Collaborator]; Sauvanet, Alain [Collaborator]; Schneider, Alexander [Collaborator]; Shvets, Oleg [Collaborator]; Stigliano, Serena [Collaborator]; Stimac, Davor [Collaborator]; Strobel, Oliver [Collaborator]; Timmerhuis, Hester [Collaborator]; Udrescu, Mihaela [Collaborator]; Vancsa, Szilard [Collaborator]; Veness, Vicki [Collaborator]; Wilschanski, Michael [Collaborator]; Witt, Heiko [Collaborator]
Pancreatic exocrine insufficiency (PEI) is defined as a reduction in pancreatic exocrine
secretion below the level that allows the normal digestion of nutrients. Pancreatic
disease and surgery are the main causes of PEI. However, other conditions and upper
gastrointestinal surgery can also affect the digestive function of the pancreas. PEI
can cause symptoms of nutritional malabsorption and deficiencies, which affect the
quality of life and increase morbidity and mortality. These guidelines were developed
following the United European Gastroenterology framework for the development of high-quality
clinical guidelines. After a systematic literature review, the evidence was evaluated
according to the Oxford Center for Evidence-Based Medicine and the Grading of Recommendations
Assessment, Development, and Evaluation methodology, as appropriate. Statements and
comments were developed by the working groups and voted on using the Delphi method.
The diagnosis of PEI should be based on a global assessment of symptoms, nutritional
status, and a pancreatic secretion test. Pancreatic enzyme replacement therapy (PERT),
together with dietary advice and support, are the cornerstones of PEI therapy. PERT
is indicated in patients with PEI that is secondary to pancreatic disease, pancreatic
surgery, or other metabolic or gastroenterological conditions. Specific recommendations
concerning the management of PEI under various clinical conditions are provided based
on evidence and expert opinions. This evidence-based guideline summarizes the prevalence,
clinical impact, and general diagnostic and therapeutic approaches for PEI, as well
as the specifics of PEI in different clinical conditions. Finally, the unmet needs
for future research are discussed.
