Glucagon-Like Peptide-1 Receptor Agonists: Their Therapeutic Potential in Cystic Fibrosis.
Tirzepatide is stepping into the cystic fibrosis research spotlight, and the early signs look promising. Researchers are taking a closer look at glucagon-like peptide-1 receptor agonists (GLP-1RAs) for cystic fibrosis (CF), especially for people dealing with cystic fibrosis-related diabetes (CFRD). The buzz? GLP-1RAs — including the dual GLP-1/GIP receptor agonist tirzepatide — are showing potential to improve blood sugar control, aid weight management, and even support better lung function.
Adv Ther
by Panou T, Gouveri E, Popovic DS et al.
“Glucagon-Like Peptide-1 Receptor Agonists: Their Therapeutic Potential in Cystic Fibrosis. Panou T(1), Gouveri E(1), Popovic DS(2)(3), Papanas N(4). Author information: (1)Diabetes Centre-Diabetic Foot Clinic, Second Department of Internal Medicine, Democritus University of Thrace, Alexandroupolis, Greece. (2)Clinic for Endocrinology, Diabetes and Metabolic Disorders, Clinical Center of Vojvodina, Novi Sad, Serbia. (3)Medical Faculty, University of Novi Sad, Novi Sad, Serbia. (4)Diabetes Centre-Diabetic Foot Clinic, Second Department of Internal Medicine, Democritus University of Thrace, Alexandroupolis, Greece. papanasnikos@yahoo.gr. Cystic fibrosis (CF) is a monogenic disorder leading to pulmonary disease, pancreatic insufficiency and cystic fibrosis-related diabetes (CFRD). Glucagon-like peptide-1 receptor agonists (GLP-1RAs) are now being investigated in people with cystic fibrosis (pwCF) and CFRD. To date, their therapeutic potential has been almost exclusively studied in case reports or case series. These agents improved glycated haemoglobin (HbA1c) and continuous glucose monitoring (CGM) parameters. Benefits were also observed in weight reduction, particularly for subjects on cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI). However, discordant results have also been reported. Moreover, GLP-1RAs have improved pulmonary function, even following lung transplantation. Importantly, the dual glucagon-like peptide 1 (GLP-1) and glucose-dependent insulinotropic polypeptide (GIP) receptor agonist tirzepatide has also yielded favourable outcomes. Finally, preliminary evidence suggests potential inhibition of bone resorption, pointing to a therapeutic perspective in cystic fibrosis-related bone disease (CFBD). However, potential adverse events should not be ignored. These include risk of acute pancreatitis, nausea/vomiting, nutritional depletion, bowel dysmotility and distal intestinal obstruction syndrome, as well as others. Adverse events should be addressed with caution, and dose adjustments may be useful. Large prospective multicentre studies are now required to validate these outcomes and to suggest implications for clinical practice. © 2026. The Author(s). Conflict of interest statement: Declarations. Conflict of Interest: Theodoros Panou has nothing to disclose. Evanthia Gouverihas attended conferences sponsored by Berlin-Chemie, Sanofi, AstraZeneca, Novo Nordisk, Lilly, Boehringer Ingelheim, Menarini, Petsiavas and KRKA Hellas; received speaker honoraria by AstraZeneca, Boehringer Ingelheim, Sanofi, Menarini. Djordje S. Popovic declares associations with: Abbott (speaker honoraria), ADOC Pharma (sponsored conferences) Alkaloid (speaker honoraria), Amicus (speaker honoraria, sponsored conferences), AstraZeneca (speaker honoraria), Boehringer Ingelheim (speaker honoraria), Berlin-Chemie (speaker honoraria), Eli Lilly (advisory board member, speaker honoraria, sponsored conferences), Galenika (speaker honoraria), Krka (speaker honoraria), Merck (speaker honoraria, sponsored conferences), Novo Nordisk (advisory board member, speaker honoraria, sponsored conferences, participation in sponsored studies), PharmaSwiss (speaker honoraria), Sanofi-Aventis (speaker honoraria, sponsored conferences, participation in sponsored studies), Servier (speaker honoraria), Viatris (speaker honoraria), and Wörwag Pharma (speaker honoraria, sponsored conferences). Nikolaos Papanas has been an advisory board member of AstraZeneca, Bayer, Boehringer Ingelheim, Menarini, MSD, Novo Nordisk, Pfizer, Roche, Takeda and TrigoCare International; has participated in sponsored studies by AstraZeneca, Eli Lilly, GSK, MSD, Novo Nordisk, Novartis and Sanofi-Aventis; has received honoraria as a speaker for AstraZeneca, Bayer, Boehringer Ingelheim, Eli Lilly, ELPEN, Galenica, KRKA, Menarini, MSD, Mylan, Novo Nordisk, Pfizer, Sanofi-Aventis, Takeda, Viatris and Vianex; and attended conferences sponsored by TrigoCare International, Bayer, Eli Lilly, Galenica, Novo Nordisk, Pfizer, Viatris and Sanofi-Aventis. Ethical Approval: This review is based on previously conducted studies and does not contain any new studies with human participants or animals performed by any of the authors.”
Most of the data so far comes from case reports and small case series, but the signals are hard to ignore. Here’s what stands out:
Improved HbA1c and continuous glucose monitoring results in CF and CFRD cases
Weight reduction, especially for CF patients on CFTR modulator therapy like elexacaftor/tezacaftor/ivacaftor (ETI)
Better lung function, including in post-lung transplant scenarios
Early signs of reduced bone resorption, suggesting value for cystic fibrosis-related bone disease (CFBD)
Tirzepatide, with its dual action on GLP-1 and GIP receptors, is getting special attention for these outcomes. The mechanism could mean even broader applications in metabolic and pulmonary research. The main message: more large, prospective studies are needed, but the door is wide open for new research with GLP-1RAs and tirzepatide in CF.
Researchers should keep an eye on managing potential side effects like GI symptoms and nutritional shifts. Dose adjustments and careful monitoring are part of the process, not a roadblock. For those sourcing compounds, the vendor directory is a solid place to connect with suppliers.
CF researchers looking for new angles — this is an area worth digging into.
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