Clinics and research in hepatology and gastroenterology · 2026
Cystic fibrosis-associated inflammatory bowel disease: a case series and literature review.
СтатьяОбзорМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Rochereau Antoine, Treton Xavier, Bourrier Anne, Abitbol Vered, Serrero Mélanie, Nancey Stéphane, Kervégant Anne-Gaëlle, Belleguic Chantal, Bouguen Guillaume
Аннотация
INTRODUCTION: The management of inflammatory bowel disease (IBD) in patients with cystic fibrosis (CF) at risk of respiratory failure and severe lung infection is poorly described. The objective of this study was to describe the phenotype and evolution of IBD associated with CF, as well as the efficacy and tolerance of IBD treatments.
MATERIALS AND METHODS: A national, multicentre, descriptive, retrospective study was conducted. The study included all adult patients with a confirmed diagnosis of CF and IBD identified across participating GETAID centres. Disease characteristics and outcomes were retrieved from patient charts.
RESULTS: A total of 10 cases with IBD associated with CF were identified (six male; median age at diagnosis, 32 years; 10 with Crohn's disease (CD)). In CF, the DeltaF508 mutation was predominant; nine patients had lung involvement, and all had exocrine pancreatic involvement. Disease location and behaviour at diagnosis were similar to those observed in the general IBD population. During follow-up, four patients underwent surgery (40%), and nine patients were treated with immunosuppressants or biologics, resulting in clinical remission for 50% and 66%, respectively. Two patients with severe CF co-colonised with Pseudomonas Aeruginosa and Staphylococcus aureus experienced severe lung infection.
CONCLUSION: Patients with CF are more likely to have CD than ulcerative colitis. In patients with advanced CF, the potential infectious risks associated with IBD therapies require individualised assessment.
MATERIALS AND METHODS: A national, multicentre, descriptive, retrospective study was conducted. The study included all adult patients with a confirmed diagnosis of CF and IBD identified across participating GETAID centres. Disease characteristics and outcomes were retrieved from patient charts.
RESULTS: A total of 10 cases with IBD associated with CF were identified (six male; median age at diagnosis, 32 years; 10 with Crohn's disease (CD)). In CF, the DeltaF508 mutation was predominant; nine patients had lung involvement, and all had exocrine pancreatic involvement. Disease location and behaviour at diagnosis were similar to those observed in the general IBD population. During follow-up, four patients underwent surgery (40%), and nine patients were treated with immunosuppressants or biologics, resulting in clinical remission for 50% and 66%, respectively. Two patients with severe CF co-colonised with Pseudomonas Aeruginosa and Staphylococcus aureus experienced severe lung infection.
CONCLUSION: Patients with CF are more likely to have CD than ulcerative colitis. In patients with advanced CF, the potential infectious risks associated with IBD therapies require individualised assessment.