Annalisa Schiepatti

Summary Background Data on mortality in coeliac disease are contrasting. Aims To systematically review the literature on all‐cause and cause‐specific mortality in coeliac disease compared to the general population, and evaluate differences across clinical phenotypes, geographical regions, and over time. Methods We searched PubMed and Embase from 1 January 1970 to 31 December 2022 for eligible studies reporting on all‐cause and cause‐specific mortality in coeliac disease compared to the general population or controls. The protocol was registered on Open Science Framework ( https://doi.org/10.17605/OSF.IO/852DN ). Results We included 25 studies. All‐cause mortality (HR 1.16, 95% CI 1.05–1.27, I 2 = 89%), mortality due to malignancies (HR 1.21, 95% CI 1.08–1.36, I 2 = 65%) and respiratory disease (HR 1.39, 95% CI 1.04–1.86, I 2 = 76%) were increased. Mortality due to non‐Hodgkin lymphoma (HR 10.14, 95% CI 2.19–46.88, I 2 = 96%) was markedly increased. Mortality significantly decreased in recent decades: 1989–2004 (HR 1.61, 95% CI 1.27–2.03, I 2 = 91%), 2005–2014 (HR 1.16, 95% CI 0.99–1.36, I 2 = 89%), 2015–2022 (HR 1.19, 95% CI 1.05–1.35, I 2 = 93%). All‐cause mortality was not increased in dermatitis herpetiformis (HR 0.85, 95% CI 0.73–0.99, I 2 = 40%) and undiagnosed coeliac disease (HR 1.09, 95% CI 0.95–1.25, I 2 = 0%). Mortality was increased in the UK (HR 1.23, 95% CI 1.03–1.47, I 2 = 91%) but not Scandinavia (HR 1.01, 95% CI 0.91–1.13, I 2 = 81%). Limitations include high heterogeneity and lack of data for many countries. Conclusion Mortality in coeliac disease is increased, predominantly due to malignancies—particularly non‐Hodgkin lymphoma—although differing significantly across disease phenotypes. Mortality of patients with coeliac disease has significantly decreased in recent decades. These results may influence diagnosis and management.