Cancer incidence and factors associated with malignancies in coeliac disease during long-term follow-up

Abstract Background The increased risk of malignancies in coeliac disease is well recognised, but data on incidence and predisposing factors are scarce. Our aim was to determine cancer incidence and its prognostic factors in coeliac disease during a long-term follow-up. Methods The study cohort comprised all (n = 1460) coeliac patients diagnosed in Tampere University Hospital catchment area during the period 1960-2000. Demographic data (age, gender, decade of diagnosis) and coeliac disease characteristics (presenting symptoms, signs of malabsorption, severity of villous atrophy) were obtained from medical records, and patients were stratified into subgroups accordingly. Standardised incidence ratios (SIRs) compared to general population of the same area were calculated for malignancies altogether and according to stratifications. Results The overall risk of malignancy was not increased (SIR 0.97; 95% confidence interval (CI) 0.84-1.11). The risk of non-Hodgkin lymphoma was elevated (SIR 3.0; 1.88-4.54) but less so after ≥15 years of follow-up (SIR 1.75; 0.70-3.60). Screen-detected coeliac disease or malabsorption at diagnosis was not associated with increased risk of non-Hodgkin lymphoma. The increased risk of gastrointestinal malignancies (SIR 1.42; 95% CI 1.07-1.85), particularly that of stomach and liver cancers, was associated with females, coeliac disease diagnosis before year 1981 and dermatitis herpetiformis. Conclusions The overall risk of malignancy was not increased in any coeliac disease phenotype. The risk of non-Hodgkin lymphoma was increased but not in screen-detected coeliac disease.