Background/Aims: The relationship between intestinal Behçet’s disease (BD) and cancer remains unclear. We conducted a nationwide, population-based study to determine the risk of cancer in patients with intestinal BD. Methods: Using the National Health Insurance claims records, we collected data on 365 patients who had been diagnosed with intestinal BD between 2011 and 2014. Standardized incidence ratios (SIRs) of overall and site-specific cancers in patients with intestinal BD in comparison with the general population were calculated. Results: Among 167 men with intestinal BD, four cases of cancer were observed; among 191 women with BD, eight cases of cancer were observed. The risk of all cancers was significantly higher in women with intestinal BD than in women of the general population (SIR, 4.27; 95% confidence interval [CI], 1.84 to 8.41). However, in men with intestinal BD, the risk of all cancers was not significantly higher than that in men of the general population (SIR, 2.08; 95% CI, 0.57 to 5.33). The risk of hematologic cancer was significantly higher in both men and women with intestinal BD than in their counterparts in the general population (SIR, 23.90; 95% CI, 2.89 to 86.32 in men; SIR, 34.47; 95% CI, 4.17 to 124.51 in women). In particular, patients with intestinal BD showed a higher risk of leukemia and myelodysplastic syndrome than the general population. Conclusions: Patients with intestinal BD demonstrated a higher risk of hematologic cancer, especially leukemia, than the general population. Furthermore, women with intestinal BD showed a higher risk of all cancers.
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