Mortality and cancer in eosinophilic gastrointestinal disorders distal to the esophagus: nationwide cohort study 1990–2017

BACKGROUND: Eosinophilic gastrointestinal disorders (EGIDs) include inflammatory conditions with enteric infiltration of eosinophils and resulting symptoms. This study aims to examine a population-based sample of patients for prevalence, mortality, and cancer risk in EGIDs distal to the esophagus. METHODS: Nationwide, population-based cohort study. EGID was identified through relevant biopsy codes from Sweden’s all 28 pathology departments through the ESPRESSO cohort. Individuals with EGID were then matched to general population reference individuals with similar age and sex. Study participants were linked to Swedish healthcare registers. Through Cox regression, we calculated adjusted hazard ratios (aHRs) adjusting for sex, age, county, calendar period, and education. RESULTS: In total, 2429 patients (56% female) were found to have EGID distal to the esophagus, representing a prevalence of about 1/4800 in the Swedish population. Mean age was 44 years with 11% children at the time of diagnosis. Mortality was increased 17% in patients with EGIDs compared to reference individuals (aHR = 1.17; 95%CI = 1.04–1.33). Excess mortality was seen in gastric and small bowel eosinophilic disease, but not colonic disease (aHR = 1.81; 95%CI = 1.32–2.48, aHR = 1.50; 95%CI = 1.18–1.89, and aHR = 0.99; 95%CI = 0.85–1.16, respectively). Cause specific mortality was driven by cancer-related death (aHR = 1.33; 95%CI = 1.05–1.69). However, this study failed to show an increase in incident cancers (aHR = 1.14; 95%CI = 0.96–1.35). Comparison of EGID individuals with their siblings yielded similar aHRs. CONCLUSIONS: This study found an increased risk of death in patients with EGIDs distal to the esophagus, with cancer death driving the increase. Proximal gut disease seems to confer the greatest risk. There was no increase in incident cancers. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00535-022-01904-5.