Higher Mortality Rate for Celiac Disease Patients

Benjamin Lebwohl, MD

Even with a wider range of patients diagnosed with celiac disease, the mortality rate is still higher than the general population.

A team, led by Benjamin Lebwohl, MD, MS, Celiac Disease Center in the Department of Medicine at the Columbia University Medical Center, explored whether celiac disease is linked to increased mortality.

While celiac disease was previously thought to be linked to a modest, but persistent increased long-term mortality risk, it is uncertain whether this risk has changed in the era of wider diagnosis rates, less severe clinical disease, and more widespread availability of gluten-free food.

The investigators examined 49,829 Swedish patients with celiac disease in the population-based cohort study. Each patient was followed up for a median of 12.5 years.

All individuals in the study were diagnosed with celiac disease between 1969-2017 and identified through the Epidemiology Strengthened by histopathology Reports in Sweden (ESPRESSO) cohort.

The patients were observed beginning on the day of their biopsy.

The investigators defined celiac disease by the presence of small intestinal villus atrophy on histopathology specimens between 1969-2017 from Sweden’s 28 pathology departments. The team matched each individual with as many as 5 control participants in the general population by age, sex, county, and calendar period.

The mean age of the study’s population was 32.2 years old and 62.4% of the participants were women.

The investigators sough a primary outcome of all-cause mortality, as well as secondary outcomes of cause-specific mortality. They also compared patients with celiac disease with controls using stratified Cox proportional modeling, stratifying by year of diagnosis.

During the follow-up period, 13.2% of the patients died.

Overall, the mortality rate was 9.7 per 1000 person-years for the patients with celiac disease, significantly higher than the mortality rate of 8.6 deaths per 1000 person-years for the general population (HR, 1.21; 95% CI, 1.17-1.25).

The relative increase in mortality risk was present in all age groups, was greatest in those diagnosed in the age range of 18 to 39 years (1.9 vs 1.1 per 1000 person-years; HR, 1.69; 95% CI, 1.47-1.94).

Among the causes of death, patients with celiac disease were at an increased risk of cardiovascular disease death (3.5 vs 3.4 per 1000 person-years; HR, 1.08; 95% CI, 1.02-1.13), cancer (2.7 vs 2.2 per 1000 person-years; HR, 1.29; 95% CI, 1.22-1.36), and respiratory disease death (0.6 vs 0.5 per 1000 person-years; HR, 1.21; 95% CI, 1.08-1.37).

The overall mortality risk was also largest in the first year after diagnosis (15.3 vs 6.5 per 1000 person-years; HR; 2.34 95% CI, 2.14-2.55).

However, the risk persisted beyond 10 years after diagnosis (10.5 vs 10.1 per 1000 person-years; HR, 1.15; 95% CI, 1.10-1.20).

“In a Swedish population studied between 1969 and 2017, a diagnosis of celiac disease compared with the general population was associated with a small but statistically significant increased mortality risk,” the authors wrote.

This increased risk of celiac disease has been attributed to the multiple morbidities associated with celiac disease including lymphoproliferative malignancy, osteoporotic fracture, type 1 diabetes, and other conditions.

The study, “Association Between Celiac Disease and Mortality Risk in a Swedish Population,” was published online in JAMA.