Sept. 15, 2009 -- Patients with celiac disease -- a genetic, inherited disorder marked by intestinal damage -- are at a modestly increased risk of death, as suspected, according to a new study.
But in a surprise finding, the researchers discovered that those with less severe degrees of celiac disease are at higher risk of death than the others.
"There is an increased risk of death from celiac disease," says Jonas Ludvigsson, MD, PhD, the study's lead author and an associate professor of pediatrics at Orebro University Hospital, Sweden. Depending on the severity of the disease, he found the increased risk to range from 35% to 72%.
"But the risk of dying is still very uncommon," he tells WebMD. "Most researchers would have expected the increase [in risk to be higher]," he says. The study is published in this week's issue of TheJournal of the American Medical Association.
About one of every 133 people has celiac disease, according to the Celiac Sprue Association, but only about 3% have been diagnosed. In people with the disease, eating certain types of protein known as gluten -- found in many breads and crackers -- triggers an autoimmune response that results in small intestine damage. That damage, in turn, decreases the ability of the small intestine to absorb nutrients. Malnutrition and other complications follow. Treatment focuses on eating a gluten-free diet.
Although the risk of death for celiac disease patients has been known, less is known about those with a less severe form of the disease. "We studied the early stage of celiac disease as well, inflammation and latent celiac disease," Ludvigsson says.
Ludvigsson and his colleagues looked at data reports on intestinal tissue studied at the microscopic level, collected from biopsies that had been taken from Swedish patients from the years 1969 to 2008.
They divided the biopsy data from more than 46,000 patients into three groups: those with celiac disease, defined by the presence of villous atrophy (intestinal damage); those with a less severe form, in which there is inflammation without villous atrophy of the intestinal lining; and those with latent disease. Patients with latent disease have positive blood tests but no physical findings of intestinal damage or inflammation, and doctors typically take a wait-and-see approach with them before treating.