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
"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 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.
The researchers compared all patients with a comparison group from the
general population and followed them for a median of about seven to nine years
(half were followed longer, half less). Among those with celiac disease, there
were 3,049 deaths; among those with inflammation, 2,967 died and among the
latent group, 183 died.
The increased risk of death, the researchers found, differed by group:
Those with inflammation had a 72% increased risk of death.
Those with celiac disease had a 39% increased risk of death.
Those with latent disease had a 35% increased risk of death.