Early diagnosis of the disease is essential, researchers say, and so is a gluten-free diet.
Celiac disease is an autoimmune disorder that is often undetected or misdiagnosed in the United States. People who have the disease are allergic to gluten, a natural protein in wheat, rye, barley and possibly oats. Eating these foods can cause severe bouts of diarrhea, cramping and other gastrointestinal problems.
"The fact that the mortality rates are different for celiac patients has not been generally known before this," says Elaine Monarch, executive director of the Celiac Disease Foundation in Studio City, Calif. "This is a nice-size study with well over 1,000 patients who have been studied for a very long period of time, so this is a very viable and important effort."
The study was done by two Italian researchers, Giovanni Corrao, a professor of statistics at the University of Milan-Bicocca, and Dr. Gino Roberto Corazza, a gastroenterologist at the University of Pavia. They followed 1,072 patients who were diagnosed with celiac disease between 1962 and 1994 as well as more than 3,000 of their first-degree relatives. They then compared the number of deaths through 1998 with the expected mortality rate of the general population.
They found that 53 of the celiac patients had died, compared to an expected mortality rate of 25 to 29. A significant number of the deaths occurred within three years of diagnosis, the researchers say, or among those who did not stick to a strict gluten-free diet. The findings were published in the Aug. 4 issue of The Lancet.
"They followed the first-degree relatives because it is well known that one-in-12 relatives of a celiac disease patient will have the disease," says one celiac disease expert, Dr. Karoly Horvath, the director of the Pediatric Gastrointestinal & Nutrition Laboratory at the University of Maryland in Baltimore. "And it's also known that a lot of these relatives are undiagnosed."
People with this disease must stay on gluten-free diets or risk damaging their small intestine and losing the ability to absorb nutrients. The study found that the death rate for those who failed to stick to a gluten-free diet was six times higher than for those who had.
An estimated one-in-4 ,700 Americans has been diagnosed with celiac disease. But recent studies suggest that the number is closer to one-in-250 persons, according to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Although it is the most commonly diagnosed genetic disease in Europe, the disease is rarely diagnosed in African, Chinese, and Japanese people.
"Most adults are misdiagnosed for years, or they are diagnosed with irritable bowel and then years later, their doctor might stumble on celiac disease," Monarch says.
"It's now considered an autoimmune disease, a multi-system -- that means intestinal tract, liver, kidneys -- multi-condition disorder," she explains. "And doctors in the United States don't look for or test for celiac disease. They will test for all the other gastrointestinal disorders, but no one looks for celiac disease."
Often the disease has no symptoms, Karoly says. "What happens with celiac disease is that it progresses very slowly. It's the first part of the 20 feet of intestine that absorbs nutrients and the disease progresses slowly down the length of the intestine. And if the lower intestine can compensate, which it does for a while, then there's no evident symptoms."
The study found the death rate was 2.6 times higher among those whose diagnosis was delayed for a year, and 3.8 times higher for those who weren't diagnosed for 10 years.
"The message in this study is that the more delayed the diagnosis of the disease, the more increased the mortality rate," Karoly says. "And there was a perception in the medical community in the U.S. that there was no such thing as celiac disease in this country, though that's changing now."
"We did a survey in the mid-90's asking celiac patients when they had their first symptoms and when they were diagnosed with the disease," Karoly explains. "The gap was 12 years. The problem is that, while using the screening techniques for celiac disease is quite routine in Europe, it has yet to be recognized as widely in the U.S."
What To Do
For more information on celiac disease, see the Celiac Disease Foundation or the National Institute of Diabetes and Digestive and Kidney Diseases.