Celiac disease is a disorder in which the small intestine is abnormally sensitive to gliadin, a component of wheat gluten.
The disease, which affects about 1 in 200 people in developed countries, results in abnormalities in the cells of the intestine, which cannot digest or absorb food normally. A gluten-free diet is essential for treatment of the disease.
Although previous studies have shown increased mortality in patients with celiac disease and their relatives, no data are available in relation to different patterns of clinical presentation.
Giovanni Corrao and Gino Roberto Corazza from the Universities of Milan-Bicocca and Pavia, Italy, assessed mortality in patients with celiac disease and their first-degree relatives (parents, children, or siblings).
1072 adult patients with celiac disease, diagnosed in 11 Italian gastroenterology units between 1962 and 1994, and their 3384 first-degree relatives, were studied prospectively.
| The mortality of patients with celiac disease is double that of the general population.
The investigators compared the number of deaths up to 1998 with the number of expected deaths.
53 celiac patients died, compared with a statistical estimate of 25.9 expected deaths (a standardized mortality ratio [SMR] of 2.0).
A substantial excess of deaths was evident during the first 3 years after diagnosis of celiac disease and in patients who had malabsorption symptoms (SMR 2.5).
SMR increased with increasing delay in diagnosis and for patients with poor compliance with a gluten-free diet.
Non-Hodgkin lymphoma was the main cause of death; no excess of deaths was recorded in relatives with celiac disease.
Giovanni Corrao comments: "Our results confirm that the overall mortality in adult celiac disease patients is double that of the general population, and show that delay in diagnosis, poor adherence to treatment, and severity of symptoms at presentation unfavourably affect patients' outlook.
"Prospective studies are needed to elucidate the progression of mild or symptomless celiac disease and their relation to intestinal lymphoma. However, the normal survival of patients with these forms of disease should not discourage either an active search for these patients or their strict and life-long avoidance of dietary gluten."