Mortality in patients with coeliac disease and their relatives: a cohort study

Background Although previous studies have shown increased mortality in pati ents with coeliac disease and their relatives, no data are available in rel ation to different patterns of clinical presentation. We assessed mortality in patients with coeliac disease and their first-degree relatives. Methods We enrolled, in a prospective cohort study, 1072 adult patients wit h coeliac disease consecutively diagnosed in 11 gastroenterology units betw een 1962 and 1994, and their 3384 first-degree relatives. We compared the n umber of deaths up to 1998 with expected deaths and expressed the compariso n as standardised mortality ratio (SMR) and relative survival ratio. Findings 53 coeliac patients died compared with 25.9 expected deaths (SMR 2 .0 [95% CI 1.5-2.7]). A significant excess of mortality was evident during the first 3 years after diagnosis of coeliac disease and in patients who pr esented with malabsorption symptoms (2.5 [1.8-3.4]), but not in those diagn osed because of minor symptoms (1.1 [0.5-2.2]) or because of antibody scree ning (1.2 [0.1-7.0]). SMR increased with increasing delay in diagnosis and for patients with poor compliance with gluten-free diet. Non-Hodgkin lympho ma was the main cause of death. No excess of deaths was recorded in relativ es with coeliac disease. Interpretation. Prompt and strict dietary treatment decreases mortality in coeliac patients. Prospective studies are needed to clarify the progression of mild or symptomless coeliac disease and its relation to intestinal lymp homa.