ArticlesMortality in patients with coeliac disease and their relatives: a cohort study
Introduction
Findings from population-based studies have shown that the true frequency of coeliac disease is high1 even in countries where it was thought to be rare.2 An increased overall and cancer mortality has been reported in adult patients with coeliac disease3, 4 and their relatives,5 which lends support to the clinical importance of this disorder. Improved knowledge of the wide clinical spectrum of coeliac disease6, 7 and the use of powerful screening tests8, 9 have radically changed the pattern of presentation of this disorder. Previous mortality could be underestimated by the inclusion of subclinical and symptom-free patients, but no information is available on the prognosis of the new forms of coeliac disease. Additionally, the reported 1·9-fold3 and 3·4-fold4 increases in mortality might be excessive because some patients with a less favourable outcome, such as those with refractory sprue and intestinal lymphoma, were probably enrolled in those series.10 The finding that the mortality excess is mainly accounted for by deaths occurring within a short time after diagnosis3 indirectly lends support to this possibility.
We did a prospective study to find out whether differences in patterns of clinical presentation of coeliac disease are associated with differences in prognosis; whether age at diagnosis, time between onset of symptoms that subsequently led to intestinal biopsy (diagnostic delay), and degree of adherance with a gluten-free diet affect mortality; and whether first-degree relatives of these patients have an increased risk of mortality.
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Patients
We identified patients with biopsy-proven coeliac disease from the records of the 11 gastroenterology units. The diagnostic criteria were subtotal or severe partial villous atrophy and crypt hyperplasia, and histological improvement after gluten-free diet. We enrolled 1072 consecutive patients older than 18 years at the time of diagnosis, between Jan 1, 1962, and 31 Dec, 1994, at 11 gastroenterology units. These units, evenly distributed through Italy, were selected from those participating in
Results
Two of the 11 gastroenterology units flagged patients from before 1974, four flagged from 1975 to 1984, and the remaining five flagged after 1985. Median number of patients per unit was 48 (IQR 29–144). Complete data were available except for diagnostic delay and dietary adherence from 68 and 89 clinical records, respectively. Distributions of patients' sex, age at diagnosis, diagnostic delay, pattern of presentation, and adherence with glutenfree diet did not significantly differ between
Discussion
Our study on the mortality of coeliac disease was large with respect to the number of patients enrolled. Although the total number of patient-years at risk accumulated by our cohort (6444) was lower than that noted by Logan and colleagues3 (8823), this difference is unlikely to have affected the accuracy of our mortality estimates, because previous studies3, 15 and our study agree that excess in mortality risk occurs mainly within the first years from diagnosis. Additionally, we have noted a
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