Elsevier

The Lancet

Volume 358, Issue 9279, 4 August 2001, Pages 356-361
The Lancet

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

https://doi.org/10.1016/S0140-6736(01)05554-4Get rights and content

Summary

Background

Although previous studies have shown increased mortality in patients with coeliac disease and their relatives, no data are available in relation 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 with coeliac disease consecutively diagnosed in 11 gastroenterology units between 1962 and 1994, and their 3384 first-degree relatives. We compared the number of deaths up to 1998 with expected deaths and expressed the comparison 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 presented with malabsorption symptoms (2·5 [1·8–3·4]), but not in those diagnosed because of minor symptoms (1·1 [0·5–2·2]) or because of antibody screening (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 lymphoma was the main cause of death. No excess of deaths was recorded in relatives 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 lymphoma.

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.

Section snippets

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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