Acta Paediatrica. Supplement 412: 78-81, 1996.
Ferguson A, Kingstone K
When compared with the general population, patients with coeliac disease (CD) have an increased risk of developing enteropathy-associated T-cell lymphoma (EATCL), esophageal and pharyngeal squamous carcinomas and small intestinal adenocarcinomas. The prevalence of histologically confirmed CD in Edinburgh and the Lothians in 1979 was 61 per 100,000. The National Health Service Central Records of all 653 subjects registered at that time have been flagged, allowing us to analyse mortality in CD. At a mean of 13.5 years, mortality overall was 1.9-fold that of the general population (115 deaths observed. 61.8 expected; p < 0.0001). For both sexes the early mortality was much greater than expected, but the excess steadily diminished with time from diagnosis. Much of the increased mortality from malignant disease was accounted for by deaths from lymphoproliferative disease and esophageal cancer. Interim re-analysis after a further 9 years shows that the pattern of later deaths is consistent with these trends. Clinical and pathological features of lymphomas in CD are described. In serum samples of 41 patients with normal villus architecture while taking a normal diet, but with minor pathological and/or immunological abnormalities, i.e. potential CD, IgA antiendomysium antibodies were positive in 7 with dermatitis herpetiformis but in only 3 others.
Rheinische Friedrich- Wilhelms- Universität Bonn