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


RESULTS
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.


CONCLUSIONS
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.
Corrao
G
G
Cattedra di Statistica Medica, Università di Milano-Bicocca, 20126, Milano, Italy. giovanni.corrao@unimib.it
Corazza
G R
GR
Bagnardi
V
V
Brusco
G
G
Ciacci
C
C
Cottone
M
M
Sategna Guidetti
C
C
Usai
P
P
Cesari
P
P
Pelli
M A
MA
Loperfido
S
S
Volta
U
U
Calabró
A
A
Certo
M
M
D000328:Adult; D002446:Celiac Disease / Q000178:diet therapy / Q000235:genetics* / Q000401:mortality*; D015331:Cohort Studies; D018753:Diet, Protein-Restricted; D005260:Female; D005983:Glutens / Q000008:administration & dosage; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000401:mortality; D008297:Male; D008875:Middle Aged; D010349:Patient Compliance; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate; D013997:Time Factors
D005983:Glutens