Objectives: Because there is controversy regarding the prevalence, familial
occurrence, and possible factors inducing splenic hypofunction in celiac d
isease, we have reassessed them in a large series of untreated patients and
their first-degree relatives. Methods: Fitted red cell counting was used t
o measure splenic function and the effect that age at diagnosis has on it,
while severity of intestinal lesions and nutritional status were estimated
by multiple linear regression analysis. Moreover, serum tuftsin activity wa
s assayed by measuring its ability to stimulate phagocytosis of opsonized S
taphylococcus aureus. Results: We found that 32.8% of untreated celiacs and
none of their relatives had pitted red cell values in the range of splenic
hypofunction (>4%), Only age at diagnosis, but not the other two covariate
s, was significantly associated with the degree of splenic hypofunction, Tu
ftsin activity was depressed in celiac disease and this reduction was signi
ficantly greater in hyposplenic patients. Conclusions: In celiac disease th
e prevalence of splenic hypofunction is lower than formerly believed. The d
uration of preexposure to gluten is a crucial factor for the prevalence and
severity of this complication that does not affect celiac relatives. In ce
liac disease splenic hypofunction is accompanied by a reduced phagocyte act
ivity linked to the decreased release of tuftsin, (Am J Gastroenterol 1999;
94:391-397, (C) 1999 by Am. Cell. of Gastroenterology).