Is it worth investigating splenic function in patients with celiac disease?

Abstract

Celiac disease, an immune-mediated enteropathy induced in genetically susceptible individuals by the ingestion of gluten, is the most frequent disorder associated with splenic hypofunction or atrophy. Defective splenic function affects more than one-third of adult patients with celiac disease, and it may predispose to a higher risk of infections by encapsulated bacteria and thromboembolic and autoimmune complications, particularly when celiac patients have concomitant pre-malignant and malignant complications (refractory celiac disease, ulcerative jejunoileitis and enteropathy-associated T-cell lymphoma). However, the clinical management of patients with celiac disease does not take into account the evaluation of splenic function, and in patients with high degree of hyposplenism or splenic atrophy the prophylactic immunization with specific vaccines against the polysaccharide antigens of encapsulated bacteria is not currently recommended. We critically re-evaluate clinical and diagnostic aspects of spleen dysfunction in celiac disease, and highlight new perspectives in the prophylactic management of infections in this condition.

Keywords: Hyposplenism; Memory B cell; Pitted red cell; Pneumococcal vaccine; Splenic atrophy.

Figure 1

Schematic representation of immune and filtering function of the spleen. IgM: Immunoglobulin M; Treg: T-regulatory cells.

Figure 2

Correlation between circulating pitted red cells and immunoglobulin M memory B cells in splenectomised patients (A) and hyposplenic celiac patients (B). Among the latter, those affected by pre-malignant or malignant complications of celiac disease are indicated with a square, those affected by concomitant autoimmune disorders are indicated with a triangle. IgM: Immunoglobulin M.