Coeliac disease with severe hypogammaglobulinaemia.

Abstract

A patient with severe late onset primary hypogammaglobulinaemia developed coeliac disease. The case illustrates that coeliac disease can occur in the virtual absence of local antibody production by plasma cells in the mucosa of the small bowel. Furthermore, our inability to demonstrate specific cellular immunity to a subfraction of gluten raises doubts about the relevance of immunological reactions in the pathogenesis of coeliac disease.

The changing immunological paradigm in coeliac disease

“…The involvement of humoral immunity in the pathogenesis of coeliac disease has been questioned on the basis of a single welldocumented case with severe generalized B-cell deficiency [70]. Nevertheless, it is difficult to exclude that an extremely sensitive effector mechanism such as the complement cascade operates in the intestinal mucosa also in hypogammaglobulinaemia.…”

“…The complement system is usually intact in these patients and they are given gammaglobulin substitution therapy. The systemically administered IgG reaches the intestinal lamina propria [70] and contains antibodies to various dietary antigens, which might initiate subepithelial complement activation as an initial trigger for cellular immune activation. It is also important that these patients often have some Ig-producing PCs in their gut mucosa, particularly of the potentially complement-activating IgM class [71], and they show evidence of increased food protein uptake [72].…”

Avidity progression of dietary antibodies in healthy and coeliac children

“…Serum samples were obtained when the first biopsy was taken (untreated coeliac disease), in remission on a gluten-free diet (treated), and/or at relapse after reintroduction of gluten (challenged). Anti-blactoglobulin antibody levels and avidities were analysed in sera from 16 children with untreated disease (median age 12 months, range [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] and nine with treated disease (median age 35 months, range . Antigliadin antibody levels and avidities were measured in 16 untreated coeliac children (median age 15 months, range 10-24), 11 treated cases (median age 32 months, range 17-57) and in eight coeliac children at relapse on gluten challenge (median age 45 months, range 32-53).…”

A difficult diagnosis of coeliac disease: Repeat duodenal histology increases diagnostic yield in patients with concomitant causes of villous atrophy

“…Consequently, in patients with CVID an alteration of duodenal mucosa poses a difficult differential diagnosis that includes CD. This problem is not only because the specific antibodies for CD have no diagnostic role in patients with CVID, but also because these conditions can be associated in the same patient [17,18]. In fact, Biagi et al [19] demonstrated that in eleven adult patients with duodenal mucosa atrophy and CVID diagnosis, CD was present in 27% of the cases (3/11) and in the other cases (5/11, 46%), the diagnosis of CD could neither be confirmed nor definitely excluded.…”