Celiac sprue: a unique autoimmune disorder

Abstract

Celiac sprue (CS) is a gluten-sensitive enteropathy with many autoimmune features. CS involves multiple organs through immune and nonimmune processes, and is frequently associated with other autoimmune disorders. This article reviews the co-occurrence of CS with autoimmune disorders of the cutaneous, nervous, endocrine, musculoskeletal, gastrointestinal and cardiovascular systems. The types of autoimmune disorders associated with CS and the prevalence of CS in other autoimmune disorders are also discussed. A brief review of the literature on the potential mechanisms behind these associations and the therapeutic effects of a gluten-free diet for autoimmune comorbidities in CS is also provided.

Figure 1. Pathogenesis of gluten-sensitive disorders

The two gluten-sensitive disorders, celiac sprue and dermatitis herpetiformis, are the consequences of an abnormal autoimmune response that occurs in the intestine in genetically susceptible individuals (HLA-DQ2 or -DQ8 positive). tTG forms a complex with G and deamidates specific glutamine residues of these peptides. DGPs are then presented to T cells by DCs or B cells. The T cells then provide help to the B cells, resulting in the production of antibodies (mainly IgA but also IgG) against gliadin and DGP, as well as antibodies specific for tTG. These abnormal responses triggered by gliadin can result in either celiac sprue (with mucosal inflammation and loss of villi) or dermatitis herpetiformis (with IgA deposits in the dermal papillae), or even both. DC Dendritic cell; DGP: Deamidated gliadin peptide; G: Gliadin peptide; tTG: Tissue transglutaminase.

Figure 2

Potential mechanisms behind the association of celiac sprue and other autoimmune disorders.