Malabsorption, Villous Atrophy, and Excessive Serum IgA in a Patient with Unusual Intestinal Immunocyte Infiltration

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Abstract
An 11-year-old boy with gastrointestinal complaints of about 4 years' duration was admitted to hospital in 1967 with severe malabsorption and villous atrophy of the small intestinal mucosa. The clinical symptoms disappeared in association with gluten withdrawal. During the subsequent 10 years, however, the villous atrophy has persisted, and malabsorption tests have been virtually unchanged. Moreover, his serum level of IgA has remained extremely high (58-66 g/l), along with normal IgM and IgG. His IgA is mainly polymeric and shows polyclonal characteristics. Immunohistochemical investigations have demonstrated that the gastrointestinal mucosa is its major production site. About 96 per cent of the intestinal immunocytes are of the IgA class. An increased number of IgA-producing cells are also present in gastric mucosa, whereas nasal mucosa and bone marrow show no abnormality, and peripheral blood contains a normal number of IgA-bearing lymphocytes. The polymeric IgA permeates dermal and mucosal connective tissue in high concentrations and appears in the urine, but there is no marked elevation of secretory IgA levels in duodenal and salivary fluids. Nevertheless, staining of glandular epithelia indicates normal transport of IgA and presence of secretory component (SC), and saliva contains IgA-associated as well as free SC. A defect in its external transport does not therefore seem to be involved in the excessive contribution of dimeric IgA to serum. The possibility is discussed that this case may bear some relation to α-chain disease or coeliac disease.

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