Selective IgA deficiency may be accompanied by other disorders. This immunologic deficit was found to occur in 1.3% of Polish children with celiac disease (CD). This study described the familial occurrance of IgA deficiency and CD. A family consisting of nine members was followed during a period of11 years. Patients were subjected to small intestinal biopsy with subsequent histopathologic evaluation of the mucosa, determination of serum immunoglobulin levels, detection of IgA and IgG anti-endomysial antibodies and HLA class I and II typing. The follow-up revealed four cases of CD, one being in association with selective IgA deficiency. The youngest child was also found to be IgA deficient. Furthermore, genetic susceptibility of the father towards CD was of clinical importance. The study demonstrated that CD may occur in association with selective IgA deficiency among members of the same family. Screening for CD should include the detection of anti-endomysial antibodies, both in IgA and IgG classes.

Keywords: celiac disease, selective IgA deficiency, anti-endomysial antibodies