Selective immune deficiency in IgA. Intestinal malabsorption syndrome and mixed connective tissue disease

We report the case of a 22-year old unmarried woman who presented with selective IgA immune deficiency, moderate intestinal malabsorption syndrome with pseudo-atrophy of the villi, and mixed connective tissue disease. Although immune deficiency and malabsorption syndrome are frequently associated, association with an autoimmune disease is rare and we were unable to find any case of association with a connective tissue disease in the literature.