Evan's syndrome, chronic active hepatitis and focal glomerulonephritis in IgA deficiency

A 10-year-old female with a complete selective IgA deficiency and recurrent autoimmune disease (chronic active hepatitis, focal glomerulonephritis, hemolytic anemia and thrombopenic purpura) is presented. Both serum IgA and saliva secretory IgA were below the detection limit. The small bowel biopsy using a peroxidase-antiperoxidase technique showed absence of plasma cells secreting IgA. Circulating antibodies against mitochondria, microsomal thyroid antigen were detected as well as rheumatoid factor. Circulating immune complexes were present. A positive Coombs' test and a slightly positive reaction for cryoagglutinins were demonstrated. No alterations in cellular immunity were observed. Clinical and analytical improvement with prednisone and azathioprine was obtained.