Immunoregulation Abnormalities in Familial Addison's Disease*

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Abstract
Brothers (3), 2 with Addison''s disease and a monozygous twin discordant for adrenal insufficiency, presented a unique opportunity to evaluate the endocrine and immunological abnormalities associated with this disorder. None of the brothers had clinical evidence of other autoimmune diseases. Each of the twins had elevated titers of antithyroglobulin and antiparietal cell antibodies, and both Addisonian siblings had cytoplasmic islet cell antibodies. The ability of nonspecific Concanavalin A-activated suppressor cells from all 3 siblings to inhibit Ig biosynthesis by pokeweed mitogen-stimulated B cells, cell proliferation by phytohemagglutinin-stimulated T cells and the proliferative response of an allogeneic mixed lymphocyte culture. In comparison to normal controls, suppressor cells from the Addisonian siblings were less efficient in inhibiting both B and T cell activities. The nonAddisonian twin had a lesser degree of impaired suppressor cell function to the B and T cell targets. Suppressor cell activity, as measured by the ability to inhibit proliferation within the mixed lymphocyte culture, was normal in all 3 siblings. The relationship of suppressor cell dysfunction, genetic predisposition and the expression of the autoimmune state are discussed.