Probable autoimmune thyroid disease and combined immunodeficiency disease
M. E. Geffner, E. R. Stiehm, D. Stephure and M. J. Cowan
An 8-year-old girl with combined immunodeficiency secondary to adenosine
deaminase deficiency developed thyroid failure of probable autoimmune
origin manifested by linear growth deceleration, marked bone-age delay, and
myxedema. To our knowledge, this association has not been previously
reported. Immunologic abnormalities included absolute T-cell lymphopenia
and markedly reduced in vitro lymphocyte responses to phytohemagglutinin
and to alloantigen in the mixed lymphocyte reaction. The diagnosis of
autoimmune thyroid disease was suggested by the presence of
antithyroglobulin antibodies in the serum and by decreased, patchy uptake
of iodine 123 on a thyroid scan. Autoimmune thyroid disease may have
developed because the immunodeficient state, with its greater deficiency of
suppressor/cytotoxic T cells, allowed expression of a clone of helper T
lymphocytes specific for thyroidal antigens. Thus, autoimmune disease may
be more common in immunodeficient states and appropriate surveillance
should be instituted.
