Autoimmune adrenal insufficiency may be familial or non familial. We report a case of identical female twins, both with Addisons disease and detectable antibodies to adrenal cortex.
Case report: Twin A at age of 27 developed Addisons disease and hypothyroidism. She was found to be positive for anti-adrenal antibodies, anti-thyroid antibodies and intrinsic factor auto antibodies. Therefore Twin B was tested for the same auto antibodies and was found to be positive for all three as well. However Twin B did not develop any symptoms until age of 40.At 40 she developed Addisons with a typical history of weight loss, hyper-pigmentation, tiredness and postural hypotension. No evidence of tuberculosis was found. She has since been well maintained on hydrocortisone25 mg and 12.5 mg along with fludrocortisone 100 micrograms. Her Thyroid function tests are normal.
Interestingly Twin A had Coeliac disease diagnosed at age of 42, but Twin B has gone on to have a normal small bowel biopsy and negative anti-endomysial antibodies.
Discussion: Addisons disease in identical twins was first reported in literature in 1963 (Smith et al). Since then only a limited number of pairs of identical twins with Addisons have been described.
In the developed world, autoimmune adrenal insufficiency accounts for 80% cases and often occurs part of the autoimmune polyglandular syndromes (APGS). Autoimmune adrenal insufficiency may be familial or non-familial. Familial occurrence of Addisons disease was noticed by Wilkins in 1965. Type 1 APGS is a rare autosomal recessive condition with no recognised HLA haplotype. Type 2 APGS presents in early adult life, has an autosomal dominant inheritance and is linked to HLA DR3 and 4.
07 - 09 Nov 2005
Society for Endocrinology