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Abstract
Amyloidosis is a multisystem disease which may cause organloss. Renal involvement is the most common clinical problem in amyloidosis,however involvement of endocrin organs is possible. In this study to assessadrenocortical function and to evaluate the usefulness of low dose ACTH testin patients with renal amyloidosis, we determined cortisol, 17-hydroxyprogesteron(17-OHP) and 11-deoxycortisol (11-DOC) responses to both 1 μg and 250 μgSynacthen. We also determined the size of adrenal glands radiologically byusing computerized tomography. Twenty one patients with renal amyloidosisand 16 healthy subjects for hormonal evaluation, and 20 patients with renalamyloidosis and 22 healthy subjects for radiologic evaluation were includedin the study. In four patients (19%) peak serum cortisol levels followingstimulation with the low dose of Synacthen were less than 20 μg/dL (550nmol/L). Two of them had also subnormal cortisol response to the 250 μgSynacthen stimulation test. Basal and stimulated levels of 11-DOC were lowerthan those of control values (p = 0.000and p < 0.01 respectively). The mean11-DOC responses to stimulation with 1 μg Synacthen were also significantlylower than the values obtained after the simulation with 250 μg Synacthen(p < 0.01 and p= 0.000). Cortisol responses to the stimulation with 250 μg Synacthen werealso lower than the control responses (p< 0.05). 17-OHP responses were similar to the control values in both tests.In the radiological evaluation the mean maximum width of right adrenal glandsand the mean anterior and maximum width of left adrenal glands were significantlygreater in the patient group (p < 0.01).In conclusion, adrenal involvement and adrenal insufficiency is common inamyloidosis. Low 11-DOC levels in amyloidosis is a new finding and furtherdetailed studies is required to explain its cause.