cosyntropin and Amyloidosis

Systemic amyloidosis with AA-type amyloid deposition is the major complication of FMF, leading to end stage renal disease. There is no clear data on the prevalence of adrenal involvement in patients with FMF amyloidosis. The aim of this study is to determine the adrenal axis function in patients FMF with amyloidosis. Twenty patients with FMF with amyloidosis (F/M: 10/10, mean age; 38 ± 11 SD years), twenty without amyloidosis (F/M: 14/6, mean age 32 ± 10 years), and healthy controls (F/M: 12/8, mean age: 30 ± 7.6 SD years) were recruited. A dose of 250 mg tetracosactide (Synacthen) was then administered intravenously and further blood samples collected 30 and 60 min later. Blood samples were separated and collected at 4°C, and serum cortisol levels were measured. A normal cortisol response to Synacthen was defined as a post-stimulation peak cortisol value of >18 mg/d either at 30 or 60 min. sample. The mean disease duration was 8.8 ± 6 SD years, (range, 2-21) in FMF patients without amyloidosis compared to 16 ± 9.5 years (range, 0-30) in FMF with amyloidosis (P = 0.001). The cortisol concentrations increased significantly at 30 and 60 min compared to baseline after injection of synacthen in all groups. There were no statistically significant differences found among three groups, for basal, 30 and 60 min for cortisol levels (P = 0.154). FMF patients with amyloidosis do not exhibit overt adrenal insufficiency even though their basal cortisol levels were mildly lower.

Topics: Adrenal Glands; Adult; Amyloidosis; Case-Control Studies; Cosyntropin; Familial Mediterranean Fever; Female; Humans; Hydrocortisone; Male; Middle Aged; Pituitary-Adrenal Function Tests; Pituitary-Adrenal System; Prospective Studies

Amyloidosis is a multisystem disease which may cause organ loss. Renal involvement is the most common clinical problem in amyloidosis, however involvement of endocrin organs is possible. In this study to assess adrenocortical function and to evaluate the usefulness of low dose ACTH test in patients with renal amyloidosis, we determined cortisol, 17-hydroxyprogesteron (17-OHP) and 11-deoxycortisol (11-DOC) responses to both 1 microg and 250 microg Synacthen. We also determined the size of adrenal glands radiologically by using computerized tomography. Twenty one patients with renal amyloidosis and 16 healthy subjects for hormonal evaluation, and 20 patients with renal amyloidosis and 22 healthy subjects for radiologic evaluation were included in the study. In four patients (19%) peak serum cortisol levels following stimulation with the low dose of Synacthen were less than 20 microg/dL (550 nmol/L). Two of them had also subnormal cortisol response to the 250 microg Synacthen stimulation test. Basal and stimulated levels of 11-DOC were lower than those of control values (p=0.000 and p<0.01 respectively). The mean 11-DOC responses to stimulation with 1 microg Synacthen were also significantly lower than the values obtained after the simulation with 250 microg Synacthen (p<0.01 and p=0.000). Cortisol responses to the stimulation with 250 microg Synacthen were also 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 glands and the mean anterior and maximum width of left adrenal glands were significantly greater in the patient group (p<0.01). In conclusion, adrenal involvement and adrenal insufficiency is common in amyloidosis. Low 11-DOC levels in amyloidosis is a new finding and further detailed studies is required to explain its cause.

Topics: 17-alpha-Hydroxyprogesterone; Adolescent; Adrenal Glands; Adult; Amyloidosis; Child; Cortodoxone; Cosyntropin; Delayed-Action Preparations; Female; Humans; Hydrocortisone; Kidney Diseases; Male; Middle Aged; Radiography

In order to know the prevalence rate of adrenal insufficiency among patients with renal failure due to amyloidosis; we have performed a stimulation test with synthetic corticotropin hormone (Nuvacthen test) in nine patients diagnosed of renal amyloidosis and in five controls. We also measured ACTH and plasma cortisol levels. In two out of nine patients (22%) a low ACTH, low plasmatic cortisol and an abnormal response to Nuvacthen test were detected being diagnosed of primary adrenal insufficiency. We conclude that adrenal insufficiency is not an infrequent finding in systemic amyloidosis and may be due to secondary amyloidotic infiltration of adrenal or pituitary glands. Though Nuvacthen test is a useful test to diagnose subclinical adrenal insufficiency, in our series patients with an abnormal response also had low levels of basal cortisol and ACTH and no one patient with normal basal levels had an abnormal response to the stimulation test.

Topics: Adrenal Insufficiency; Adrenocorticotropic Hormone; Adult; Aged; Amyloidosis; Cosyntropin; Female; Humans; Hydrocortisone; Kidney Failure, Chronic; Male; Middle Aged; Stimulation, Chemical; Time Factors

Amyloidosis is a multi-system disease. Renal involvement often leads to end-stage renal failure, which carries a poor prognosis. This paper reports the adrenal status of 22 patients with renal amyloid who were considered for or who had been commenced on renal replacement therapy. Twelve patients were considered or found to have AA amyloid and the remaining 10 had AL amyloid. Of 16 patients tested, seven demonstrated an abnormal response to a synacthen test. Four patients died at Addisonian crisis and hypo-adrenalism probably contributed to the deaths of a further two patients. Amyloid deposition was found in the adrenal glands in seven patients who died of systemic amyloidosis and renal failure. It is recommended that all patients with renal amyloid should have an assessment of adrenal function performed and if abnormal replacement steroid therapy should be commenced.

Topics: Adrenal Gland Diseases; Adrenal Glands; Adult; Aged; Amyloidosis; Cosyntropin; Female; Humans; Kidney; Kidney Diseases; Kidney Failure, Chronic; Kidney Transplantation; Liver; Male; Middle Aged; Myocardium