Etiological Diagnosis of Primary Adrenal Insufficiency Using an Original Flowchart of Immune and Biochemical Markers1
Approximately 70–80% of cases of primary adrenal insufficiency are classified as idiopathic. An effective protocol for the etiological diagnosis of primary adrenal insufficiency is needed to ensure correct patient management. With the aim of developing an algorithm for the etiological diagnosis of p...
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Published in | The journal of clinical endocrinology and metabolism Vol. 83; no. 9; pp. 3163 - 3168 |
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Main Authors | , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
Endocrine Society
01.09.1998
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Online Access | Get full text |
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Summary: | Approximately 70–80% of cases of primary adrenal insufficiency are
classified as idiopathic. An effective protocol for the etiological
diagnosis of primary adrenal insufficiency is needed to ensure correct
patient management. With the aim of developing an algorithm for the
etiological diagnosis of primary adrenal insufficiency, we studied 56
Italian patients with nonsurgical primary adrenal insufficiency and 24
French patients with X-linked adrenoleukodystrophy (ALD) for serum
levels of adrenal cortex, steroid-21-hydroxylase (21OHAb), islet cell
(ICA), glutamate decarboxylase (GAD65Ab), IA2/ICA512 (ICA512Ab),
thyroid peroxidase (TPOAb) autoantibodies, and plasmatic concentrations
of very long chain fatty acids (VLCFA). High levels of 21OH and adrenal
cortex antibodies were found in 35/42 (83%) and 17/42 (40%) Italian
patients with idiopathic adrenal insufficiency, respectively. Levels of
adrenal autoantibodies correlated inversely with disease duration
(P < 0.0001). Elevated VLCFA were found in 4/42
(10%) idiopathic patients. A total of 34/35 (97%) idiopathic patients
with a disease duration of less than 20 yr was positive for either
21OHAb or elevated levels of VLCFA. None of 14 patients with
posttuberculosis adrenal insufficiency had elevated levels of either
adrenal antibodies or VLCFA. ICA, GAD65Ab, ICA512Ab, and TPOAb were
found in 6/56 (11%), 8/56 (14%), 4/56 (7%), and 23/56 (41%)
patients, respectively. None of 24 French ALD patients with adrenal
insufficiency was positive for organ-specific autoantibodies. The
measuring of 21OH antibodies and plasma VLCFA levels enabled a correct
diagnosis of autoimmune (89%) and ALD (8%) in 97% of patients with
idiopathic primary adrenal insufficiency of less than 20 yr of
duration. The results of our study have important therapeutic and
prognostic implications. |
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ISSN: | 0021-972X 1945-7197 |
DOI: | 10.1210/jcem.83.9.5103 |