Abstract
Primary adrenal insufficiency (PAI) is clinically evident in one in 8000 individuals. A correct etiological classification is critical for correct disease management. To update the diagnostic criteria for the etiological classification of PAI, a multicentric network was established in Italy, and 222 patients with PAI were studied. Both 21-hydroxylase and adrenal cortex autoantibodies (21OHAb and ACA, respectively) were tested in two independent laboratories on coded samples and found in 65-66% and 58-61% of cases, respectively. Autoimmune polyendocrine syndrome I was diagnosed in 11 of the 222 patients. Of the remaining 211 patients, 38 (18%) had a nonautoimmune form of PAI. In 145 subjects (65%), the presence of adrenal autoantibodies, without signs of other forms of PAI, led to a diagnosis of autoimmune Addison's disease. In six cases (3%), PAI remained idiopathic. Logistic regression analysis showed a 92.2-92.7% probability of correct reclassification for the two 21OHAb assays and 84.5-85.9% for the ACA assays. We conclude that the simultaneous presence of both 21OHAb and ACA permits unambiguous diagnosis of autoimmune Addison's, whereas subjects with low antibody titers should undergo both instrumental and biochemical tests to exclude other causes of PAI. Lastly, we developed a comprehensive flowchart for the classification of PAI for use in routine clinical practice.
| Original language | English |
|---|---|
| Pages (from-to) | 1598-1604 |
| Number of pages | 7 |
| Journal | Journal of Clinical Endocrinology and Metabolism |
| Volume | 89 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - Apr 2004 |
Fingerprint
ASJC Scopus subject areas
- Biochemistry
- Endocrinology, Diabetes and Metabolism
Cite this
Italian addison network study : Update of diagnostic criteria for the etiological classification of primary adrenal insufficiency. / Falorni, Alberto; Laureti, Stefano; De Bellis, Annamaria; Zanchetta, Renato; Tiberti, Claudio; Arnaldi, Giorgio; Bini, Vittorio; Beck-Peccoz, Paolo; Bizzarro, Antonio; Dotta, Francesco; Mantero, Franco; Bellastella, Antonio; Betterle, Corrado; Santeusanio, Fausto.
In: Journal of Clinical Endocrinology and Metabolism, Vol. 89, No. 4, 04.2004, p. 1598-1604.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Italian addison network study
T2 - Update of diagnostic criteria for the etiological classification of primary adrenal insufficiency
AU - Falorni, Alberto
AU - Laureti, Stefano
AU - De Bellis, Annamaria
AU - Zanchetta, Renato
AU - Tiberti, Claudio
AU - Arnaldi, Giorgio
AU - Bini, Vittorio
AU - Beck-Peccoz, Paolo
AU - Bizzarro, Antonio
AU - Dotta, Francesco
AU - Mantero, Franco
AU - Bellastella, Antonio
AU - Betterle, Corrado
AU - Santeusanio, Fausto
PY - 2004/4
Y1 - 2004/4
N2 - Primary adrenal insufficiency (PAI) is clinically evident in one in 8000 individuals. A correct etiological classification is critical for correct disease management. To update the diagnostic criteria for the etiological classification of PAI, a multicentric network was established in Italy, and 222 patients with PAI were studied. Both 21-hydroxylase and adrenal cortex autoantibodies (21OHAb and ACA, respectively) were tested in two independent laboratories on coded samples and found in 65-66% and 58-61% of cases, respectively. Autoimmune polyendocrine syndrome I was diagnosed in 11 of the 222 patients. Of the remaining 211 patients, 38 (18%) had a nonautoimmune form of PAI. In 145 subjects (65%), the presence of adrenal autoantibodies, without signs of other forms of PAI, led to a diagnosis of autoimmune Addison's disease. In six cases (3%), PAI remained idiopathic. Logistic regression analysis showed a 92.2-92.7% probability of correct reclassification for the two 21OHAb assays and 84.5-85.9% for the ACA assays. We conclude that the simultaneous presence of both 21OHAb and ACA permits unambiguous diagnosis of autoimmune Addison's, whereas subjects with low antibody titers should undergo both instrumental and biochemical tests to exclude other causes of PAI. Lastly, we developed a comprehensive flowchart for the classification of PAI for use in routine clinical practice.
AB - Primary adrenal insufficiency (PAI) is clinically evident in one in 8000 individuals. A correct etiological classification is critical for correct disease management. To update the diagnostic criteria for the etiological classification of PAI, a multicentric network was established in Italy, and 222 patients with PAI were studied. Both 21-hydroxylase and adrenal cortex autoantibodies (21OHAb and ACA, respectively) were tested in two independent laboratories on coded samples and found in 65-66% and 58-61% of cases, respectively. Autoimmune polyendocrine syndrome I was diagnosed in 11 of the 222 patients. Of the remaining 211 patients, 38 (18%) had a nonautoimmune form of PAI. In 145 subjects (65%), the presence of adrenal autoantibodies, without signs of other forms of PAI, led to a diagnosis of autoimmune Addison's disease. In six cases (3%), PAI remained idiopathic. Logistic regression analysis showed a 92.2-92.7% probability of correct reclassification for the two 21OHAb assays and 84.5-85.9% for the ACA assays. We conclude that the simultaneous presence of both 21OHAb and ACA permits unambiguous diagnosis of autoimmune Addison's, whereas subjects with low antibody titers should undergo both instrumental and biochemical tests to exclude other causes of PAI. Lastly, we developed a comprehensive flowchart for the classification of PAI for use in routine clinical practice.
UR - http://www.scopus.com/inward/record.url?scp=11144354944&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=11144354944&partnerID=8YFLogxK
U2 - 10.1210/jc.2003-030954
DO - 10.1210/jc.2003-030954
M3 - Article
C2 - 15070918
AN - SCOPUS:11144354944
VL - 89
SP - 1598
EP - 1604
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
SN - 0021-972X
IS - 4
ER -