Evaluation of the diagnostic criteria for Cushing’s disease in Japan [Review]

Adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome is caused by an ACTH-producing tumor, as is the case with Cushing’s disease and ectopic ACTH syndrome (EAS). Diagnosis and differential diagnosis of Cushing’s disease from EAS in ACTH-dependent Cushing’s syndrome are thus challenging pr...

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Bibliographic Details
Published in:ENDOCRINE JOURNAL 2013, Vol.60(2), pp.127-135
Main Authors: Kageyama, Kazunori, Oki, Yutaka, Sakihara, Satoru, Nigawara, Takeshi, Terui, Ken, Suda, Toshihiro
Format: Article
Language:eng ; jpn
Subjects:
ACTH
ACTH Syndrome, Ectopic - diagnosis
ACTH Syndrome, Ectopic - ethnology
ACTH Syndrome, Ectopic - etiology
ACTH-Secreting Pituitary Adenoma - diagnosis
ACTH-Secreting Pituitary Adenoma - ethnology
ACTH-Secreting Pituitary Adenoma - physiopathology
Cortisol
Cushing Syndrome - diagnosis
Cushing Syndrome - ethnology
Cushing Syndrome - etiology
Cushing’s disease
Diagnosis, Differential
Ectopic ACTH syndrome
Glucocorticoids
Humans
Japan
Pituitary
Pituitary ACTH Hypersecretion - diagnosis
Pituitary ACTH Hypersecretion - ethnology
Pituitary ACTH Hypersecretion - etiology
Pituitary ACTH Hypersecretion - physiopathology
Pituitary Function Tests
Pituitary Gland - drug effects
Pituitary Gland - metabolism
Practice Guidelines as Topic
Severity of Illness Index
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Summary:Adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome is caused by an ACTH-producing tumor, as is the case with Cushing’s disease and ectopic ACTH syndrome (EAS). Diagnosis and differential diagnosis of Cushing’s disease from EAS in ACTH-dependent Cushing’s syndrome are thus challenging problems in clinical endocrinology. The diagnostic criteria for Cushing’s disease in Japan, established by the working group of the Japan Ministry of Health, Labour and Welfare, were originally reported in 2003 and revised in 2007 and 2010. In addition, criteria for subclinical Cushing’s disease were established in Japan in 2010. In this review, we evaluate the usefulness and accuracy of the most recent diagnostic criteria. Previous data suggest that as an initial test of Cushing’s syndrome, 0.5 mg dexamethasone is more sensitive than 1 mg in the overnight dexamethasone suppression test (DST). Here, we recommend 0.5 mg plus a plasma cortisol cut-off level of 3 μg/dL as a suitable low-dose overnight DST for screening of all cases of ACTH-dependent Cushing’s syndrome in Japan. Recently, standardization of cortisol measurements by the ID-LC/MS/MS method using seven assay kits with standard plasma material containing synthetic hydrocortisone-d4 was carried out in Japan. The resulting relative standard deviation was within 10%. The cut-off value remains valid even after standardization of plasma cortisol measurements. Although the recent diagnostic criteria achieve higher diagnostic specificity, care should be taken since data for Cushing’s disease partially overlaps with some cases of EAS. Overall, therefore, this review suggests that the accuracy of each diagnostic test should be considered.
ISSN:0918-8959
1348-4540
DOI:10.1507/endocrj.EJ12-0299