Diagnostic criteria for adrenal overt Cushing syndrome: prepared jointly by the Japan Endocrine Society and the Health and Labour Sciences Research Grant (Research on Intractable Diseases) Research Group on Disorders of Adrenal Hormones

Abstract

Adrenal overt Cushing syndrome (CS) is a rare disease but presents with a high risk of serious, life-threatening complications. Although florid CS is less frequently reported now, the interval between symptom onset and diagnosis still remains as long as four years. In this study, we investigated the current status of patients presenting with adrenal overt CS in the 2000s using data from the “Study of Advancing Care and Pathogenesis of Intractable Adrenal Diseases in Japan (ACPA-J study).” Based on the findings, we have established the diagnostic criteria for adrenal overt CS through the deliberation with members of the Health and Labour Sciences Research Grant “Research group on Disorders of adrenal hormones” and the public comment process conducted by the Japan Endocrine Society. We expect these diagnostic criteria to facilitate the designation of adrenal overt CS as an intractable disease and enhance standards of care.