The Cushing Syndrome: Quest for the Holy Grail
Authors: David N. Orth
Journal: Annals of Internal Medicine (1994)

Editorials1 September 1994The Cushing Syndrome: Quest for the Holy GrailDavid N. Orth, MDDavid N. Orth, MDVanderbilt University Medical Center; Nashville, TN 37232-2250Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-121-5-199409010-00012 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail The endogenous Cushing syndrome results from excessive cortisol secretion that is usually secondary to excessive adrenocorticotropin (ACTH) secretion. About 85% of patients with the ACTH-dependent Cushing syndrome have ACTH-secreting pituitary microadenomas (Cushing disease). Most of the remaining patients have ACTH-secreting nonpituitary tumors (the ectopic ACTH syndrome), usually small-cell lung carcinomas, and follow an acute clinical course different from that of patients with Cushing disease [1]. However, about one fifth of patients with the ectopic ACTH syndrome have indolent tumors (usually bronchial carcinoids) and may present with a clinical picture indistinguishable from that of Cushing disease. Further, the classic test for ...References1. Liddle GW, Nicholson WE, Island DP, Orth DN, Abe K, Lowder SC. Clinical and laboratory studies of ectopic humoral syndromes. Recent Prog Horm Res. 1969; 25:283-314. Google Scholar2. Liddle GW. Tests of pituitary-adrenal suppressibility in the diagnosis of Cushing's syndrome. J Clin Endocrinol Metab. 1960; 20:1539-60. Google Scholar3. Strott CA, Nugent CA, Tyler FH. Cushing's syndrome caused by bronchial adenomas. Am J Med. 1968; 44:97-104. Google Scholar4. Mason AM, Ratcliffe JG, Buckle RM, Mason AS. ACTH secretion by bronchial carcinoid tumours. Clin Endocrinol (Oxf). 1972; 1:3-25. Google Scholar5. Nieman LK, Chrousos GP, Oldfield EH, Avgerinos PC, Cutler GB Jr, Loriaux DL. The ovine corticotropin-releasing hormone stimulation test and the dexamethasone suppression test in the differential diagnosis of Cushing's syndrome. Ann Intern Med. 1986; 105:862-7. Google Scholar6. Oldfield EH, Doppman JL, Nieman LK, Chrousos GP, Miller DL, Katz DA, et al. Petrosal sinus sampling with and without corticotropin-releasing hormone for the differential diagnosis of Cushing's syndrome. N Engl J Med. 1991; 325:897-905. Google Scholar7. Avgerinos PC, Yanovski JA, Oldfield EH, Nieman LK, Cutler GB Jr. A comparison of the metyrapone test and the dexamethasone suppression test for the differential diagnosis of adrenocorticotropin-dependent Cushing syndrome. Ann Intern Med. 1994; 121:318-327. Google Scholar8. Liddle GW, Estep HL, Kendall JW Jr, Williams WC Jr, Townes AW. Clinical application of a new test of pituitary reserve. J Clin Endocrinol Metab. 1959; 19:875-94. Google Scholar9. Orth DN. The old and the new in Cushing's syndrome (Editorial). N Engl J Med. 1984; 310:649-51. Google Scholar10. Orth DN. Differential diagnosis of Cushing's syndrome (Editorial). N Engl J Med. 1991; 325:957-9. Google Scholar11. Flack MR, Oldfield EH, Cutler GB Jr, Zweig MH, Malley JD, Chrousos GP, et al. Urine free cortisol in the high-dose dexamethasone suppression test for the differential diagnosis of the Cushing syndrome. Ann Intern Med. 1992; 116:211-7. Google Scholar12. Yanovski JA, Cutler GB Jr, Chrousos GP, Nieman LK. Corticotropin-releasing hormone stimulation following low-dose dexamethasone administration. A new test to distinguish Cushing's syndrome from pseudo-Cushing's states. JAMA. 1993; 269:2232-8. Google Scholar13. Orth DN, Kovacs WJ, DeBold CR. The adrenal cortex. In: Wilson JD, Foster DW, eds. Williams Textbook of Endocrinology. Philadelphia: W.B. Saunders; 1992:489-619. Google Scholar14. Miller J, Crapo L. The biochemical analysis of hypercortisolism. Endocrinologist. 1994; 4:7-16. Google Scholar15. Orth DN. Cushing's syndrome (Medical Progress). N Engl J Med. 1994; (In press). Google Scholar Author, Article, and Disclosure InformationAuthors: David N. Orth, MDAffiliations: Vanderbilt University Medical Center; Nashville, TN 37232-2250Grant Support: In part by NIH Research Grants DK46070 and RR00095. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoThe Metyrapone and Dexamethasone Suppression Tests for the Differential Diagnosis of the Adrenocorticotropin-Dependent Cushing Syndrome: A Comparison Peter C. Avgerinos , Jack A. Yanovski , Edward H. Oldfield , Lynnette K. Nieman , and Gordon B. Cutler Metrics Cited byEndocrine Hypertensive EmergenciesCushing’s syndrome: Why is diagnosis so difficult?Adrenal Metastases and Rare Adrenal TumorsDiscriminatory Value of the Low-Dose Dexamethasone Suppression Test in Establishing the Diagnosis and Differential Diagnosis of Cushing’s SyndromeActualización en el diagnóstico y diagnóstico diferencial del síndrome de CushingThe Diagnosis and Differential Diagnosis of Cushing’s Syndrome and Pseudo-Cushing’s StatesEffectiveness Versus Efficacy: The Limited Value in Clinical Practice of High Dose Dexamethasone Suppression Testing in the Differential Diagnosis of Adrenocorticotropin-Dependent Cushing’s SyndromeNEW DIAGNOSTIC TESTS FOR CUSHING'S SYNDROME: USES OF NALOXONE, VASOPRESSIN AND ALPRAZOLAM 1 September 1994Volume 121, Issue 5Page: 377-378KeywordsAdrenocorticotropic hormoneBlood plasmaCortisolCreatinineDatabasesDexamethasone suppression testExcretionHormonesSpecificityUrine ePublished: 15 August 2000 Issue Published: 1 September 1994 Copyright & PermissionsCopyright © 1994 by American College of Physicians. 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