{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T12:18:27Z","timestamp":1793794707144,"version":"4.6.1"},"reference-count":115,"publisher":"American Medical Association (AMA)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA"],"published-print":{"date-parts":[[2023,7,11]]},"abstract":"<jats:sec id=\"ab-jrv230013-1\">\n                    <jats:title>Importance<\/jats:title>\n                    <jats:p>Cushing syndrome is defined as a prolonged increase in plasma cortisol levels that is not due to a physiological etiology. Although the most frequent cause of Cushing syndrome is exogenous steroid use, the estimated incidence of Cushing syndrome due to endogenous overproduction of cortisol ranges from 2 to 8 per million people annually. Cushing syndrome is associated with hyperglycemia, protein catabolism, immunosuppression, hypertension, weight gain, neurocognitive changes, and mood disorders.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-jrv230013-2\">\n                    <jats:title>Observations<\/jats:title>\n                    <jats:p>Cushing syndrome characteristically presents with skin changes such as facial plethora, easy bruising, and purple striae and with metabolic manifestations such as hyperglycemia, hypertension, and excess fat deposition in the face, back of the neck, and visceral organs. Cushing disease, in which corticotropin excess is produced by a benign pituitary tumor, occurs in approximately 60% to 70% of patients with Cushing syndrome due to endogenous cortisol production. Evaluation of patients with possible Cushing syndrome begins with ruling out exogenous steroid use. Screening for elevated cortisol is performed with a 24-hour urinary free cortisol test or late-night salivary cortisol test or by evaluating whether cortisol is suppressed the morning after an evening dexamethasone dose. Plasma corticotropin levels can help distinguish between adrenal causes of hypercortisolism (suppressed corticotropin) and corticotropin-dependent forms of hypercortisolism (midnormal to elevated corticotropin levels). Pituitary magnetic resonance imaging, bilateral inferior petrosal sinus sampling, and adrenal or whole-body imaging can help identify tumor sources of hypercortisolism. Management of Cushing syndrome begins with surgery to remove the source of excess endogenous cortisol production followed by medication that includes adrenal steroidogenesis inhibitors, pituitary-targeted drugs, or glucocorticoid receptor blockers. For patients not responsive to surgery and medication, radiation therapy and bilateral adrenalectomy may be appropriate.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-jrv230013-3\">\n                    <jats:title>Conclusions and Relevance<\/jats:title>\n                    <jats:p>The incidence of Cushing syndrome due to endogenous overproduction of cortisol is 2 to 8 people per million annually. First-line therapy for Cushing syndrome due to endogenous overproduction of cortisol is surgery to remove the causative tumor. Many patients will require additional treatment with medications, radiation, or bilateral adrenalectomy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1001\/jama.2023.11305","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T11:01:08Z","timestamp":1689073268000},"page":"170","source":"Crossref","is-referenced-by-count":271,"title":["Cushing Syndrome"],"prefix":"10.1001","volume":"330","author":[{"given":"Martin","family":"Reincke","sequence":"first","affiliation":[{"name":"Medizinische Klinik und Poliklinik IV, Klinikum der Universit\u00e4t, Ludwig-Maximilians-Universit\u00e4t, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Fleseriu","sequence":"additional","affiliation":[{"name":"Departments of Medicine and Neurological Surgery and Pituitary Center, Oregon Health & Science University, Portland, Oregon"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10","reference":[{"issue":"3","key":"jrv230013r1","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1530\/EJE-19-0305","article-title":"Fifteen-year nationwide trends in systemic glucocorticoid drug use in Denmark.","volume":"181","author":"Laugesen","year":"2019","journal-title":"Eur J Endocrinol"},{"issue":"5","key":"jrv230013r2","doi-asserted-by":"publisher","first-page":"1526","DOI":"10.1210\/jc.2008-0125","article-title":"The diagnosis of Cushing\u2019s syndrome: an Endocrine Society clinical practice guideline.","volume":"93","author":"Nieman","year":"2008","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"jrv230013r3","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1007\/s11102-014-0569-6","article-title":"Incidence of Cushing\u2019s syndrome and Cushing\u2019s disease in commercially-insured patients &lt;65 years old in the United States.","volume":"18","author":"Broder","year":"2015","journal-title":"Pituitary"},{"issue":"16","key":"jrv230013r4","doi-asserted-by":"publisher","first-page":"1386","DOI":"10.1001\/jama.2023.5444","article-title":"Diagnosis and management of pituitary adenomas: a review.","volume":"329","author":"Tritos","year":"2023","journal-title":"JAMA"},{"issue":"3","key":"jrv230013r5","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1530\/EJE-11-0272","article-title":"The European Registry on Cushing\u2019s syndrome: 2-year experience. baseline demographic and clinical characteristics.","volume":"165","author":"Valassi","year":"2011","journal-title":"Eur J Endocrinol"},{"issue":"8","key":"jrv230013r6","doi-asserted-by":"publisher","DOI":"10.1111\/jne.v34.8","article-title":"Long-term effects of glucocorticoid excess on the brain.","volume":"34","author":"Dekkers","year":"2022","journal-title":"J Neuroendocrinol"},{"key":"jrv230013r7","doi-asserted-by":"publisher","first-page":"766","DOI":"10.3389\/fendo.2019.00766","article-title":"Toward a diagnostic score in Cushing\u2019s syndrome.","volume":"10","author":"Braun","year":"2019","journal-title":"Front Endocrinol (Lausanne)"},{"issue":"12","key":"jrv230013r8","doi-asserted-by":"publisher","first-page":"847","DOI":"10.1016\/S2213-8587(21)00235-7","article-title":"Consensus on diagnosis and management of Cushing\u2019s disease: a guideline update.","volume":"9","author":"Fleseriu","year":"2021","journal-title":"Lancet Diabetes Endocrinol"},{"key":"jrv230013r9","doi-asserted-by":"publisher","first-page":"740","DOI":"10.3389\/fendo.2019.00740","article-title":"Hypercortisolemia recurrence in Cushing\u2019s disease; a diagnostic challenge.","volume":"10","author":"Hinojosa-Amaya","year":"2019","journal-title":"Front Endocrinol (Lausanne)"},{"issue":"9","key":"jrv230013r10","doi-asserted-by":"publisher","first-page":"e3723","DOI":"10.1210\/clinem\/dgac379","article-title":"Whom should we screen for Cushing syndrome? the Endocrine Society Practice guideline recommendations 2008 revisited.","volume":"107","author":"Braun","year":"2022","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"jrv230013r11","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1210\/jc.86.1.117","article-title":"Incidence and late prognosis of Cushing\u2019s syndrome: a population-based study.","volume":"86","author":"Lindholm","year":"2001","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"jrv230013r12","doi-asserted-by":"publisher","first-page":"688","DOI":"10.3803\/EnM.2021.1000","article-title":"Descriptive epidemiology and survival analysis of prolactinomas and Cushing\u2019s disease in Korea.","volume":"36","author":"Park","year":"2021","journal-title":"Endocrinol Metab (Seoul)"},{"issue":"2","key":"jrv230013r13","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1007\/s11102-019-00951-1","article-title":"The incidence of Cushing\u2019s disease: a nationwide Swedish study.","volume":"22","author":"Ragnarsson","year":"2019","journal-title":"Pituitary"},{"issue":"6","key":"jrv230013r14","doi-asserted-by":"publisher","first-page":"2375","DOI":"10.1210\/jc.2018-02524","article-title":"Overall and disease-specific mortality in patients with Cushing disease: a Swedish nationwide study.","volume":"104","author":"Ragnarsson","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"jrv230013r15","doi-asserted-by":"publisher","first-page":"436","DOI":"10.1111\/cen.2011.75.issue-4","article-title":"Mortality and morbidity in Cushing\u2019s syndrome in New Zealand.","volume":"75","author":"Bolland","year":"2011","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"8","key":"jrv230013r16","doi-asserted-by":"publisher","DOI":"10.1111\/jne.v34.8","article-title":"Clinical presentation and etiology of Cushing\u2019s syndrome: Data from ERCUSYN.","volume":"34","author":"Valassi","year":"2022","journal-title":"J Neuroendocrinol"},{"issue":"3","key":"jrv230013r17","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgz136","article-title":"Time to diagnosis in Cushing\u2019s syndrome: a meta-analysis based on 5367 patients.","volume":"105","author":"Rubinstein","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"key":"jrv230013r18","doi-asserted-by":"publisher","DOI":"10.1155\/2019\/7159696","article-title":"Demographic characteristics, etiology, and comorbidities of patients with Cushing's syndrome: a 10-year retrospective study at a large general hospital in China.","author":"Zhou","year":"2019","journal-title":"Int J Endocrinol"},{"issue":"10","key":"jrv230013r19","doi-asserted-by":"publisher","first-page":"937","DOI":"10.1056\/NEJMra1810772","article-title":"Pituitary-tumor endocrinopathies.","volume":"382","author":"Melmed","year":"2020","journal-title":"N Engl J Med"},{"issue":"2","key":"jrv230013r20","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1016\/j.ecl.2018.02.007","article-title":"Genetics of Cushing\u2019s syndrome.","volume":"47","author":"Hern\u00e1ndez-Ram\u00edrez","year":"2018","journal-title":"Endocrinol Metab Clin North Am"},{"issue":"1","key":"jrv230013r21","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1038\/ng.3166","article-title":"Mutations in the deubiquitinase gene USP8 cause Cushing\u2019s disease.","volume":"47","author":"Reincke","year":"2015","journal-title":"Nat Genet"},{"issue":"9996","key":"jrv230013r22","doi-asserted-by":"publisher","first-page":"913","DOI":"10.1016\/S0140-6736(14)61375-1","article-title":"Cushing\u2019s syndrome.","volume":"386","author":"Lacroix","year":"2015","journal-title":"Lancet"},{"issue":"6","key":"jrv230013r23","doi-asserted-by":"publisher","first-page":"516","DOI":"10.1210\/er.2017-00189","article-title":"Genetic causes of functional adrenocortical adenomas.","volume":"38","author":"Zennaro","year":"2017","journal-title":"Endocr Rev"},{"issue":"1","key":"jrv230013r24","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1530\/EJE-21-1032","article-title":"Identification of predictive criteria for pathogenic variants of primary bilateral macronodular adrenal hyperplasia (PBMAH) gene ARMC5 in 352 unselected patients.","volume":"187","author":"Bouys","year":"2022","journal-title":"Eur J Endocrinol"},{"issue":"6","key":"jrv230013r25","doi-asserted-by":"publisher","first-page":"1003","DOI":"10.1210\/endrev\/bnac010","article-title":"Clinical biology of the pituitary adenoma.","volume":"43","author":"Melmed","year":"2022","journal-title":"Endocr Rev"},{"issue":"3","key":"jrv230013r26","doi-asserted-by":"publisher","first-page":"531","DOI":"10.3171\/2008.8.JNS08339","article-title":"Outcome of using the histological pseudocapsule as a surgical capsule in Cushing disease.","volume":"111","author":"Jagannathan","year":"2009","journal-title":"J Neurosurg"},{"issue":"2","key":"jrv230013r27","doi-asserted-by":"publisher","first-page":"404","DOI":"10.3171\/2016.1.JNS152119","article-title":"Cushing\u2019s disease: pathobiology, diagnosis, and management.","volume":"126","author":"Lonser","year":"2017","journal-title":"J Neurosurg"},{"issue":"9","key":"jrv230013r28","doi-asserted-by":"publisher","first-page":"4101","DOI":"10.1210\/jc.2019-00333","article-title":"Why we should still treat by neurosurgery patients with Cushing disease and a normal or inconclusive pituitary MRI.","volume":"104","author":"Cristante","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"jrv230013r29","doi-asserted-by":"publisher","first-page":"3171","DOI":"10.1038\/s41467-018-05275-5","article-title":"Identification of recurrent USP48 and BRAF mutations in Cushing\u2019s disease.","volume":"9","author":"Chen","year":"2018","journal-title":"Nat Commun"},{"issue":"4","key":"jrv230013r30","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1007\/s11102-019-00973-9","article-title":"Cushing\u2019s disease due to somatic USP8 mutations: a systematic review and meta-analysis.","volume":"22","author":"Wanichi","year":"2019","journal-title":"Pituitary"},{"issue":"4","key":"jrv230013r31","doi-asserted-by":"publisher","first-page":"453","DOI":"10.1530\/EJE-16-0809","article-title":"Prognostic factors in ectopic Cushing\u2019s syndrome due to neuroendocrine tumors: a multicenter study.","volume":"176","author":"Davi\u2019","year":"2017","journal-title":"Eur J Endocrinol"},{"issue":"11","key":"jrv230013r32","doi-asserted-by":"publisher","first-page":"1019","DOI":"10.1056\/NEJMoa1310359","article-title":"Constitutive activation of PKA catalytic subunit in adrenal Cushing\u2019s syndrome.","volume":"370","author":"Beuschlein","year":"2014","journal-title":"N Engl J Med"},{"issue":"4","key":"jrv230013r33","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1002\/bjs.9743","article-title":"Diagnosis, treatment and outcome of adrenocortical cancer.","volume":"102","author":"Mihai","year":"2015","journal-title":"Br J Surg"},{"issue":"6","key":"jrv230013r34","doi-asserted-by":"publisher","first-page":"1051","DOI":"10.1210\/endrev\/bnac012","article-title":"Update on biology and genomics of adrenocortical carcinomas: rationale for emerging therapies.","volume":"43","author":"Lerario","year":"2022","journal-title":"Endocr Rev"},{"issue":"11","key":"jrv230013r35","doi-asserted-by":"publisher","first-page":"699","DOI":"10.1038\/s41574-022-00718-y","article-title":"Primary bilateral macronodular adrenal hyperplasia: definitely a genetic disease.","volume":"18","author":"Cavalcante","year":"2022","journal-title":"Nat Rev Endocrinol"},{"issue":"7","key":"jrv230013r36","doi-asserted-by":"publisher","first-page":"611","DOI":"10.1016\/S2213-8587(16)00086-3","article-title":"Complications of Cushing\u2019s syndrome: state of the art.","volume":"4","author":"Pivonello","year":"2016","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"2","key":"jrv230013r37","doi-asserted-by":"publisher","first-page":"514","DOI":"10.3390\/ijerph8020514","article-title":"Hospital malnutrition: prevalence, identification and impact on patients and the healthcare system.","volume":"8","author":"Barker","year":"2011","journal-title":"Int J Environ Res Public Health"},{"issue":"6","key":"jrv230013r38","doi-asserted-by":"publisher","first-page":"904","DOI":"10.1111\/add.12899","article-title":"Global statistics on addictive behaviours: 2014 status report.","volume":"110","author":"Gowing","year":"2015","journal-title":"Addiction"},{"issue":"4","key":"jrv230013r39","doi-asserted-by":"publisher","first-page":"351","DOI":"10.1002\/da.v27:4","article-title":"Age differences in the prevalence and co-morbidity of DSM-IV major depressive episodes: results from the WHO World Mental Health Survey Initiative.","volume":"27","author":"Kessler","year":"2010","journal-title":"Depress Anxiety"},{"issue":"4","key":"jrv230013r40","doi-asserted-by":"publisher","first-page":"M33","DOI":"10.1530\/EJE-15-0464","article-title":"Cushing\u2019s syndrome: update on signs, symptoms and biochemical screening.","volume":"173","author":"Nieman","year":"2015","journal-title":"Eur J Endocrinol"},{"issue":"1","key":"jrv230013r41","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1016\/j.diabres.2009.10.007","article-title":"Global estimates of the prevalence of diabetes for 2010 and 2030.","volume":"87","author":"Shaw","year":"2010","journal-title":"Diabetes Res Clin Pract"},{"issue":"1","key":"jrv230013r42","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1056\/NEJMoa1614362","article-title":"Health effects of overweight and obesity in 195 countries over 25 years.","volume":"377","author":"Afshin","year":"2017","journal-title":"N Engl J Med"},{"issue":"2","key":"jrv230013r43","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1111\/cen.2007.67.issue-2","article-title":"Screening of Cushing\u2019s syndrome in adult patients with newly diagnosed diabetes mellitus.","volume":"67","author":"Reimondo","year":"2007","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"3","key":"jrv230013r44","doi-asserted-by":"publisher","first-page":"564","DOI":"10.3803\/EnM.2021.1069","article-title":"Cushing syndrome associated myopathy: it is time for a change.","volume":"36","author":"Reincke","year":"2021","journal-title":"Endocrinol Metab (Seoul)"},{"issue":"5","key":"jrv230013r45","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1210\/endrev\/bnab046","article-title":"Molecular derangements and the diagnosis of ACTH-dependent Cushing\u2019s syndrome.","volume":"43","author":"Nieman","year":"2022","journal-title":"Endocr Rev"},{"issue":"2","key":"jrv230013r46","doi-asserted-by":"publisher","first-page":"201","DOI":"10.1007\/s11102-015-0634-9","article-title":"Screening for Cushing\u2019s syndrome: is it worthwhile?","volume":"18","author":"Shimon","year":"2015","journal-title":"Pituitary"},{"issue":"8","key":"jrv230013r47","doi-asserted-by":"publisher","first-page":"541","DOI":"10.7326\/0003-4819-147-8-200710160-00006","article-title":"Subclinical hypercortisolism among outpatients referred for osteoporosis.","volume":"147","author":"Chiodini","year":"2007","journal-title":"Ann Intern Med"},{"issue":"2","key":"jrv230013r48","doi-asserted-by":"publisher","first-page":"G1","DOI":"10.1530\/EJE-16-0467","article-title":"Management of adrenal incidentalomas: European Society of Endocrinology Clinical practice guideline in collaboration with the European Network for the Study of ADRENAL TUMORS.","volume":"175","author":"Fassnacht","year":"2016","journal-title":"Eur J Endocrinol"},{"issue":"9","key":"jrv230013r49","doi-asserted-by":"publisher","first-page":"4106","DOI":"10.1210\/jc.2010-0457","article-title":"Approach to the patient with an adrenal incidentaloma.","volume":"95","author":"Nieman","year":"2010","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"jrv230013r50","doi-asserted-by":"publisher","first-page":"343","DOI":"10.1093\/ejendo\/lvad038","article-title":"Mild autonomous cortisol secretion in adrenal incidentalomas and risk of fragility fractures: a large cross-sectional study.","volume":"188","author":"Zavatta","year":"2023","journal-title":"Eur J Endocrinol"},{"issue":"10","key":"jrv230013r51","doi-asserted-by":"publisher","first-page":"3747","DOI":"10.1210\/jc.2016-1673","article-title":"A probabilistic model for Cushing\u2019s syndrome screening in at-risk populations: a prospective multicenter study.","volume":"101","author":"Le\u00f3n-Justel","year":"2016","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"jrv230013r52","doi-asserted-by":"publisher","first-page":"R205","DOI":"10.1530\/EJE-16-0946","article-title":"Diagnosis of endocrine disease: differentiation of pathologic\/neoplastic hypercortisolism (Cushing\u2019s syndrome) from physiologic\/non-neoplastic hypercortisolism (formerly known as pseudo-Cushing\u2019s syndrome).","volume":"176","author":"Findling","year":"2017","journal-title":"Eur J Endocrinol"},{"issue":"5","key":"jrv230013r53","doi-asserted-by":"publisher","first-page":"1553","DOI":"10.1210\/jc.2008-0139","article-title":"Accuracy of diagnostic tests for Cushing\u2019s syndrome: a systematic review and metaanalyses.","volume":"93","author":"Elamin","year":"2008","journal-title":"J Clin Endocrinol Metab"},{"issue":"10","key":"jrv230013r54","doi-asserted-by":"publisher","DOI":"10.1210\/jendso\/bvaa107","article-title":"Prospective evaluation of late-night salivary cortisol and cortisone by EIA and LC-MS\/MS in suspected Cushing syndrome.","volume":"4","author":"Kannankeril","year":"2020","journal-title":"J Endocr Soc"},{"issue":"6","key":"jrv230013r55","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgaa105","article-title":"Accuracy of laboratory tests for the diagnosis of Cushing syndrome.","volume":"105","author":"Galm","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"jrv230013r56","doi-asserted-by":"publisher","first-page":"R29","DOI":"10.1530\/EJE-19-0877","article-title":"Management of endocrine disease: Cushing\u2019s syndrome due to ectopic ACTH secretion: an expert operational opinion.","volume":"182","author":"Young","year":"2020","journal-title":"Eur J Endocrinol"},{"issue":"3","key":"jrv230013r57","doi-asserted-by":"publisher","first-page":"e1221","DOI":"10.1210\/clinem\/dgab724","article-title":"Interpretation of abnormal dexamethasone suppression test is enhanced with use of synchronous free cortisol assessment.","volume":"107","author":"Genere","year":"2022","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"jrv230013r58","doi-asserted-by":"publisher","DOI":"10.1016\/j.beem.2021.101519","article-title":"Biochemical diagnosis of Cushing\u2019s disease: screening and confirmatory testing.","volume":"35","author":"Petersenn","year":"2021","journal-title":"Best Pract Res Clin Endocrinol Metab"},{"issue":"5","key":"jrv230013r59","doi-asserted-by":"publisher","first-page":"693","DOI":"10.1007\/s11102-022-01249-5","article-title":"Overnight 1 mg dexamethasone suppression test and 24 h urine free cortisol-accuracy and pitfalls when screening for Cushing\u2019s syndrome.","volume":"25","author":"Petersenn","year":"2022","journal-title":"Pituitary"},{"issue":"5","key":"jrv230013r60","doi-asserted-by":"publisher","first-page":"698","DOI":"10.1007\/s11102-022-01214-2","article-title":"Late night salivary cortisol in the diagnosis of neoplastic hypercortisolism (including cyclic Cushing\u2019s syndrome).","volume":"25","author":"Raff","year":"2022","journal-title":"Pituitary"},{"issue":"1","key":"jrv230013r61","doi-asserted-by":"publisher","first-page":"G1","DOI":"10.1530\/EJE-20-0352","article-title":"Endocrinology in the time of COVID-19: management of Cushing\u2019s syndrome.","volume":"183","author":"Newell-Price","year":"2020","journal-title":"Eur J Endocrinol"},{"issue":"1","key":"jrv230013r62","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1210\/jc.85.1.42","article-title":"Cortisolemic indices predict severe infections in Cushing syndrome due to ectopic production of adrenocorticotropin.","volume":"85","author":"Sarlis","year":"2000","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"jrv230013r63","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1111\/cen.2014.80.issue-2","article-title":"High variability in baseline urinary free cortisol values in patients with Cushing\u2019s disease.","volume":"80","author":"Petersenn","year":"2014","journal-title":"Clin Endocrinol (Oxf)"},{"issue":"17","key":"jrv230013r64","doi-asserted-by":"publisher","first-page":"2232","DOI":"10.1001\/jama.1993.03500170062035","article-title":"Corticotropin-releasing hormone stimulation following low-dose dexamethasone administration. a new test to distinguish Cushing\u2019s syndrome from pseudo-Cushing\u2019s states.","volume":"269","author":"Yanovski","year":"1993","journal-title":"JAMA"},{"issue":"6","key":"jrv230013r65","doi-asserted-by":"publisher","first-page":"1011","DOI":"10.1530\/EJE-09-0046","article-title":"The prevalence and characteristic features of cyclicity and variability in Cushing\u2019s disease.","volume":"160","author":"Alexandraki","year":"2009","journal-title":"Eur J Endocrinol"},{"issue":"11","key":"jrv230013r66","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgaa564","article-title":"Human corticotropin-releasing hormone tests: 10 years of real-life experience in pituitary and adrenal disease.","volume":"105","author":"Ceccato","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"jrv230013r67","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgz194","article-title":"The optimal cut-off of BIPSS in differential diagnosis of ACTH-dependent Cushing\u2019s syndrome: is stimulation necessary?","volume":"105","author":"Chen","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"jrv230013r68","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1530\/EJE-16-0967","article-title":"Diagnostic tests for Cushing\u2019s syndrome differ from published guidelines: data from ERCUSYN.","volume":"176","author":"Valassi","year":"2017","journal-title":"Eur J Endocrinol"},{"issue":"108","key":"jrv230013r69","doi-asserted-by":"publisher","first-page":"e178","DOI":"10.1210\/clinem\/dgac686","article-title":"Dynamic testing for differential diagnosis of ACTH-dependent Cushing syndrome: a systematic review and meta-analysis.","volume":"13","author":"Ceccato","year":"2022","journal-title":"J Clin Endocrinol Metab"},{"issue":"10","key":"jrv230013r70","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgaa409","article-title":"Non-invasive diagnostic strategy in ACTH-dependent Cushing\u2019s syndrome.","volume":"105","author":"Frete","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"jrv230013r71","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1227\/NEU.0b013e31824f8e2e","article-title":"Transsphenoidal surgery for cushing disease after nondiagnostic inferior petrosal sinus sampling.","volume":"71","author":"Sheth","year":"2012","journal-title":"Neurosurgery"},{"issue":"5","key":"jrv230013r72","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1007\/s11102-019-00972-w","article-title":"Diagnostic utility of Gallium-68-somatostatin receptor PET\/CT in ectopic ACTH-secreting tumors: a systematic literature review and single-center clinical experience.","volume":"22","author":"Varlamov","year":"2019","journal-title":"Pituitary"},{"issue":"3","key":"jrv230013r73","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1097\/MED.0000000000000061","article-title":"Primary bilateral macronodular adrenal hyperplasia.","volume":"21","author":"De Venanzi","year":"2014","journal-title":"Curr Opin Endocrinol Diabetes Obes"},{"issue":"8","key":"jrv230013r74","doi-asserted-by":"publisher","first-page":"2807","DOI":"10.1210\/jc.2015-1818","article-title":"Treatment of Cushing\u2019s syndrome: an Endocrine Society clinical practice guideline.","volume":"100","author":"Nieman","year":"2015","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"jrv230013r75","doi-asserted-by":"publisher","first-page":"551","DOI":"10.1007\/s11102-022-01239-7","article-title":"Surgical strategies in the treatment of MR-negative Cushing\u2019s Disease: a systematic review and treatment algorithm.","volume":"25","author":"Yang","year":"2022","journal-title":"Pituitary"},{"issue":"6","key":"jrv230013r76","doi-asserted-by":"publisher","first-page":"R227","DOI":"10.1530\/EJE-14-0883","article-title":"Therapy of endocrine disease: outcomes in patients with Cushing\u2019s disease undergoing transsphenoidal surgery: systematic review assessing criteria used to define remission and recurrence.","volume":"172","author":"Petersenn","year":"2015","journal-title":"Eur J Endocrinol"},{"issue":"5","key":"jrv230013r77","doi-asserted-by":"publisher","first-page":"524","DOI":"10.1007\/s11102-018-0893-3","article-title":"Endoscopic vs microscopic transsphenoidal surgery for Cushing\u2019s disease: a systematic review and meta-analysis.","volume":"21","author":"Broersen","year":"2018","journal-title":"Pituitary"},{"issue":"3","key":"jrv230013r78","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1007\/s11102-022-01218-y","article-title":"Glucocorticoid withdrawal syndrome following treatment of endogenous Cushing syndrome.","volume":"25","author":"He","year":"2022","journal-title":"Pituitary"},{"issue":"4","key":"jrv230013r79","doi-asserted-by":"publisher","first-page":"523","DOI":"10.1210\/er.2001-0014","article-title":"Endocrine withdrawal syndromes.","volume":"24","author":"Hochberg","year":"2003","journal-title":"Endocr Rev"},{"issue":"2","key":"jrv230013r80","doi-asserted-by":"publisher","DOI":"10.1016\/j.beem.2021.101487","article-title":"Medical therapy in severe hypercortisolism.","volume":"35","author":"Marques","year":"2021","journal-title":"Best Pract Res Clin Endocrinol Metab"},{"issue":"2","key":"jrv230013r81","doi-asserted-by":"publisher","first-page":"522","DOI":"10.1007\/s00464-007-9555-7","article-title":"Perioperative results of 214 laparoscopic adrenalectomies by anterior transperitoneal approach.","volume":"22","author":"Lezoche","year":"2008","journal-title":"Surg Endosc"},{"issue":"3","key":"jrv230013r82","doi-asserted-by":"publisher","first-page":"341","DOI":"10.1007\/s00423-015-1287-x","article-title":"Laparoscopic adrenalectomy\u201410-year experience at a teaching hospital.","volume":"400","author":"Sommerey","year":"2015","journal-title":"Langenbecks Arch Surg"},{"issue":"3","key":"jrv230013r83","doi-asserted-by":"publisher","first-page":"216","DOI":"10.1016\/S2213-8587(14)70142-1","article-title":"Diagnosis and management of adrenal insufficiency.","volume":"3","author":"Bancos","year":"2015","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"4","key":"jrv230013r84","doi-asserted-by":"publisher","first-page":"G1","DOI":"10.1530\/EJE-18-0608","article-title":"European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors.","volume":"179","author":"Fassnacht","year":"2018","journal-title":"Eur J Endocrinol"},{"issue":"12","key":"jrv230013r85","doi-asserted-by":"publisher","first-page":"4417","DOI":"10.1210\/jc.2015-2662","article-title":"Unilateral adrenalectomy as a first-line treatment of Cushing\u2019s syndrome in patients with primary bilateral macronodular adrenal hyperplasia.","volume":"100","author":"Debillon","year":"2015","journal-title":"J Clin Endocrinol Metab"},{"issue":"7","key":"jrv230013r86","doi-asserted-by":"publisher","first-page":"2985","DOI":"10.1210\/jc.2018-02204","article-title":"Long-term outcome of primary bilateral macronodular adrenocortical hyperplasia after unilateral adrenalectomy.","volume":"104","author":"Osswald","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"jrv230013r87","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1097\/PAS.0000000000000748","article-title":"Cushing syndrome in Carney complex: clinical, pathologic, and molecular genetic findings in the 17 affected mayo clinic patients.","volume":"41","author":"Lowe","year":"2017","journal-title":"Am J Surg Pathol"},{"issue":"7","key":"jrv230013r88","doi-asserted-by":"publisher","first-page":"2114","DOI":"10.1210\/clinem\/dgab122","article-title":"Approach to the patient treated with steroidogenesis inhibitors.","volume":"106","author":"Castinetti","year":"2021","journal-title":"J Clin Endocrinol Metab"},{"issue":"7","key":"jrv230013r89","doi-asserted-by":"publisher","first-page":"e2882","DOI":"10.1210\/clinem\/dgac178","article-title":"Randomized trial of osilodrostat for the treatment of Cushing disease.","volume":"107","author":"Gadelha","year":"2022","journal-title":"J Clin Endocrinol Metab"},{"issue":"11","key":"jrv230013r90","doi-asserted-by":"publisher","first-page":"855","DOI":"10.1016\/S2213-8587(19)30313-4","article-title":"Efficacy and safety of levoketoconazole in the treatment of endogenous Cushing\u2019s syndrome (SONICS): a phase 3, multicentre, open-label, single-arm trial.","volume":"7","author":"Fleseriu","year":"2019","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"6","key":"jrv230013r91","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1007\/s11102-022-01263-7","article-title":"Levoketoconazole in the treatment of patients with endogenous Cushing\u2019s syndrome: a double-blind, placebo-controlled, randomized withdrawal study (LOGICS).","volume":"25","author":"Pivonello","year":"2022","journal-title":"Pituitary"},{"issue":"9","key":"jrv230013r92","doi-asserted-by":"publisher","first-page":"748","DOI":"10.1016\/S2213-8587(20)30240-0","article-title":"Efficacy and safety of osilodrostat in patients with Cushing\u2019s disease (LINC 3): a multicentre phase III study with a double-blind, randomised withdrawal phase.","volume":"8","author":"Pivonello","year":"2020","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"4","key":"jrv230013r93","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1530\/EJE-22-0317","article-title":"Long-term outcomes of osilodrostat in Cushing\u2019s disease: LINC 3 study extension.","volume":"187","author":"Fleseriu","year":"2022","journal-title":"Eur J Endocrinol"},{"issue":"6","key":"jrv230013r94","doi-asserted-by":"publisher","first-page":"859","DOI":"10.1530\/EJE-22-0506","article-title":"Levoketoconazole treatment in endogenous Cushing\u2019s syndrome: extended evaluation of clinical, biochemical, and radiologic outcomes.","volume":"187","author":"Fleseriu","year":"2022","journal-title":"Eur J Endocrinol"},{"issue":"10","key":"jrv230013r95","doi-asserted-by":"publisher","first-page":"914","DOI":"10.1056\/NEJMoa1105743","article-title":"A 12-month phase 3 study of pasireotide in Cushing\u2019s disease.","volume":"366","author":"Colao","year":"2012","journal-title":"N Engl J Med"},{"issue":"1","key":"jrv230013r96","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/S2213-8587(17)30326-1","article-title":"Efficacy and safety of once-monthly pasireotide in Cushing\u2019s disease: a 12 month clinical trial.","volume":"6","author":"Lacroix","year":"2018","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"4","key":"jrv230013r97","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1210\/er.2013-1048","article-title":"The treatment of Cushing\u2019s disease.","volume":"36","author":"Pivonello","year":"2015","journal-title":"Endocr Rev"},{"issue":"12","key":"jrv230013r98","doi-asserted-by":"publisher","first-page":"1445","DOI":"10.1007\/s40618-018-0936-7","article-title":"Effect of cabergoline monotherapy in Cushing\u2019s disease: an individual participant data meta-analysis.","volume":"41","author":"Palui","year":"2018","journal-title":"J Endocrinol Invest"},{"key":"jrv230013r99","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2022.935759","article-title":"Pasireotide-induced shrinkage in GH and ACTH secreting pituitary adenoma: a systematic review and meta-analysis.","volume":"13","author":"Mondin","year":"2022","journal-title":"Front Endocrinol (Lausanne)"},{"issue":"6","key":"jrv230013r100","doi-asserted-by":"publisher","first-page":"2039","DOI":"10.1210\/jc.2011-3350","article-title":"Mifepristone, a glucocorticoid receptor antagonist, produces clinical and metabolic benefits in patients with Cushing\u2019s syndrome.","volume":"97","author":"Fleseriu","year":"2012","journal-title":"J Clin Endocrinol Metab"},{"issue":"3","key":"jrv230013r101","doi-asserted-by":"publisher","first-page":"801","DOI":"10.3171\/2021.2.JNS204440","article-title":"Stereotactic radiosurgery for secretory pituitary adenomas: systematic review and International Stereotactic Radiosurgery Society practice recommendations.","volume":"136","author":"Mathieu","year":"2021","journal-title":"J Neurosurg"},{"issue":"1","key":"jrv230013r102","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1097\/RLU.0000000000000971","article-title":"Ectopic Corticotropin-producing neuroendocrine tumor of the pancreas treated with 177Lu dotatate induction and maintenance peptide receptor radionuclide therapy.","volume":"41","author":"Makis","year":"2016","journal-title":"Clin Nucl Med"},{"issue":"9","key":"jrv230013r103","doi-asserted-by":"publisher","first-page":"3743","DOI":"10.1210\/jc.2019-00029","article-title":"Adjuvant radiation improves recurrence-free survival and overall survival in adrenocortical carcinoma.","volume":"104","author":"Gharzai","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"issue":"4","key":"jrv230013r104","doi-asserted-by":"publisher","first-page":"1300","DOI":"10.1210\/jc.2014-3632","article-title":"Time to recovery of adrenal function after curative surgery for Cushing\u2019s syndrome depends on etiology.","volume":"100","author":"Berr","year":"2015","journal-title":"J Clin Endocrinol Metab"},{"issue":"2","key":"jrv230013r105","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1007\/s12020-020-02432-z","article-title":"Recurrence after pituitary surgery in adult Cushing's disease: a systematic review on diagnosis and treatment.","volume":"70","author":"Braun","year":"2020","journal-title":"Endocrine"},{"issue":"1","key":"jrv230013r106","doi-asserted-by":"publisher","first-page":"178","DOI":"10.1186\/s13023-022-02320-x","article-title":"Current clinical practice for thromboprophylaxis management in patients with Cushing\u2019s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN).","volume":"17","author":"van Haalen","year":"2022","journal-title":"Orphanet J Rare Dis"},{"issue":"5","key":"jrv230013r107","doi-asserted-by":"publisher","first-page":"R207","DOI":"10.1530\/EJE-20-1309","article-title":"Management of endocrine disease: cardiovascular risk assessment, thromboembolism, and infection prevention in Cushing\u2019s syndrome: a practical approach.","volume":"184","author":"Varlamov","year":"2021","journal-title":"Eur J Endocrinol"},{"issue":"4","key":"jrv230013r108","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1210\/er.2018-00005","article-title":"Pituitary diseases and bone.","volume":"39","author":"Mazziotti","year":"2018","journal-title":"Endocr Rev"},{"issue":"6","key":"jrv230013r109","doi-asserted-by":"publisher","first-page":"813","DOI":"10.1530\/EJE-20-1285","article-title":"Patients with low IGF-I after curative surgery for Cushing\u2019s syndrome have an adverse long-term outcome of hypercortisolism-induced myopathy.","volume":"184","author":"Vogel","year":"2021","journal-title":"Eur J Endocrinol"},{"issue":"3","key":"jrv230013r110","doi-asserted-by":"publisher","first-page":"e1316","DOI":"10.1210\/clinem\/dgaa858","article-title":"The hypothalamic-pituitary-thyroid axis in Cushing syndrome before and after curative surgery.","volume":"106","author":"Shekhar","year":"2021","journal-title":"J Clin Endocrinol Metab"},{"issue":"8","key":"jrv230013r111","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgaa291","article-title":"Excess morbidity persists in patients with Cushing\u2019s disease during long-term remission: a Swedish nationwide study.","volume":"105","author":"Papakokkinou","year":"2020","journal-title":"J Clin Endocrinol Metab"},{"issue":"5","key":"jrv230013r112","doi-asserted-by":"publisher","first-page":"461","DOI":"10.1530\/EJE-19-0464","article-title":"High mortality within 90 days of diagnosis in patients with Cushing\u2019s syndrome: results from the ERCUSYN registry.","volume":"181","author":"Valassi","year":"2019","journal-title":"Eur J Endocrinol"},{"issue":"7","key":"jrv230013r113","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1016\/S2213-8587(16)30005-5","article-title":"Mortality in patients with Cushing\u2019s disease more than 10 years after remission: a multicentre, multinational, retrospective cohort study.","volume":"4","author":"Clayton","year":"2016","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"6","key":"jrv230013r114","doi-asserted-by":"publisher","DOI":"10.1210\/jendso\/bvac045","article-title":"Increased mortality persists after treatment of Cushing\u2019s disease: a matched nationwide cohort study.","volume":"6","author":"Bengtsson","year":"2022","journal-title":"J Endocr Soc"},{"issue":"8","key":"jrv230013r115","doi-asserted-by":"publisher","first-page":"2377","DOI":"10.1210\/clinem\/dgac265","article-title":"The effect of endogenous Cushing syndrome on all-cause and cause-specific mortality.","volume":"107","author":"Limumpornpetch","year":"2022","journal-title":"J Clin Endocrinol Metab"}],"container-title":["JAMA"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/jamanetwork.com\/journals\/jama\/articlepdf\/2807073\/jama_reincke_2023_rv_230013_1688654398.89936.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T11:01:12Z","timestamp":1689073272000},"score":1,"resource":{"primary":{"URL":"https:\/\/jamanetwork.com\/journals\/jama\/fullarticle\/2807073"}},"subtitle":["A Review"],"short-title":[],"issued":{"date-parts":[[2023,7,11]]},"references-count":115,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2023,7,11]]}},"URL":"https:\/\/doi.org\/10.1001\/jama.2023.11305","relation":{},"ISSN":["0098-7484"],"issn-type":[{"value":"0098-7484","type":"print"}],"subject":[],"published":{"date-parts":[[2023,7,11]]}}}