This Article Full Text Full Text (PDF) Submit a related Letter to the Editor Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Arnaldi, G. Articles by Boscaro, M. Search for Related Content PubMed PubMed Citation Articles by Arnaldi, G. Articles by Boscaro, M.

Diagnosis and Complications of Cushing’s Syndrome: A Consensus Statement

Division of Endocrinology (G.A., B.K., T.M., M.B.), Department of Internal Medicine, University of Ancona, 60100 Ancona, Italy; Division of Internal Medicine (A.A.), Department of Clinical and Biological Science, University of Torino, 10063 Torino, Italy; Regional Centre for Endocrinology and Diabetes (A.B.A.), Royal Victoria Hospital and Queen’s University, BT126BA Belfast, United Kingdom; Department of Endocrinology (X.B.), University Renè Descartes and Institut Cochin, Institut National de la Santé et de la Recherche Médicale U567 and Faculty Cochin, 75014 Paris, France; Department of Endocrinology (F.C.), San Luca Hospital, Italian Auxological Institute, Instituto di Ricovero e Cura a Carattere Scientifico, University of Milan, 20149 Milan, Italy; National Institute of Child Health and Human Development, National Institutes of Health (G.P.C., L.K.N.), Bethesda, Maryland 20892-1583; Department of Psychology (G.A.F.), University of Bologna, 40127 Bologna, Italy; Endocrine-Diabetes Center (J.W.F.), St. Luke’s Medical Center, Milwaukee, Wisconsin 53215; Division of Endocrinology, Diabetology, and Metabolism (R.C.G.), University Hospital, Centre Hospitalier Universitaire Vandois, CH-1011 Lausanne, Switzerland; Department of Endocrinology (A.B.G.), St. Bartholomew’s Hospital, London EC1A 7BE, United Kingdom; Department of Medicine (A.L.), Centre hospitalier de l’Université de Montréal, H2W1T7 Montréal, Canada; Division of Endocrinology (F.M.), Department of Medical and Surgical Sciences, University of Padova, 35128 Padova, Italy; Division of Clinical Sciences (J.N.-P.), Sheffield University, Northern General Hospital, 557AU Sheffield, United Kingdom; Department of Statistical Sciences and Department of Mental Health (N.S.), University of Padova, 35128 Padova, Italy; University of Virginia Health System (M.L.V.), Charlottesville, Virginia 22908-0746; and Department of Internal Medicine/Endocrine Section (A.G.), University of Brescia, 25125 Brescia, Italy

Address all correspondence and requests for reprints to: Dr. Giorgio Arnaldi, Clinica di Endocrinologia Azienda Ospedaliera Umberto I, Sede Torrette 60100 Ancona, Italia. E-mail: g.arnaldi{at}aoumbertoprimo.marche.it.

In October 2002, a workshop was held in Ancona, Italy, to reach a Consensus on the management of Cushing’s syndrome. The workshop was organized by the University of Ancona and sponsored by the Pituitary Society, the European Neuroendocrine Association, and the Italian Society of Endocrinology. Invited international participants included almost 50 leading endocrinologists with specific expertise in the management of Cushing’s syndrome. The consensus statement on diagnostic criteria and the diagnosis and treatment of complications of this syndrome reached at the workshop is hereby summarized.

Participants in the Consensus Workshop were: B. Ambrosi, A. Angeli, G. Arnaldi, E. Arvat, A. B. Atkinson, X. Bertagna, M. Boscaro, F. Casanueva, F. Cavagnini, G. P. Chrousos, R. N. Clayton, A. Colao, Y. de Keyzer, W. de Herder, G. Faglia, F. Fallo, G. A. Fava, J. W. Findling, R. C. Gaillard, M. Gasperi, A. Giustina, A. B. Grossman, B. Kola, A. Lacroix, A. Lania, R. Lauro, P. Loli, M. Losa, T. Mancini, M. Mannelli, F. Mantero, J. Newell-Price, L. K. Nieman, R. Pasquali, F. Pecori Giraldi, R. Pivonello, M. Reincke, F. Santeusanio, M. T. Sartori, N. Sonino, G. K. Stalla, M. Terzolo, S. Tsagarakis, M. L. Vance, and R. Vettor.

Abbreviations: BIPSS, Bilateral IPS sampling; BMD, bone mineral density; CBG, corticosteroid-binding globulin; CD, Cushing’s disease; CS, Cushing’s syndrome; CT, computed tomography; DEXA, dual energy x-ray absorptiometry; DST, dexamethasone suppression test(s); IPS, inferior petrosal sinus; MRI, magnetic resonance imaging; UFC, urinary free cortisol.

This article has been cited by other articles:

				S M Webb, X Badia, M J Barahona, A Colao, C J Strasburger, A Tabarin, M O van Aken, R Pivonello, G Stalla, S W J Lamberts, et al. Evaluation of health-related quality of life in patients with Cushing's syndrome with a new questionnaire Eur. J. Endocrinol., May 1, 2008; 158(5): 623 - 630. [Abstract] [Full Text] [PDF]

				M. B. Elamin, M. H. Murad, R. Mullan, D. Erickson, K. Harris, S. Nadeem, R. Ennis, P. J. Erwin, and V. M. Montori Accuracy of Diagnostic Tests for Cushing's Syndrome: A Systematic Review and Metaanalyses J. Clin. Endocrinol. Metab., May 1, 2008; 93(5): 1553 - 1562. [Abstract] [Full Text] [PDF]

				C. G. Patil, D. M. Prevedello, S. P. Lad, M. L. Vance, M. O. Thorner, L. Katznelson, and E. R. Laws Jr. Late Recurrences of Cushing's Disease after Initial Successful Transsphenoidal Surgery J. Clin. Endocrinol. Metab., February 1, 2008; 93(2): 358 - 362. [Abstract] [Full Text] [PDF]

				F Castinetti, I Morange, P Jaquet, B Conte-Devolx, and T Brue Ketoconazole revisited: a preoperative or postoperative treatment in Cushing's disease Eur. J. Endocrinol., January 1, 2008; 158(1): 91 - 99. [Abstract] [Full Text] [PDF]

				S. Kidambi, H. Raff, and J. W Findling Limitations of nocturnal salivary cortisol and urine free cortisol in the diagnosis of mild Cushing's syndrome Eur. J. Endocrinol., December 1, 2007; 157(6): 725 - 731. [Abstract] [Full Text] [PDF]

				F. Pecori Giraldi, A. G. Ambrogio, M. De Martin, L. M. Fatti, M. Scacchi, and F. Cavagnini Specificity of First-Line Tests for the Diagnosis of Cushing's Syndrome: Assessment in a Large Series J. Clin. Endocrinol. Metab., November 1, 2007; 92(11): 4123 - 4129. [Abstract] [Full Text] [PDF]

				I. Chiodini, M. L. Mascia, S. Muscarella, C. Battista, S. Minisola, M. Arosio, S. A. Santini, G. Guglielmi, V. Carnevale, and A. Scillitani Subclinical Hypercortisolism among Outpatients Referred for Osteoporosis Ann Intern Med, October 16, 2007; 147(8): 541 - 548. [Abstract] [Full Text] [PDF]

				J R Meinardi, B H R Wolffenbuttel, and R P F Dullaart Cyclic Cushing's syndrome: a clinical challenge Eur. J. Endocrinol., September 1, 2007; 157(3): 245 - 254. [Abstract] [Full Text] [PDF]

				F Castinetti, I Morange, H Dufour, P Jaquet, B Conte-Devolx, N Girard, and T Brue Desmopressin test during petrosal sinus sampling: a valuable tool to discriminate pituitary or ectopic ACTH-dependent Cushing's syndrome Eur. J. Endocrinol., September 1, 2007; 157(3): 271 - 277. [Abstract] [Full Text] [PDF]

				D. L. Batista, J. Riar, M. Keil, and C. A. Stratakis Diagnostic Tests for Children Who Are Referred for the Investigation of Cushing Syndrome Pediatrics, September 1, 2007; 120(3): e575 - e586. [Abstract] [Full Text] [PDF]

				A. Dovio, B. Allasino, E. Palmas, M. Ventura, A. Pia, L. Saba, E. Aroasio, M. Terzolo, and A. Angeli Increased Osteoprotegerin Levels in Cushing's Syndrome Are Associated with an Adverse Cardiovascular Risk Profile J. Clin. Endocrinol. Metab., May 1, 2007; 92(5): 1803 - 1808. [Abstract] [Full Text] [PDF]

				L F Chan, H L Storr, P N Plowman, L A Perry, G M Besser, A B Grossman, and M O Savage Long-term anterior pituitary function in patients with paediatric Cushing's disease treated with pituitary radiotherapy Eur. J. Endocrinol., April 1, 2007; 156(4): 477 - 482. [Abstract] [Full Text] [PDF]

				S. H. Golden, S. Malhotra, G. S. Wand, F. L. Brancati, D. Ford, and K. Horton Adrenal Gland Volume and Dexamethasone-Suppressed Cortisol Correlate with Total Daily Salivary Cortisol in African-American Women J. Clin. Endocrinol. Metab., April 1, 2007; 92(4): 1358 - 1363. [Abstract] [Full Text] [PDF]

				R M Testa, N Albiger, G Occhi, F Sanguin, M Scanarini, S Berlucchi, M P Gardiman, C Carollo, F Mantero, and C Scaroni The usefulness of combined biochemical tests in the diagnosis of Cushing's disease with negative pituitary magnetic resonance imaging Eur. J. Endocrinol., February 1, 2007; 156(2): 241 - 248. [Abstract] [Full Text] [PDF]

				I. C. Mitchell and F. E. Nwariaku Adrenal Masses in the Cancer Patient: Surveillance or Excision Oncologist, February 1, 2007; 12(2): 168 - 174. [Abstract] [Full Text] [PDF]

				F Castinetti, M Nagai, H Dufour, J-M Kuhn, I Morange, P Jaquet, B Conte-Devolx, J Regis, and T Brue Gamma knife radiosurgery is a successful adjunctive treatment in Cushing's disease Eur. J. Endocrinol., January 1, 2007; 156(1): 91 - 98. [Abstract] [Full Text] [PDF]

				I. Chiodini, G. Adda, A. Scillitani, F. Coletti, V. Morelli, S. Di Lembo, P. Epaminonda, B. Masserini, P. Beck-Peccoz, E. Orsi, et al. Cortisol Secretion in Patients With Type 2 Diabetes: Relationship with chronic complications Diabetes Care, January 1, 2007; 30(1): 83 - 88. [Abstract] [Full Text] [PDF]

				G. Assie, H. Bahurel, J. Coste, S. Silvera, M. Kujas, M.-A. Dugue, F. Karray, B. Dousset, J. Bertherat, P. Legmann, et al. Corticotroph Tumor Progression after Adrenalectomy in Cushing's Disease: A Reappraisal of Nelson's Syndrome J. Clin. Endocrinol. Metab., January 1, 2007; 92(1): 172 - 179. [Abstract] [Full Text] [PDF]

				A. Klibanski, A. E. Stephen, M. F. Greene, M. A. Blake, and C.-L. Wu Case 36-2006 -- A 35-Year-Old Pregnant Woman with New Hypertension. N. Engl. J. Med., November 23, 2006; 355(21): 2237 - 2245. [Full Text] [PDF]

				R. PASQUALI, V. VICENNATI, M. CACCIARI, and U. PAGOTTO The Hypothalamic-Pituitary-Adrenal Axis Activity in Obesity and the Metabolic Syndrome Ann. N.Y. Acad. Sci., November 1, 2006; 1083(1): 111 - 128. [Abstract] [Full Text] [PDF]

				C J Peters, H L Storr, A B Grossman, and M O Savage The role of corticotrophin-releasing hormone in the diagnosis of Cushing's syndrome Eur. J. Endocrinol., November 1, 2006; 155(suppl_1): S93 - S98. [Abstract] [Full Text] [PDF]

				S. Bilodeau, S. Vallette-Kasic, Y. Gauthier, D. Figarella-Branger, T. Brue, F. Berthelet, A. Lacroix, D. Batista, C. Stratakis, J. Hanson, et al. Role of Brg1 and HDAC2 in GR trans-repression of the pituitary POMC gene and misexpression in Cushing disease. Genes & Dev., October 15, 2006; 20(20): 2871 - 2886. [Abstract] [Full Text] [PDF]

				J. W. Findling and H. Raff Cushing's Syndrome: Important Issues in Diagnosis and Management J. Clin. Endocrinol. Metab., October 1, 2006; 91(10): 3746 - 3753. [Abstract] [Full Text] [PDF]

				V. Castillo, D. Giacomini, M. Paez-Pereda, J. Stalla, M. Labeur, M. Theodoropoulou, F. Holsboer, A. B. Grossman, G. K. Stalla, and E. Arzt Retinoic Acid as a Novel Medical Therapy for Cushing's Disease in Dogs Endocrinology, September 1, 2006; 147(9): 4438 - 4444. [Abstract] [Full Text] [PDF]

				J. Zhang, F. T F Tsai, and D. S Geller Differential interaction of RU486 with the progesterone and glucocorticoid receptors. J. Mol. Endocrinol., August 1, 2006; 37(1): 163 - 173. [Abstract] [Full Text] [PDF]

				A. A KASPERLIK-ZALUSKA, E. ROSLONOWSKA, J. SLOWINSKA-SRZEDNICKA, M. OTTO, A. CICHOCKI, J. CWIKLA, R. SLAPA, and G. EISENHOFER 1,111 patients with adrenal incidentalomas observed at a single endocrinological center: incidence of chromaffin tumors. Ann. N.Y. Acad. Sci., August 1, 2006; 1073: 38 - 46. [Abstract] [Full Text] [PDF]

				S. PETERSENN, N. UNGER, M. K WALZ, and K. MANN Diagnostic value of biochemical parameters in the differential diagnosis of an adrenal mass. Ann. N.Y. Acad. Sci., August 1, 2006; 1073: 348 - 357. [Abstract] [Full Text] [PDF]

				R. J. Weil, A. O. Vortmeyer, L. K. Nieman, H. L. DeVroom, J. Wanebo, and E. H. Oldfield Surgical Remission of Pituitary Adenomas Confined to the Neurohypophysis in Cushing's Disease J. Clin. Endocrinol. Metab., July 1, 2006; 91(7): 2656 - 2664. [Abstract] [Full Text] [PDF]

				M. B. C C. Neto, M. C. Machado, F. Mesquita, N. R. de Castro Musolino, A. C. Toscanini, G. Ochman, V. A. S Cescato, R. Marino Jr, and M. J. Teixeira Thymus hyperplasia after resolution of hypercortisolism in ACTH-dependent Cushing's syndrome: the importance of thymic vein catheterization. Eur. J. Endocrinol., June 1, 2006; 154(6): 807 - 811. [Abstract] [Full Text] [PDF]

				T Taguchi, T Takao, Y Iwasaki, M Nishiyama, K Asaba, and K Hashimoto Suppressive effects of dehydroepiandrosterone and the nuclear factor-{kappa}B inhibitor parthenolide on corticotroph tumor cell growth and function in vitro and in vivo J. Endocrinol., February 1, 2006; 188(2): 321 - 331. [Abstract] [Full Text] [PDF]

				J. R. Lindsay, T. Nansel, S. Baid, J. Gumowski, and L. K. Nieman Long-Term Impaired Quality of Life in Cushing's Syndrome despite Initial Improvement after Surgical Remission J. Clin. Endocrinol. Metab., February 1, 2006; 91(2): 447 - 453. [Abstract] [Full Text] [PDF]

				T. L. Mazzuco, O. Chabre, N. Sturm, J.-J. Feige, and M. Thomas Ectopic Expression of the Gastric Inhibitory Polypeptide Receptor Gene Is a Sufficient Genetic Event to Induce Benign Adrenocortical Tumor in a Xenotransplantation Model Endocrinology, February 1, 2006; 147(2): 782 - 790. [Abstract] [Full Text] [PDF]

				C. Kristo, R. Jemtland, T. Ueland, K. Godang, and J. Bollerslev Restoration of the coupling process and normalization of bone mass following successful treatment of endogenous Cushing's syndrome: A prospective, long-term study Eur. J. Endocrinol., January 1, 2006; 154(1): 109 - 118. [Abstract] [Full Text] [PDF]

				G. G.-v. Simson, B. Kohn, and F. B. Axelrod Cushing Syndrome from Topical Foam Steroid Use in an Adolescent Male Clinical Pediatrics, January 1, 2006; 45(1): 97 - 100. [PDF]

				G. Minniti, V. Esposito, M. Piccirilli, A. Fratticci, A. Santoro, and M.-L. Jaffrain-Rea Diagnosis and management of pituitary tumours in the elderly: a review based on personal experience and evidence of literature Eur. J. Endocrinol., December 1, 2005; 153(6): 723 - 735. [Abstract] [Full Text] [PDF]

				P Haentjens, L De Meirleir, R Abs, J Verhelst, K Poppe, and B Velkeniers Glomerular filtration rate in patients with Cushing's disease: a matched case-control study Eur. J. Endocrinol., December 1, 2005; 153(6): 819 - 829. [Abstract] [Full Text] [PDF]

				I. Chiodini, M. Torlontano, A. Scillitani, M. Arosio, S. Bacci, S. Di Lembo, P. Epaminonda, G. Augello, R. Enrini, B. Ambrosi, et al. Association of subclinical hypercortisolism with type 2 diabetes mellitus: a case-control study in hospitalized patients Eur. J. Endocrinol., December 1, 2005; 153(6): 837 - 844. [Abstract] [Full Text] [PDF]

				K. Templeton Secondary Osteoporosis J. Am. Acad. Ortho. Surg., November 1, 2005; 13(7): 475 - 486. [Abstract] [Full Text] [PDF]

				R. Giordano, A. Picu, U. Pagotto, R. De Iasio, L. Bonelli, F. Prodam, F. Broglio, L. Marafetti, R. Pasquali, M. Maccario, et al. The negative association between total ghrelin levels, body mass and insulin secretion is lost in hypercortisolemic patients with Cushing's disease Eur. J. Endocrinol., October 1, 2005; 153(4): 535 - 543. [Abstract] [Full Text] [PDF]

				J. R. Lindsay and L. K. Nieman The Hypothalamic-Pituitary-Adrenal Axis in Pregnancy: Challenges in Disease Detection and Treatment Endocr. Rev., October 1, 2005; 26(6): 775 - 799. [Abstract] [Full Text] [PDF]

				A. Viardot, P. Huber, J. J. Puder, H. Zulewski, U. Keller, and B. Muller Reproducibility of Nighttime Salivary Cortisol and Its Use in the Diagnosis of Hypercortisolism Compared with Urinary Free Cortisol and Overnight Dexamethasone Suppression Test J. Clin. Endocrinol. Metab., October 1, 2005; 90(10): 5730 - 5736. [Abstract] [Full Text] [PDF]

				M. Locatelli, M. L. Vance, and E. R. Laws CLINICAL REVIEW: The Strategy of Immediate Reoperation for Transsphenoidal Surgery for Cushing's Disease J. Clin. Endocrinol. Metab., September 1, 2005; 90(9): 5478 - 5482. [Abstract] [Full Text] [PDF]

				M. H. Bassett, B. Mayhew, K. Rehman, P. C. White, F. Mantero, G. Arnaldi, P. M. Stewart, I. Bujalska, and W. E. Rainey Expression Profiles for Steroidogenic Enzymes in Adrenocortical Disease J. Clin. Endocrinol. Metab., September 1, 2005; 90(9): 5446 - 5455. [Abstract] [Full Text] [PDF]

				M. Terzolo, S. Bovio, A. Pia, P. A. Conton, G. Reimondo, C. Dall'Asta, D. Bemporad, A. Angeli, G. Opocher, M. Mannelli, et al. Midnight serum cortisol as a marker of increased cardiovascular risk in patients with a clinically inapparent adrenal adenoma Eur. J. Endocrinol., August 1, 2005; 153(2): 307 - 315. [Abstract] [Full Text] [PDF]

				Y. S. Woo, A. M. Isidori, W. Z. Wat, G. A. Kaltsas, F. Afshar, I. Sabin, P. J. Jenkins, J. P. Monson, G. M. Besser, and A. B. Grossman Clinical and Biochemical Characteristics of Adrenocorticotropin-Secreting Macroadenomas J. Clin. Endocrinol. Metab., August 1, 2005; 90(8): 4963 - 4969. [Abstract] [Full Text] [PDF]

				J. van der Hoek, M. Waaijers, P. M. van Koetsveld, D. Sprij-Mooij, R. A. Feelders, H. A. Schmid, P. Schoeffter, D. Hoyer, D. Cervia, J. E. Taylor, et al. Distinct functional properties of native somatostatin receptor subtype 5 compared with subtype 2 in the regulation of ACTH release by corticotroph tumor cells Am J Physiol Endocrinol Metab, August 1, 2005; 289(2): E278 - E287. [Abstract] [Full Text] [PDF]

				J. R. Lindsay, J. Jonklaas, E. H. Oldfield, and L. K. Nieman Cushing's Syndrome during Pregnancy: Personal Experience and Review of the Literature J. Clin. Endocrinol. Metab., May 1, 2005; 90(5): 3077 - 3083. [Abstract] [Full Text] [PDF]

				G. D. Hammer, J. B. Tyrrell, K. R. Lamborn, C. B. Applebury, E. T. Hannegan, S. Bell, R. Rahl, A. Lu, and C. B. Wilson Transsphenoidal Microsurgery for Cushing's Disease: Initial Outcome and Long-Term Results J. Clin. Endocrinol. Metab., December 1, 2004; 89(12): 6348 - 6357. [Abstract] [Full Text] [PDF]

				M. Yaneva, H. Mosnier-Pudar, M.-A. Dugue, S. Grabar, Y. Fulla, and X. Bertagna Midnight Salivary Cortisol for the Initial Diagnosis of Cushing's Syndrome of Various Causes J. Clin. Endocrinol. Metab., July 1, 2004; 89(7): 3345 - 3351. [Abstract] [Full Text] [PDF]