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Endocrine Abstracts (2014) 35 OC5.1 | DOI: 10.1530/endoabs.35.OC5.1

1IIB-Sant Pau and Department of Endocrinology/Medicine, Hospital Sant Pau, UAB and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unidad 747), ISCIII, Barcelona, Spain; 2Association pour le Devéloppement des Recherches Biologiques et Médicales, Marseilles, France; 3Department of Medicine, Division of Endocrinology, Radboud University, Nijmegen Medical Center, Nijmegen, The Netherlands; 4Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands; 5Department of Pituitary and Adrenal Diseases, Medical University of Sofia, Sofia, Bulgaria; 6Athens Polyclinic General Hospital and Evangelisnos Hospital, Athens, Greece; 7Department of Endocrinology, University Medical Center, Ljubljana, Slovenia; 8Univ. Paris-Sud, UMR-S693, Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Service de Endocrinologie et des Maladies de la Reproduction, Institut National de la Santé et de la Recherche Méd, Paris, France; 9Service d’Endocrinologie-Diabétologie-Nutrition, Grenoble Cedex, France; 10Division of Clinical Endocrinology, Department of Medicine CCM, Charité- Universitätsmedizin, Berlin, Germany; 112nd Department of Internal Medicine, Semmelweis University, Budapest, Hungary; 12Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Oxford, UK; 13Praxis für Endokrinologie, Oldenburg, Germany; 14Université Catholique de Louvain, St-Luc Hospital, Louvain, Belgium; 15Department of Endocrinology, University Hospital, Zagreb, Croatia; 16Moscow Regional Res. Clin. Inst. n.a.Vladimirsky, Moscow, Russia; 17Lohmann and Birkner Health Care Consulting GmbH, Berlin, Germany.

The European Registry on Cushing’s Syndrome (ERCUSYN) is designed to collect prospective and follow-up data on patients with Cushing’s syndrome (CS) and currently (Sep 2013) includes 1006 patients (804 F, 202 M; mean age (+S.D.) 44.7±13.3 years) from 57 centers in 28 countries.

Six hundred and sixty one (66%) had pituitary-dependent CS (PIT-CS), 242 (24%) adrenal-dependent CS (ADR-CS), and 103 (10%) CS from other etiologies, including ectopic (ECT-CS). Death occurred in 35 patients (3%), 43% in PIT-CS and 31% in ECT-CS. Urinary free cortisol (UFC) supported the correct diagnosis in 92% PIT-CS, 86% ADR-CS and 100% ECT-CS. Overnight 1 mg dexamethasone suppression test (1 mg DST) reliably identified 87% PIT-CS, 90% ADR-CS, and 96% ECT-CS. Late night salivary cortisol was diagnostic in 92% PIT-CS, 87% ADR-CS, and 93% ECT-CS. Medical treatment was the first therapeutic option in 286 patients, the majority having PIT-CS (74%). Surgery was the first-line treatment in 624 patients, 399 with PIT-CS (64%), 185 with ADR-CS (30%) and 40 with other etiologies (6%). Of 553 PIT-CS patients who underwent transsphenoidal surgery, 376 (68%) experienced short-term remission (within 2 weeks of surgery), whereas 81 (15%) were not cured. Immediate clinical improvement was only observed in 19/95 (20%) patients with normal postoperative cortisol, and did not occur in a subset of 14 of 351 (4%) with postoperative hypocortisolism. Bilateral adrenalectomy was performed in 6%, 38% with ECT-CS. Long-term remission was described in 84% of PIT-CS with available data (mean follow-up, 43+38 months), 87% of ADR-CS (after 34+28 months), and 50% of ECT-CS (after 43+27 months).

After a mean follow-up of 3.5 years >80% of PIT-CS and ADR-CS in ERCUSYN are in long term remission, but only 50% of ECT-CS. ERCUSYN provides an extensive overview of currently used diagnostic and therapeutic procedures in CS at EU level.

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