ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

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

Diagnostic and therapeutic outcome in ERCUSYN: Preliminary report in over 1000 patients

Elena Valassi1, Alicia Santos1, Thierry Brue2, Romana T. Netea-Maier3, Richard A. Feelders4, Maria Yaneva5, Stylianos Tsagarakis6, Marija Pfeifer7, Philippe Chanson8, Olivier Chabre9, Kathrin Zopf10, Judit Toke11, John AH Wass12, Michael Droste13, Dominique Maiter14, Tina Dusek15, Irina Komerdus16, Holger Franz17, Steven W.J. Lamberts4 & Christian J. Strasburger10

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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