Endocrine Abstracts (2011) 26 P20

Clinical presentation of Cushing's syndrome in a large series from the European Registry on Cushing's syndrome (ERCUSYN)

Elena Valassi1, Alicia Santos1, Maria Yaneva2, Miklos Toth3, Christian Strasburger4, Phillippe Chanson5, John Wass6, Olivier Chabre7, Marija Pfeifer8, Richard Feelders9, Stylianos Tsagarakis10, Peter Trainer11, Holger Franz12, Kathrin Zopf4, Sabina Zacharieva2, Steven Lamberts9 & Susan Webb1

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; 2Medical University of Sofia, Sofia, Bulgaria; 3Semmelweis Egyetem, II. Belgyógyászati Klinika, Budapest, Hungary; 4Division of Clinical Endocrinology, Department of Medicine CCM, Charité-Universitätsmedizin, Berlin, Germany; 5Assistance Publique, Hôpitaux de Paris, Paris, France; 6Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, Oxford, UK; 7Service d’Endocrinologie-Diabétologie-Nutrition, Grenoble Cedex, France; 8Klinicni center Ljubljana, Ljubljana, Slovenia; 9Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands; 10Athens Polyclinic General Hospital and Evangelisnos Hospital, Athens, Greece; 11Department of Endocrinology, Christie Hospital, Manchester, UK; 12Lohmann and Birkner, Berlin, Germany.

The European Registry on Cushing’s syndrome (ERCUSYN) is designed to collect prospective and follow-up data at EU level on patients with Cushing’s syndrome (CS). Baseline data on 481 CS patients (390 females, 91 males; mean age (±S.D.): 44±14 years) collected from 36 centres in 23 countries. This cohort included new patients since 2008 and retrospective cases since 2005. Patients were divided into four major etiologic groups: pituitary-dependent CS (PIT-CS) (66%), adrenal-dependent CS (ADR-CS) (27%), CS from an ectopic source (ECT-CS) (5%) and CS from other etiologies (OTH-CS) (2%). Proportion of men in the ECT-CS group was significantly higher than in the other etiologic groups (P<0.05). Patients in the ADR-CS group were significantly older than those in the PIT-CS group (P<0.05).

Prevalence of hirsutism and diabetes in ECT-CS patients was 92 and 74%, respectively, which was significantly higher as compared with the other etiologic groups (P<0.05 for hirsutism and P<0.01 for diabetes). Patients with PIT-CS had significantly more skin alterations, menstrual irregularities and hirsutism than those with ADR-CS (P<0.01). Reduced libido was more prevalent in men than in women (P<0.01). Prevalence of osteoporosis at spine was significantly higher in men as compared with women (P<0.05), and males had significantly more vertebral and rib fractures than females (52 vs 18% for vertebrae; P<0.001; 34 vs 23% for ribs; P<0.05). Patients with ECT-CS consulted a diabetologist more frequently than those with ADR-CS (P<0.05), while a gynecologist was consulted more often by women with either PIT-CS or ADR-CS than those in the ECT-CS group (P<0.05). Overall, weight gain was significantly more common in women as compared with men (P<0.01). EuroQoL-VAS score in PIT-CS was significantly better than in ADR-CS (P<0.05), while CushingQoL score did not differ between groups. The ERCUSYN project allows an analysis of the heterogeneous clinical presentation and course of CS at a European level, depending on gender and etiology.

Supported by a PHP grant 800200 of the EU.