Introduction: CD is a rare disease. Our aim was to describe demographic and clinical characteristics together with initial management of patients with CD.
Patients and methods: A retrospective analysis of 437 CD patients diagnosed between 1996 and 2009, followed in 5 tertiary centers (Bordeaux, Grenoble, Lyon, Marseille, Paris) was performed in the frame of the national program for rare diseases.
Results: 347 (80%) were women. Mean age at diagnosis was 40.5±15.8 years, and mean time between first symptoms and diagnosis was 34.4±38.4 months. A bilateral inferior petrosal sinus sampling was performed in pretreatment evaluation in 32% of patients. Pretreatment pituitary magnetic resonance (MR) imaging was normal in 20% of patients, allowed adenoma visualisation in 66.7% and was inconclusive in 13.3%. Initial clinical and biological evaluation before treatment showed: body mass index above 25 and 30 kg/m2 in respectively 31 and 35%, hypertension (53%), elevated fasting or post-prandial glycemia (26%), psychiatric disorder (32%), clinical hypogonadism (38%), and fractures (21%). Pretreatment bone mineral density was only available in 110 patients: lombar T-score and femoral T-score were under 2.5 DS in respectively 16 and 8% of these patients. 338 patients (77%) had transsphenoidal surgery as first line treatment, leading to positive histology of corticotroph adenoma in 81%. In the 299 patients with immediate postoperative data available, remission was obtained in 225 (75%) cases. Remission rate was higher in patient with positive pre-treatment MR (89 vs 71%, P=0.004).
Conclusion: This large multicenter cohort of patients with CD allowed to characterize initial demographics and clinical manifestations of the disease, together with immediate pituitary surgery outcomes. The longitudinal study of this cohort should enable to determine long term evolution of CD manifestation after treatment.
30 Apr - 04 May 2011
European Society of Endocrinology