Fifteen years of the European Registry of Cushing’s syndrome (ERCUSYN)

It is now 15 years since Steven Lamberts, John Wass, Peter Trainer, Christian Strasburger and Susan Webb teamed up to apply for the European Union (EU) call under the Public Health Program for funds to set up a European Registry of Cushing’s syndrome (ERCUSYN). Cushing’s is a disease for which, at that time, there were very few medical treatment options. The proposal was successfully funded in 2007-2010. With the invaluable contribution of the Berlin-based IT company Lohmann & Birkner, a specific database was designed, updated in 2018. To date, the project has recruited information from over 2000 patients, from 57 centres covering 26 countries.

 The aims of the registry include:

  • Developing an international collaboration to increase understanding of the natural history of Cushing’s syndrome (CS), a rare disorder with an annual incidence of 2-3 new cases per million inhabitants
  • increasing awareness of CS among general practitioners and primary care physicians throughout Europe
  • making earlier diagnosis and reducing the diagnostic delay, often of several years •
  • improving long-term prognosis and “normalising” the increased morbidity and mortality associated with CU, even if not treated correctly
  • helping prevent the impairment in quality of life for patients
  • setting up European guidelines for diagnosis and therapeutic strategies for CU

From the beginning it was clear that this European initiative had to involve the European Society of Endocrinology, who is the owner of the database and has supported the project in many ways. It is run by a steering committee which is currently formed by SM Webb, C. Strasburger, A Tabarin and J Newell-Price, a quality control manager (E Valassi) and a project manager (A Santos), while analysis, IT and database support is provided by H Franz, and D Thyroke, of the firm Lohmann & Birkner Health Care Consulting GmbH, Berlin, Germany.

Manuscripts generated by ERCUSYN

(some of them among the most cited of the year in European Journal of Endocrinology)

  1. The European Registry on Cushing's syndrome: 2-year experience. Baseline demographic and clinical characteristics. Eur J Endocrinol 2011, 165:383-92
  2. The value of a European registry for pituitary adenomas: the example of Cushing's syndrome registry. Ann Endocrinol (Paris). 2012, 73(2):83-9
  3. Mapping Cushing QoL scores to EQ-5D utility values using data from the European Registry on Cushing's syndrome (ERCUSYN). Qual Life Res 2013, 22:2941-50
  4. Diagnostic tests for Cushing's syndrome differ from published guidelines: data from ERCUSYN. Eur J Endocrinol 2017, 176:613-24
  5. Preoperative medical treatment in Cushing's syndrome: frequency of use and its impact on postoperative assessment: data from ERCUSYN. Eur J Endocrinol 2018, 178:399-409
  6. Worse Health-Related Quality of Life at long-term follow-up in patients with Cushing’s disease than patients with cortisol producing adenoma. Data from the ERCUSYN. Clin Endocrinol 2018, 88:787-98
  7. High mortality within 90 days of diagnosis in patients with Cushing's syndrome - Results from the ERCUSYN registry. Eur J Endocrinol 2019, 181:461-72

A new manuscript on Nelson’s syndrome within ERCUSYN is currently being finalised. Sustainability of the database over these years has been a challenge, and only possible after reaching agreements, fostered on occasions by the European Medicines Agency (EMA), connecting the project with pharmaceutical companies who had to perform post-authorisation safety and security studies for several of the new drugs for Cushing’s syndrome that have become available over the last decade (i.e. Novartis and HRA-Pharma), or otherwise (Ipsen).

All partners are encouraged to send scientific proposals on possible data analysis, which are periodically evaluated by the scientific steering committee. The ERCUSYN project welcomes European centres of excellence for CS to join and contribute to the project, if they are prepared to include patients and update their outcome periodically.

For further information contact asantos@ercusyn.eu

Susan M Webb, Alicia Santos, Elena Valassi

IIB-Sant Pau and Department of Endocrinology/Medicine, Hospital Sant Pau, Universitat Autónoma de Barcelona, and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBER-ER, Unit 747), ISCIII; Carrer de Sant Quintí 89, 08041- Barcelona, Spain