Introduction: Current understanding about the frequency of adrenal crisis in patients with adrenal insufficiency (AI) relies exclusively on retrospective data (~6.3 adrenal crisis per 100 patient-years). In addition, no data are available about the frequency of intercurrent illness events in AI patients. For the first time we prospectively used a patients diary in recording events of adrenal crisis and intercurrent illness in AI patients.
Design: The European Adrenal Insufficiency Registry (EU-AIR) with 20 centres across Germany, the Netherlands, Sweden and the UK started enrolling patients with AI in August 2012. At enrolment, comprehensive demographic and baseline data, etiology of AI, and details of glucocorticoid replacement therapy, are collected electronically. Safety data (intercurrent illnesses and adrenal crisis) and treatment information are collected prospectively at subsequent clinic visits. Patient diaries are used to record intercurrent illnesses and illness-related dose changes between visits and are entered in the database at subsequent clinic visits.
Results: Up to December 2013, 683 patients were registered in EU-AIR (241 primary AI, 399 secondary AI and 43 CAH) resulting in a total of 165 patient years (78 py in primary AI, 74 py in secondary AI and 13 py in CAH). Diary entries revealed a mean of 2.23 events of intercurrent illness/py with no specific differences among different AI etiologies. Adrenal crisis occurred with a frequency of 9.7/100 py, ranging from 8.1/100 py in patients with secondary AI to 12.8/100 py in patients with primary AI.
Conclusions: Using a patients diary improves the detection of intercurrent illness and adrenal crisis events in AI patients. Using this prospective methodology the rate of adrenal crisis appears significantly higher than previously suggested.
03 - 07 May 2014
European Society of Endocrinology