European Journal of Endocrinology (2009) In press
Copyright © 2009 by European Society of Endocrinology
Stefanie Hahner, Melanie Loeffler, Benjamin Bleicken, Christiane Drechsler, Danijela Milovanovic, Martin Fassnacht, Manfred Ventz, Marcus Quinkler and Bruno Allolio
S Hahner, Endocrinology and Diabetes Unit, University of Wuerzburg, Wuerzburg, D-97080 , Germany
M Loeffler, University of Wuerzburg, Endocrinology and Diabetes Unit, Würzburg, Germany
B Bleicken, Dept. of Medicine I, University of Wuerzburg, Würzburg, Germany
C Drechsler, Dept. of Nephrology, University of Würzburg, Würzburg, Germany
D Milovanovic, University of Wuerzburg, Endocrinology and Diabetes Unit, Würzburg, Germany
M Fassnacht, Dept. of Medicine I, University of Würzburg, Würzburg, Germany
M Ventz, Dept. of Medicine I, University of Wuerzburg, Würzburg, Germany
M Quinkler, Dept. of Medicine I, University of Wuerzburg, Würzburg, Germany
B Allolio, University of Wuerzburg, Endocrinology and Diabetes Unit, Würzburg, Germany
Correspondence: Stefanie Hahner, Email: firstname.lastname@example.org
Objective: Adrenal crisis (AC) is a life-threatening complication of adrenal insufficiency (AI). Here we evaluated frequency, causes and risk factors of AC in patients with chronic AI.
Methods: In a cross-sectional study 883 patients with AI were contacted by mail. 526 patients agreed to participate and received a disease specific questionnaire.
Results: 444 data sets were available for analysis (primary adrenal insufficiency, PAI n=254, secondary adrenal insufficiency, SAI n=190). 42% (PAI 47%, SAI 35%) reported at least one crisis. 384 AC in 6092 patient years were documented (frequency of 6.3 crises/100 patient years). Precipitating causes were mainly gastrointestinal infection and fever (45%) but also other stressful events (e.g. major pain, surgery, psychic distress, heat, pregnancy). Sudden onset of apparently unexplained AC was also reported (PAI 6.6%, SAI 12.7%). Patients with PAI reported more frequent emergency glucocorticoid administration (42.5% vs 28.4%, p=0.003)) Crisis incidence was not influenced by educational status, BMI, glucocorticoid dose, DHEA treatment, age at diagnosis, hypogonadism, hypothyroidism or growth hormone deficiency. In PAI, patients with concomitant non-endocrine disease were at higher risk of crisis (OR=2.02, 95% CI 1.05-3.89, p=0.036). In SAI, female sex (OR=2.18, 95%CI 1.06-4.5, p=0.035) and diabetes insipidus (RR=2.71, 95%CI 1.22-5.99, p=0.014) were associated with higher crisis incidence.
Conclusion: AC occurs in a substantial proportion of patients with chronic AI, mainly triggered by infectious disease. Only a limited number of risk factors suitable for targeting prevention of AC were identified. These findings indicate the need for new concepts of crisis prevention in patients with AI.