Adrenal crisis (AC) is a rare but life-threatening complication in chronic adrenal failure. Here we evaluated frequency, causes and potential risk factors of AC in a large sample of patients with primary AI (PAI) or secondary AI (SAI).
In a cross-sectional study 883 patients with AI were contacted by mail. 526 patients agreed to participate and received a disease specific questionnaire. Diagnoses and co-morbidities were verified by review of medical records.
Four hundred and forty four data sets were available for further analysis (PAI n=254, SAI n=190). 42% (PAI 47%, SAI 35%) reported at least one AC. 384 adrenal crises in 6092 patient years were documented corresponding to a frequency of 6.3 crises/100 patient years (PAI 5.1, SAI 8.2). Precipitating causes were mainly gastrointestinal infection and fever (45% of cases) but also other stressful events (e.g. major pain, surgery, psychic distress, heat, pregnancy) and even sudden unexplained occurrence were reported. Patients with PAI reported significantly more emergency glucocorticoid administrations (P=0.003) and AC (P=0.019). However, among those patients who had experienced at least one crisis, the frequency of AC was slightly higher in SAI than in PAI (15/100 patient years vs 13.4/100 patient years). Incidence of AC 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 tended to be at higher risk for AC (RR=1.24, P=0.057). In SAI, female gender (RR=1.26, P=0.043) and diabetes insipidus (RR=1.25, P=0.033) were significantly associated with the incidence of AC.
AC occurs frequently in both PAI and SAI, mainly triggered by infectious disease. However, so far only minor risk factors for AC could be identified in this large cohort, indicating the need of repeated crisis prevention training in all patients with AI.