Adrenal crisis: incidence, clinical presentation and risk factors in patients with Addison's disease

Adrenal crisis (AC) is the most severe manifestation of primary adrenal insufficiency (PAI) and is associated with high mortality. Its insidious presentation often leads to a delayed diagnosis and treatment. In this study, we aim to determine the incidence, characteristics, precipitating and predisposing factors for AC.

A total of 132 patients with PAI were evaluated retrospectively. Patients' features and information on their previous AC were collected through a structured interview and clinical documentation, supplied by patients or retrieved from archives of the Hospitals of Padova and Venice-Mestre.

Among enrolled patients, 95 (71.9%) were females, the mean age was 48.8 ± 13.5 years, and the mean PAI duration was 14.9 ± 11.8 years. The most frequent cause of PAI was autoimmune adrenalitis (92.4%), mainly associated with other autoimmune comorbidities. The 65.9% of patients experienced at least one AC, with an incidence of 10.5/100 patient-years. A significant number of AC (116/206) occurred in patients already undergoing glucocorticoid therapy. The most frequent features of AC were fatigue (96%), gastrointestinal symptoms (85%), and hyponatremia (77%). The most frequent triggers were vomiting and/or diarrhoea (65%), infections (38%), and emotional stress (29%). Patients with more autoimmune comorbidities and those with premature ovarian insufficiency (POI) were at higher risk of AC (p < 0.02).

AC is still a frequent complication of patients with PAI, particularly in those with additional autoimmune comorbidities or with POI. Awareness of AC among clinicians, patients, and care-givers is crucial for the prevention, recognition, and proper treatment of this life-threatening complication.