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

Purpose: 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.

Methods: 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.

Results: 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).

Conclusions: 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.