Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group

Abstract

IntroductionPrimary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI.AimsTo characterize the clinical presentation, diagnostic workup, treatment and follow‐up of Portuguese patients with confirmed PAI.MethodsThis multicentre retrospective study examined PAI patients in 12 Portuguese hospitals.ResultsWe investigated 278 patients with PAI (55.8% were females), with a mean age of 33.6 ± 19.3 years at diagnosis. The most frequent presenting clinical features were asthenia (60.1%), mucocutaneous hyperpigmentation (55.0%) and weight loss (43.2%); 29.1% of the patients presented with adrenal crisis. Diagnosis was established by high plasma ACTH and low serum cortisol in most patients (43.9%). The most common aetiology of PAI was autoimmune adrenalitis (61.0%). There were 38 idiopathic cases. Autoimmune comorbidities were found in 70% of the patients, the most frequent being autoimmune thyroiditis (60.7%) and type 1 diabetes mellitus (17.3%). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3 ± 8.3 mg/day) mostly in three (57.5%) or two (37.4%) daily doses. The remaining patients were treated with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were also on fludrocortisone (median dose of 100 µg/day). Since diagnosis, 33.5% of patients were hospitalized for disease decompensation. In the last appointment, 17.2% of patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% had electrolyte disturbances.ConclusionThis is the first multicentre Portuguese study regarding PAI. The results emphasize the need for standardization in diagnostic tests and etiological investigation and provide a framework for improving treatment.

Eosinophil counts can be a predictive marker of immune checkpoint inhibitor-induced secondary adrenal insufficiency: a retrospective cohort study

“…Patients with pituitary irAEs often present with hyponatremia, defined as a serum sodium level of < 135 mmol/L and a detection rate of 40–100% 10 , 16 – 18 . It is also observed in adrenal crisis, the detection rate using the lower limit of the reference ranges from 9 to 50% 8 , 19 – 21 . Katabami et al developed a prediction model for adrenal crisis using data from the same individuals with chronic AI in a stable period during glucocorticoid replacement therapy as a control.…”

Poor quality of life and sleep in patients with adrenal insufficiency—another cause of increased mortality?

“…In this context, it may be interesting that patients with AI often suffer from psychiatric disorders [33,34]. Causal research is, however, challenging due to frequent co-morbidity with other endocrine disorders [35] which are also associated with a cumulative occurrence of affective disorders [36].…”

Development of a simple prediction model for adrenal crisis diagnosis

“…Although hyponatraemia is a distinguishing feature of AC, the detection rate using the lower limit of the reference ranges from 9 to 50% 8 – 10 . In agreement with the results of previous studies, in our study, the prevalence of hyponatraemia based on the reference range of each centre was 47.8%.…”