MANAGEMENT OF ENDOCRINE DISEASE: Epidemiology, quality of life and complications of primary adrenal insufficiency: a review

Abstract

In this article, we review published studies covering epidemiology, natural course and mortality in primary adrenal insufficiency (PAI) or Addison's disease. Autoimmune PAI is a rare disease with a prevalence of 100-220 per million inhabitants. It occurs as part of an autoimmune polyendocrine syndrome in more than half of the cases. The patients experience impaired quality of life, reduced parity and increased risk of preterm delivery. Following a conventional glucocorticoid replacement regimen leads to a reduction in bone mineral density and an increase in the prevalence of fractures. Registry studies indicate increased mortality, especially evident in patients diagnosed with PAI at a young age and in patients with the rare disease autoimmune polyendocrine syndrome type-1. Most notably, unnecessary deaths still occur because of adrenal crises. All these data imply the need to improve the therapy and care of patients with PAI.

Incidence, prevalence and seasonal onset variation of Addison’s disease among persons with type 1 diabetes mellitus: nationwide, matched cohort studies

“…Based on our study design and selection of persons, we could not calculate the total prevalence of AD; however, based on the results from the control subjects without T1DM in the general population, the prevalence of 208 per million inhabitants suggests that our estimate together with the more recent data from Iceland (12) is one of the highest reported. Both the incidence and the prevalence of T1DM are increasing globally (1) and the same trend might therefore be expected for AD (5). Our study showed an at least 2-fold higher incidence (based on 95% CI) of AD compared to older studies.…”

“…Both the incidence and the prevalence of AD are probably increasing in persons with T1DM (5). The prevalence of AD in different European populations varies between 93 and 221 per million and has an estimated incidence of 4.4-6.2 per million per year (Table 1) (6,7,8,9,10,11,12).…”

Addison’s Disease and Dilated Cardiomyopathy: A Case Report and Review of the Literature

“…Although autoimmune adrenalitis is considered to be the major cause of Addison's disease in up to 90% of diagnosed individuals, prevalent in female patients between 30 and 50 years of age, other etiologies include infectious, drug induced, and/or genetic factors [2, 3]. Common manifestations of this condition are hyponatremia, hyperkalemia, and/or hypoglycemia along with mucosal and skin hyperpigmentations [2, 3].…”

“…Although autoimmune adrenalitis is considered to be the major cause of Addison's disease in up to 90% of diagnosed individuals, prevalent in female patients between 30 and 50 years of age, other etiologies include infectious, drug induced, and/or genetic factors [2, 3]. Common manifestations of this condition are hyponatremia, hyperkalemia, and/or hypoglycemia along with mucosal and skin hyperpigmentations [2, 3]. Although cardiovascular manifestations of Addison's disease include hypotension, syncope, and arrhythmias, the development of a dilated cardiomyopathy and heart failure are an uncommon life-threatening complication [4–8].…”

Автоімунний Полігландулярний Синдром 2-Го Типу (Клінічний Випадок Синдрому Карпентера)

“…The main cause of the disease is autoimmune adrenalitis (about 90 % of patients), other etiologic factors include infectious, iatrogenic and genetic [2,3]. The main clinical manifestations of the disease include electrolyte disorders (hyponatremia and hyperkalemia, hypoglycemia, hyperpigmentation of the skin and mucous membranes) [2,3].…”