About this Research Topic
Primary adrenal insufficiency (PAI) is a rare, life-threating disease characterized by impaired secretion of steroids from the adrenal cortex, including glucocorticoids, mineralocorticoids, and androgens. During the last century, tuberculosis was the most common cause of PAI. Nowadays, in industrialized countries, PAI is mainly caused by an autoimmune adrenalitis.
Treatment consists of lifelong replacement with glucocorticoids and mineralocorticoids. Androgen replacement is seldom administered in women. Standard replacement regimen in PAI involves short-acting GCs, as hydrocortisone or cortisone acetate, usually administered twice or thrice-daily. Optimization of GC replacement therapy is challenging due to the lack of reliable objective biomarkers of GC effects. Available data suggest that many PAI patients may be over-replaced with GCs. Moreover, there is considerable evidence that the current replacement regime fails to reproduce the physiological circadian rhythm of cortisol.
Over-replacement and unphysiological exposure to cortisol have been associated with poor long-term outcomes. Particularly, it has been shown that patients with PAI on current replacement have a significantly reduced health-related quality of life (QoL), irrespective of age, sex, and concomitant disease. QoL seems to be worse in patients receiving hydrocortisone doses higher than 30 mg daily and in those diagnosed with a longer diagnostic delay.
It is widely recognized that patients with PAI have excess morbidity and reduced life expectancy, mainly due to cardiovascular and infectious diseases as well as malignancies. There are still several issues on clinical management of PAI and its associated complications that remain unclear.
The aim of this Research Topic is to promote a better understanding of this disease to improve management and long-term outcomes. Potential authors are encouraged to submit either original research articles or systematic reviews. Authors are also encouraged to analyze clinical outcomes with standard glucocorticoid replacement therapy in patients with primary adrenal insufficiency. Specifically, these issues will be addressed:
• Cognitive function and quality of life
• Cardiovascular comorbidities
• Bone metabolism
• Glucose metabolism
• Insights on novel therapeutic strategies
Treatment consists of lifelong replacement with glucocorticoids and mineralocorticoids. Androgen replacement is seldom administered in women. Standard replacement regimen in PAI involves short-acting GCs, as hydrocortisone or cortisone acetate, usually administered twice or thrice-daily. Optimization of GC replacement therapy is challenging due to the lack of reliable objective biomarkers of GC effects. Available data suggest that many PAI patients may be over-replaced with GCs. Moreover, there is considerable evidence that the current replacement regime fails to reproduce the physiological circadian rhythm of cortisol.
Over-replacement and unphysiological exposure to cortisol have been associated with poor long-term outcomes. Particularly, it has been shown that patients with PAI on current replacement have a significantly reduced health-related quality of life (QoL), irrespective of age, sex, and concomitant disease. QoL seems to be worse in patients receiving hydrocortisone doses higher than 30 mg daily and in those diagnosed with a longer diagnostic delay.
It is widely recognized that patients with PAI have excess morbidity and reduced life expectancy, mainly due to cardiovascular and infectious diseases as well as malignancies. There are still several issues on clinical management of PAI and its associated complications that remain unclear.
The aim of this Research Topic is to promote a better understanding of this disease to improve management and long-term outcomes. Potential authors are encouraged to submit either original research articles or systematic reviews. Authors are also encouraged to analyze clinical outcomes with standard glucocorticoid replacement therapy in patients with primary adrenal insufficiency. Specifically, these issues will be addressed:
• Cognitive function and quality of life
• Cardiovascular comorbidities
• Bone metabolism
• Glucose metabolism
• Insights on novel therapeutic strategies
Keywords: Primary Adrenal Insufficiency, Glucocorticoid replacement, Quality of life, Morbidity, Mortality
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