Research Topic

Primary Adrenal Insufficiency: Quality of Life and Long-term outcome with Current Treatment Strategies

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


Keywords: Primary Adrenal Insufficiency, Glucocorticoid replacement, Quality of life, Morbidity, Mortality


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

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


Keywords: Primary Adrenal Insufficiency, Glucocorticoid replacement, Quality of life, Morbidity, Mortality


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

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Submission Deadlines

01 December 2020 Abstract
24 March 2021 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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Topic Editors

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Submission Deadlines

01 December 2020 Abstract
24 March 2021 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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