Extra if needed
Diagnosis of PAI initially involves blood assay for elevated ACTH and plasma renin activity (PRA) with decreased serum cortisol and aldosterone, DHEAS [1, 2]. Exclusion of other autoimmune conditions and imaging of the adrenal glands completes diagnostic testing and results [3].
Diagnostic Testing and Results
Optimal levels of serum cortisol and plasma ACTH are simultaneously measured early morning between 8-9am, in healthy individuals the serum cortisol levels can range between 275-555 nmol/L. In individuals with PAI serum cortisol concentrations can be as low as <5 nmol/l would also connote a diagnosis of PAI [3,4].
As many autoimmune conditions can resemble PAI it is essential to exclude these prior to a diagnosis confirmation. …show more content…
Oral
Diagnostic Testing and Results
This rare disease can sometimes be challenging to diagnose due to gradual changes and atypical symptoms. Sixty percent of PAI patients had seen two or more physicians prior to confirmation of the diagnosis [2].
The majority of cases of PAI are generally brought on by autoimmune damage to the adrenal cortex, which can subsequently increase the risk of developing additional autoimmune conditions [1].
There are three steps to diagnosing PAI.
1. Mid-morning blood assay may show elevated plasma adrenocorticotropic hormone ACTH and plasma renin activity PRA with decreased serum cortisol and serum aldosterone and dehydroepiandrosterone sulphate DHEAS [1, 3]. ACTH and cortisol concentrations have a diurnal variation that differs in patients with PAI.
2. Is to exclude secondary conditions that result in adrenal insufficiency such as HIV and tuberculosis [2,3].
3. If autoantibodies are negative, then imaging of the adrenal glands will be performed to rule out adrenoleukodystrophy (ALD) and confirm the diagnosis …show more content…
Some of these conditions include cardiogenic shock, hyperkalemia, hypovolemia, sepsis, exogenous intoxication. We can differently diagnose by examining the patients’ medical history and the existence of associated disorders [3]. If the adrenal gland is enlarged and calcified on an abdominal CT then adrenoleukodystrophy (ALD), a rare genetic metabolic disorder can be ruled out [3].
Treatment
To best manage PAI yearly reviews by an endocrinologist are recommended to look at mineralcorticoid replacement therapy and in order to check for new autoimmune diseases that might have arisen. Patient education surrounding crisis prevention and dose regulation is an important part of management [1].
Serum sodium and potassium, and plasma renin activity concentrationsas well as the patient’s blood pressure are used as basis to calculate the correct dose of fludrocortisone - the mineralocorticoid replacement required to help control symptoms of PAI [4]. Appropriate dosing levels require regular monitoring, particularly in warmer climates where temperatures may exceed 29OC. Fludrocortisone doses, usually administered in the morning, vary between 0.05-.020 mg
Diagnosis of PAI initially involves blood assay for elevated ACTH and plasma renin activity (PRA) with decreased serum cortisol and aldosterone, DHEAS [1, 2]. Exclusion of other autoimmune conditions and imaging of the adrenal glands completes diagnostic testing and results [3].
Diagnostic Testing and Results
Optimal levels of serum cortisol and plasma ACTH are simultaneously measured early morning between 8-9am, in healthy individuals the serum cortisol levels can range between 275-555 nmol/L. In individuals with PAI serum cortisol concentrations can be as low as <5 nmol/l would also connote a diagnosis of PAI [3,4].
As many autoimmune conditions can resemble PAI it is essential to exclude these prior to a diagnosis confirmation. …show more content…
Oral
Diagnostic Testing and Results
This rare disease can sometimes be challenging to diagnose due to gradual changes and atypical symptoms. Sixty percent of PAI patients had seen two or more physicians prior to confirmation of the diagnosis [2].
The majority of cases of PAI are generally brought on by autoimmune damage to the adrenal cortex, which can subsequently increase the risk of developing additional autoimmune conditions [1].
There are three steps to diagnosing PAI.
1. Mid-morning blood assay may show elevated plasma adrenocorticotropic hormone ACTH and plasma renin activity PRA with decreased serum cortisol and serum aldosterone and dehydroepiandrosterone sulphate DHEAS [1, 3]. ACTH and cortisol concentrations have a diurnal variation that differs in patients with PAI.
2. Is to exclude secondary conditions that result in adrenal insufficiency such as HIV and tuberculosis [2,3].
3. If autoantibodies are negative, then imaging of the adrenal glands will be performed to rule out adrenoleukodystrophy (ALD) and confirm the diagnosis …show more content…
Some of these conditions include cardiogenic shock, hyperkalemia, hypovolemia, sepsis, exogenous intoxication. We can differently diagnose by examining the patients’ medical history and the existence of associated disorders [3]. If the adrenal gland is enlarged and calcified on an abdominal CT then adrenoleukodystrophy (ALD), a rare genetic metabolic disorder can be ruled out [3].
Treatment
To best manage PAI yearly reviews by an endocrinologist are recommended to look at mineralcorticoid replacement therapy and in order to check for new autoimmune diseases that might have arisen. Patient education surrounding crisis prevention and dose regulation is an important part of management [1].
Serum sodium and potassium, and plasma renin activity concentrationsas well as the patient’s blood pressure are used as basis to calculate the correct dose of fludrocortisone - the mineralocorticoid replacement required to help control symptoms of PAI [4]. Appropriate dosing levels require regular monitoring, particularly in warmer climates where temperatures may exceed 29OC. Fludrocortisone doses, usually administered in the morning, vary between 0.05-.020 mg