Questions and Answers
What is the primary cause of Addison's disease in developing countries?
What is the term used to describe the destruction or dysfunction of adrenal cortical tissue?
What is the term used to describe the failure of the adrenal glands to produce hormones, resulting from injury or surgery to the hypothalamus or pituitary gland?
What is the leading cause of Addison's disease in developed countries, including the US?
Signup and view all the answers
What is the term used to describe the life-threatening complication of inadequately treated Addison's disease?
Signup and view all the answers
What is the significance of Addison's disease?
Signup and view all the answers
Who provided the first detailed clinical description of CPAI?
Signup and view all the answers
What is the common term used to describe the ability of patients with Addison's disease to respond to stress?
Signup and view all the answers
What is a characteristic of Addisonian crisis?
Signup and view all the answers
What is the most common cause of Addison's disease (AD)?
Signup and view all the answers
What is a possible cause of chronic fatigue in patients with AD?
Signup and view all the answers
What is a characteristic of autoimmune AD?
Signup and view all the answers
What is a complication of adrenoleukodystrophy?
Signup and view all the answers
What is a type of autoimmune disease that can occur in patients with AD?
Signup and view all the answers
What is a feature of localized autoimmune adrenal insufficiency?
Signup and view all the answers
What is the approximate percentage of cases of AD caused by tuberculosis in developed countries?
Signup and view all the answers
What is the typical age range for Addison disease to develop in PGAD I patients?
Signup and view all the answers
Which of the following is NOT a complication of PGAD I?
Signup and view all the answers
What is the mode of inheritance of PGAD I?
Signup and view all the answers
What is the typical presentation of PGAD II?
Signup and view all the answers
Which of the following is associated with PGAD II?
Signup and view all the answers
What is the underlying abnormality in PGAD I?
Signup and view all the answers
Which of the following is NOT a feature of PGAD I?
Signup and view all the answers
What is the typical complication of PGAD I that affects the stomach?
Signup and view all the answers
What is the primary cause of Addison disease?
Signup and view all the answers
At what percentage of total adrenocortical tissue dysfunction does the onset of Addison disease typically occur?
Signup and view all the answers
What is the function of corticotropin-releasing hormone (CRH) in the HPA axis?
Signup and view all the answers
Which of the following hormones is responsible for stimulating cortisol release from the adrenal glands?
Signup and view all the answers
What is the role of cortisol in the body's physiological response to stress?
Signup and view all the answers
What is the most common and specific antibody associated with autoimmune Addison disease?
Signup and view all the answers
What is the consequence of a deficiency of functional suppressor T lymphocytes in autoimmune Addison disease?
Signup and view all the answers
What is the significance of detecting antibodies to steroid 21-hydroxylase in patients without symptomatic Addison disease?
Signup and view all the answers
What percentage of patients with AD experience an addisonian crisis when they first see a health professional?
Signup and view all the answers
What is the percentage of patients with CPAI who exhibit hyperpigmentation of the skin and mucous membranes?
Signup and view all the answers
What is the typical duration of time that hyperpigmentation precedes other clinical manifestations of AD?
Signup and view all the answers
What is the normal range of cortisol levels in the morning, when baseline cortisol levels are normally highest each day?
Signup and view all the answers
What is the primary test used to diagnose AD, which assesses the functional capacity of the adrenal cortex to synthesize cortisol?
Signup and view all the answers
What is the dose of synthetic ACTH (cosyntropin) used in the rapid ACTH stimulation test?
Signup and view all the answers
How many times does a normal adrenal cortex release its basal plasma cortisol output within 15-30 minutes of ACTH treatment?
Signup and view all the answers
At what time interval after cosyntropin administration are two more blood samples drawn for cortisol assay and aldosterone determination?
Signup and view all the answers
Study Notes
Addison's Disease
- Also known as chronic primary adrenocortical insufficiency (CPAI), a potentially life-threatening neuroendocrine or hormonal disorder and chronic metabolic condition.
- Caused by destruction or dysfunction of adrenal cortical tissue.
- Characterized by diminished glucocorticoid (cortisol) and, less commonly, mineralocorticoid (aldosterone) and adrenal androgen function.
History
- First detailed clinical description by Thomas Addison in 1855.
- Before 1950, CPAI usually resulted from an infection of the adrenal glands, with tuberculosis (TB) being the leading cause.
- In developed countries, non-specific destruction of the adrenal glands by cells of the immune system (autoimmune reaction) is the most common cause.
Causes and Risk Factors
- Insults to the adrenal glands that cause AD are multiple.
- 80% of cases are caused by gradual destruction of the outer layer of the adrenal gland (adrenal cortex) by the body's immune system, referred to as autoimmune AD.
- Other causes include:
- Tuberculosis (10% of cases in developed countries)
- Adrenoleukodystrophy (X-linked recessive disorder)
- Hypogonadism (small gonads resulting in ovarian or testicular failure)
- Polyglandular autoimmune disease (PGAD)
Pathophysiology
- The HPA axis regulates the secretion of cortisol from the adrenal glands in response to stress.
- Corticotropin-releasing hormone (CRH) stimulates adrenocorticotropic hormone (ACTH) release, which stimulates cortisol release.
- Addison disease specific antibodies are responsible for the autoimmune destruction of the adrenal cortex.
Diagnosis
- Hyperpigmentation of the skin and mucous membranes is a clinical hallmark of AD, present in 90% of patients.
- Low plasma cortisol (< 3 µg/dL) between 6-8 A.M. strongly suggests AD.
- Definitive diagnosis rests on the assessment of the functional capacity of the adrenal cortex to synthesize cortisol using the rapid ACTH stimulation test.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Description
Learn about Addison's disease, a hormonal disorder caused by adrenal cortical tissue dysfunction or destruction, leading to diminished glucocorticoid and mineralocorticoid production.