Addison's Disease: Chronic Primary Adrenocortical Insufficiency
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Addison's Disease: Chronic Primary Adrenocortical Insufficiency

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Questions and Answers

What is the primary cause of Addison's disease in developing countries?

  • Hypocortisolism
  • Autoimmune reaction
  • Tuberculosis (correct)
  • Adrenocortical hypofunction
  • What is the term used to describe the destruction or dysfunction of adrenal cortical tissue?

  • Hypocortisolism
  • Chronic primary adrenocortical insufficiency (correct)
  • Adrenocortical hypofunction
  • Adrenal insufficiency
  • 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?

  • Primary adrenocortical insufficiency
  • Acute adrenal insufficiency
  • Adrenal crisis
  • Secondary adrenocortical insufficiency (correct)
  • What is the leading cause of Addison's disease in developed countries, including the US?

    <p>Autoimmune reaction</p> Signup and view all the answers

    What is the term used to describe the life-threatening complication of inadequately treated Addison's disease?

    <p>Acute addisonian crisis</p> Signup and view all the answers

    What is the significance of Addison's disease?

    <p>It is a hormonal disorder that can be life-threatening if not treated</p> Signup and view all the answers

    Who provided the first detailed clinical description of CPAI?

    <p>Thomas Addison</p> 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?

    <p>Limited ability</p> Signup and view all the answers

    What is a characteristic of Addisonian crisis?

    <p>Hypotension, hypoglycemia, and hyperkalemia</p> Signup and view all the answers

    What is the most common cause of Addison's disease (AD)?

    <p>Autoimmune adrenal insufficiency</p> Signup and view all the answers

    What is a possible cause of chronic fatigue in patients with AD?

    <p>Epinephrine deficiency</p> Signup and view all the answers

    What is a characteristic of autoimmune AD?

    <p>Destruction of the adrenal cortex</p> Signup and view all the answers

    What is a complication of adrenoleukodystrophy?

    <p>Cognitive impairment</p> Signup and view all the answers

    What is a type of autoimmune disease that can occur in patients with AD?

    <p>Polyglandular autoimmune disease</p> Signup and view all the answers

    What is a feature of localized autoimmune adrenal insufficiency?

    <p>Destruction of only the adrenal cortex</p> Signup and view all the answers

    What is the approximate percentage of cases of AD caused by tuberculosis in developed countries?

    <p>10%</p> Signup and view all the answers

    What is the typical age range for Addison disease to develop in PGAD I patients?

    <p>By 15 years</p> Signup and view all the answers

    Which of the following is NOT a complication of PGAD I?

    <p>Rheumatoid arthritis</p> Signup and view all the answers

    What is the mode of inheritance of PGAD I?

    <p>Autosomal recessive</p> Signup and view all the answers

    What is the typical presentation of PGAD II?

    <p>In adulthood with autoimmune disorders</p> Signup and view all the answers

    Which of the following is associated with PGAD II?

    <p>Hashimoto thyroiditis</p> Signup and view all the answers

    What is the underlying abnormality in PGAD I?

    <p>T cell-mediated immunity</p> Signup and view all the answers

    Which of the following is NOT a feature of PGAD I?

    <p>Hyperthyroidism</p> Signup and view all the answers

    What is the typical complication of PGAD I that affects the stomach?

    <p>Pernicious anemia</p> Signup and view all the answers

    What is the primary cause of Addison disease?

    <p>Underproduction of cortisol by the adrenal glands</p> Signup and view all the answers

    At what percentage of total adrenocortical tissue dysfunction does the onset of Addison disease typically occur?

    <p>90%</p> Signup and view all the answers

    What is the function of corticotropin-releasing hormone (CRH) in the HPA axis?

    <p>Acting as a chemical messenger between the hypothalamus and the pituitary gland</p> Signup and view all the answers

    Which of the following hormones is responsible for stimulating cortisol release from the adrenal glands?

    <p>Adrenocorticotropic hormone (ACTH)</p> Signup and view all the answers

    What is the role of cortisol in the body's physiological response to stress?

    <p>Contributing to the body's physiological response to stress</p> Signup and view all the answers

    What is the most common and specific antibody associated with autoimmune Addison disease?

    <p>Antibodies to steroid 21-hydroxylase</p> Signup and view all the answers

    What is the consequence of a deficiency of functional suppressor T lymphocytes in autoimmune Addison disease?

    <p>Proliferation of plasma cells that produce immunoglobulins against cells within the adrenal cortex</p> Signup and view all the answers

    What is the significance of detecting antibodies to steroid 21-hydroxylase in patients without symptomatic Addison disease?

    <p>It indicates a high risk of developing adrenal insufficiency</p> Signup and view all the answers

    What percentage of patients with AD experience an addisonian crisis when they first see a health professional?

    <p>25%</p> Signup and view all the answers

    What is the percentage of patients with CPAI who exhibit hyperpigmentation of the skin and mucous membranes?

    <p>90%</p> Signup and view all the answers

    What is the typical duration of time that hyperpigmentation precedes other clinical manifestations of AD?

    <p>Months to years</p> 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?

    <p>5-10 µg/dL</p> 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?

    <p>Rapid ACTH stimulation test</p> Signup and view all the answers

    What is the dose of synthetic ACTH (cosyntropin) used in the rapid ACTH stimulation test?

    <p>0.25 mg</p> 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?

    <p>Two to five times</p> 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?

    <p>30-60 minutes</p> 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.

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    Description

    Learn about Addison's disease, a hormonal disorder caused by adrenal cortical tissue dysfunction or destruction, leading to diminished glucocorticoid and mineralocorticoid production.

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