Addison's disease Flashcards

1
Q

Addison’s Disease (Primary Adrenal Insufficiency)
Definition:

A

Caused by destruction of adrenal cortex, leading to reduced production of glucocorticoids (Cortisol), mineralocorticoids, and adrenal androgens.

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2
Q

Causes of Addison’s Disease

A

Autoimmunity (90% in western countries), long-term corticosteroid use, congenital adrenal hyperplasia, infections like tuberculosis.

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3
Q

Clinical Presentation of Addison’s Crisis

A

Sudden onset precipitated by illness or stress, featuring hypotension, hypovolemic shock, abdominal pain, vomiting, reduced consciousness.

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4
Q

Emergency Management of Addison’s Crisis

A

Immediate hydrocortisone administration, transfer to hospital, never delay for investigations.

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5
Q

Symptoms Indicative of Addison’s Disease

A

Fatigue, hyperpigmentation, GI symptoms, salt craving, postural hypotension, muscle weakness, cramps.

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6
Q

Diagnostic Tests for Addison’s Disease

A

Serum cortisol <100 nmol/L indicates likely admission, 100-500 nmol/L warrants endocrinology referral. ACTH stimulation test confirms diagnosis.

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7
Q

Treatment of Addison’s Disease

A

Initiated by endocrinologist, lifelong therapy essential. Hydrocortisone for glucocorticoid, fludrocortisone for mineralocorticoid replacement.

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8
Q

Management Strategies for Addison’s Disease

A

Increase steroid coverage during stress, recognize adrenal crisis symptoms, carry steroid treatment card and MedicAlert identification

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9
Q

Differentiating Causes of Adrenal Insufficiency

A

While Addison’s disease results from adrenal cortex destruction, other causes include prolonged corticosteroid use or hypothalamus/pituitary gland disorders.

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10
Q

Causes of Adrenal Insufficiency

A

Long-term corticosteroid use or disorders of the hypothalamus or pituitary gland can cause adrenal insufficiency, distinct from Addison’s disease.

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11
Q

Pathophysiology of Addison’s Disease

A

Destruction of adrenal cortex reduces cortisol, aldosterone, and adrenal androgen production, leading to increased ACTH due to reduced negative feedback

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12
Q

Causes of Secondary Adrenal Insufficiency

A

Insufficient pituitary ACTH production, leading to reduced cortisol and adrenal atrophy. Causes include pituitary tumors, subarachnoid hemorrhage, traumatic brain injury.

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13
Q

Secondary Adrenal Insufficiency
Occurs when

A

pituitary ACTH production is insufficient due to intracranial disorders, leading to decreased cortisol production and adrenal atrophy

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14
Q

Causes of Tertiary Adrenal Insufficiency

A

Disruption of hypothalamic corticotropin-releasing hormone production, leading to insufficient ACTH from the anterior pituitary. Causes include long-term corticosteroid use, tumors, radiotherapy, or surgery affecting the hypothalamus

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15
Q

Tertiary Adrenal Insufficiency
Results from

A

disrupted hypothalamic CRH production, leading to insufficient ACTH, commonly due to long-term corticosteroid use or hypothalamic tumors

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16
Q

Common Causes of Addison’s Disease in Western Countries

A

Autoimmunity accounts for about 90% of cases, with approximately 40% affecting the adrenal gland alone and 60% occurring as part of multi-organ autoimmune polyendocrine syndrome.

17
Q

Other Causes of Primary Adrenal Insufficiency

A

congenital adrenal hyperplasia (most common in children), genetic conditions like adrenoleukodystrophy, infections such as tuberculosis, meningococcus, haemophilus influenzae, cryptococcosis, CMV, and HIV.

18
Q

Tuberculosis as a Cause of Primary Adrenal Insufficiency

A

Significant in endemic regions, contributing to adrenal insufficiency due to infection-related destruction of the adrenal gland.

19
Q

Less Common Causes of Primary Adrenal Insufficiency

A

adrenal metastases, amyloidosis, hemochromatosis, iatrogenic factors like bilateral adrenalectomy, adrenal hemorrhage, and use of immune checkpoint inhibitors in cancer therapy.

20
Q

Conditions associated with Addison’s disease include

A

autoimmune thyroid disease, pernicious anaemia, vitiligo, and type 1 diabetes mellitus.

21
Q

Adrenal Crisis in Addison’s Disease

A

Severe complication occurring during physical stress when the adrenal glands fail to produce enough cortisol, leading to life-threatening symptoms like dehydration, hypotension, shock, altered consciousness, seizures, stroke, or cardiac arrest.

22
Q

Causes of Adrenal Crisis in Addison’s Disease

A

Include gastrointestinal illness (23%), other infections (25%), perioperative period (10%), and physiological stress/pain (9%).

23
Q

Complications of Addison’s Disease

A

Include reduced quality of life, adverse maternal and neonatal outcomes, and premature ovarian insufficiency (POI).

24
Q

Reduced Quality of Life in Addison’s Disease

A

Factors include fatigue, loss of energy, depression, anxiety, reduced ability to cope with daily activities, and loss of libido, especially in women.

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Adverse Maternal and Neonatal Outcomes in Addison's Disease
Poorly managed Addison's disease during pregnancy associated with increased risk of maternal mortality, miscarriage, preterm delivery, impaired fetal growth, and congenital abnormalities.
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Premature Ovarian Insufficiency (POI) in Addison's Disease
10-20% of women with autoimmune Addison's disease develop POI before age 40, with higher risk in autoimmune polyendocrine syndrome type 1 (up to 50-70% in some studies).
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