Addison's disease

Question 1

Addison’s Disease (Primary Adrenal Insufficiency)
Definition:

Answer 1

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

Question 2

Causes of Addison’s Disease

Answer 2

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

Question 3

Clinical Presentation of Addison’s Crisis

Answer 3

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

Question 4

Emergency Management of Addison’s Crisis

Answer 4

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

Question 5

Symptoms Indicative of Addison’s Disease

Answer 5

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

Question 6

Diagnostic Tests for Addison’s Disease

Answer 6

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

Question 7

Treatment of Addison’s Disease

Answer 7

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

Question 8

Management Strategies for Addison’s Disease

Answer 8

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

Question 9

Differentiating Causes of Adrenal Insufficiency

Answer 9

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

Question 10

Causes of Adrenal Insufficiency

Answer 10

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

Question 11

Pathophysiology of Addison’s Disease

Answer 11

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

Question 12

Causes of Secondary Adrenal Insufficiency

Answer 12

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

Question 13

Secondary Adrenal Insufficiency
Occurs when

Answer 13

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

Question 14

Causes of Tertiary Adrenal Insufficiency

Answer 14

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

Question 15

Tertiary Adrenal Insufficiency
Results from

Answer 15

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

Question 16

Common Causes of Addison’s Disease in Western Countries

Answer 16

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.

Question 17

Other Causes of Primary Adrenal Insufficiency

Answer 17

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

Question 18

Tuberculosis as a Cause of Primary Adrenal Insufficiency

Answer 18

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

Question 19

Less Common Causes of Primary Adrenal Insufficiency

Answer 19

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

Question 20

Conditions associated with Addison’s disease include

Answer 20

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

Question 21

Adrenal Crisis in Addison’s Disease

Answer 21

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.

Question 22

Causes of Adrenal Crisis in Addison’s Disease

Answer 22

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

Question 23

Complications of Addison’s Disease

Answer 23

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

Question 24

Reduced Quality of Life in Addison’s Disease

Answer 24

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

Question 25

Adverse Maternal and Neonatal Outcomes in Addison’s Disease

Answer 25

Poorly managed Addison’s disease during pregnancy associated with increased risk of maternal mortality, miscarriage, preterm delivery, impaired fetal growth, and congenital abnormalities.

Question 26

Premature Ovarian Insufficiency (POI) in Addison’s Disease

Answer 26

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).