Addisonian Crisis -

Addisonian Crisis

Addisonian crisis is a life-threatening emergency resulting from acute insufficiency of cortisol, with or without aldosterone deficiency.

It can occur in:

Known Addison’s disease (primary adrenal insufficiency)
Pituitary disease (secondary insufficiency)
Critically ill patients with undiagnosed adrenal insufficiency

📍 Key Definition:

“Acute adrenal crisis is the inability of the adrenal glands to meet the increased demand of cortisol during stress (e.g., illness, trauma), leading to shock and metabolic derangements.”

🧬 Pathophysiology:

Hormone Deficiency	Effects
↓ Cortisol	↓ Vascular tone, hypoglycemia, ↑ inflammation
↓ Aldosterone	↓ Na⁺, ↑ K⁺, ↓ intravascular volume → shock
↑ ACTH (if primary)	Skin hyperpigmentation
Inadequate response to stress	Exacerbates illness severity

🔥 Triggers / Precipitating Factors:

Type	Example
Stress	Infection (esp. sepsis), surgery, trauma
Medication non-compliance	Steroid withdrawal
Adrenal destruction	Autoimmune, TB, metastasis
Drugs	Etomidate (inhibits 11-β hydroxylase), rifampicin
Bilateral adrenal hemorrhage	e.g., Waterhouse-Friderichsen syndrome (meningococcemia)

🩺 Clinical Features:

System	Manifestations
CVS	Hypotension, refractory shock, tachycardia
GI	Nausea, vomiting, abdominal pain (mimics surgical abdomen)
CNS	Lethargy, confusion, coma
Skin	Hyperpigmentation (if chronic/primary)
Electrolytes	Hyponatremia, hyperkalemia, hypoglycemia, metabolic acidosis
Other	Hypovolemia, low urine output

🧪 Laboratory Findings:

Test	Expected Result
Serum cortisol	<5 µg/dL in acute illness (critical)
ACTH	↑ (in primary), ↓ or normal (in secondary)
Na⁺	↓
K⁺	↑ (not in secondary AI)
Glucose	↓
Urea/creatinine	↑ (pre-renal AKI from volume depletion)
ABG	Metabolic acidosis ± hypoNa/hypoglycemia

A cosyntropin stimulation test is definitive but not necessary before emergency treatment.

🛌 Diagnosis:

Clinical diagnosis is crucial:

Shock unresponsive to fluids/vasopressors
Hypoglycemia, hyponatremia, hyperkalemia
History of steroid use, autoimmune disease, TB

If time permits:

Draw blood for baseline cortisol and ACTH before giving steroids

🧪 Cortisol and ACTH Sampling Guidelines:

Parameter	Ideal Timing	Fasting?	Notes
Serum Cortisol	8 AM (morning sample)	Yes(preferably)	Cortisol has a diurnal rhythm—peaks in the early morning
Plasma ACTH	Same time as cortisol	Yes(preferably)	Must be sent on ice, in pre-chilled EDTA tube
Random Cortisol (in emergency)	Anytime	No	Acceptable in critically ill or suspected Addisonian crisis

🧑‍⚕️ Management: ABC + Steroids + Supportive care

🟩 1. Hydrocortisone:

100 mg IV bolus, then
50–100 mg IV q6–8h, or continuous infusion
Has glucocorticoid + mineralocorticoid activity

In known secondary AI (e.g., pituitary): dexamethasone 4 mg IV can be used (won’t interfere with cortisol assay)

🟦 2. Fluid Resuscitation:

Rapid IV NS 1–3 L in first 24 hours
If hypoglycemia: add dextrose

🟧 3. Correct Electrolyte Imbalance:

Treat hyperkalemia if severe
Sodium correction via fluids and cortisol

🟥 4. Treat Underlying Cause:

Start empirical antibiotics if infection suspected
Investigate for sepsis, hemorrhage, TB, etc.

🔄 Tapering:

Once stable: taper IV steroids to oral hydrocortisone (15–25 mg/day)
Mineralocorticoid (fludrocortisone 0.1 mg/day) added in primary AI

🧠 Differentiating Types of Adrenal Insufficiency:

Feature	Primary AI (Addison’s)	Secondary AI (Pituitary)
Cortisol	↓↓↓	↓↓↓
Aldosterone	↓↓↓	Normal
ACTH	↑↑↑	↓
Hyperpigmentation	Yes	No
Hyperkalemia	Yes	No

🧾 Key ICU Notes:

Etomidate can cause adrenal suppression in septic patients
In septic shock unresponsive to vasopressors, consider Relative Adrenal Insufficiency (RAI)
Always stress-dose steroids in known AI before surgery or major illness

🧠 Mnemonic – “Addison CRISIS”:

C – Coma, Confusion
R – Refractory hypotension
I – Increased K⁺, low Na⁺
S – Shock
I – Infections (common trigger)
S – Steroid deficiency, Skin pigmentation

Comparison Table: Cushing’s vs Addison’s vs Nelson’s Syndrome

Feature	Cushing’s Syndrome	Addison’s Disease	Nelson’s Syndrome
Definition	Chronic glucocorticoid excess	Primary adrenal insufficiency	ACTH-secreting pituitary tumor (post-adrenalectomy)
Cortisol Level	↑↑	↓↓↓	↓ (due to adrenalectomy)
ACTH Level	↑ (Cushing’s disease) or ↓ (adrenal cause)	↑↑↑ (due to primary adrenal failure)	↑↑↑↑ (pituitary tumor continues secreting)
Aldosterone	Normal/↑ (variable)	↓↓↓ (primary deficiency)	Normal
Electrolytes	↑ Na⁺, ↓ K⁺	↓ Na⁺, ↑ K⁺	Usually normal
Glucose	Hyperglycemia	Hypoglycemia	Normal or mildly ↓
BP	Hypertension	Hypotension	Often normal
Pigmentation	± Present (only if ACTH ↑)	Yes (ACTH ↑↑ stimulates MSH)	Marked hyperpigmentation
Skin Changes	Thin skin, purple striae	Dry skin	Darkening of skin and mucosa
Weight	Weight gain (central obesity)	Weight loss	No major weight change
Facial Features	Moon face, acne, hirsutism	Pale, gaunt face	No specific facial feature
Other Signs	Osteoporosis, proximal myopathy, depression	Fatigue, GI pain, craving for salt	Mass effect from pituitary tumor (headache, visual loss)
Diagnosis	24-h cortisol, dex suppression, ACTH	Low cortisol + high ACTH	High ACTH + MRI pituitary
Etiology	Exogenous steroids, pituitary tumor, adrenal tumor	Autoimmune, TB, metastatic destruction	After bilateral adrenalectomy for Cushing’s
Treatment	Depends on cause; surgery ± meds	Steroid + mineralocorticoid replacement	Pituitary surgery or radiation

🧠 Mnemonics:

CUSHING = CUSH:
Central obesity, Upper body fat, Skin changes, Hyperglycemia
ADDISON = ADD Salt, Sugar, Steroids
NELSON = NEL:
No cortisol (post-adrenalectomy), Enormous ACTH, Local tumor symptoms