ADDISONIAN CRISIS

By- Prem Mohan JhaJr3 (medicine)

INTRODUCTION Life-threatening emergency Triggered by anything that increases the

person’s normal stress level The body is unable to release sufficient cortisol

to respond appropriately May lead to shock & vascular collapse

Typically resistant to catecholamine and IVF resuscitation

ADRENAL FUNCTION SUMMARYThe Adrenal Gland releases… Androgens (sex

hormones)Mineralocorticoids

Glucocorticoids

Glucocortoicoids(most potent is cortisol)

Action: anti-inflammatory, growth suppressing,

affects sleep patterns & awareness, stress response

Mineralcorticoids(Aldosterone)

Action: Managessodium/potassium balance

Internet.

NORMAL REGULATION OF ADRENAL GLUCOCORTICOID SECRETION

Kronenberg - Williams Textbook of Endocrinology 11th ed

CLINICAL AND LABORATORY FEATURES OF ADRENAL CRISIS

Dehydration, hypotension, or shock out of proportion to severity of current illness

Nausea and vomiting with a history of weight lost and anorexia

Abdominal pain, so-called acute abdomen Unexplained hypoglycemia Unexplained fever Hyponatremia, hyperkalemia, azotemia, hypercalcemia, or

eosinophilia Hyperpigmentation or vitiligo Other autoimmune endocrine deficiencies, such as

hypothyroidism or gonadal failure Kronenberg - Williams Textbook of Endocrinology 11th ed

CAUSES Abrupt adrenal failure usually from

Bilateral adrenal infarction Bilateral adrenal Hemorrhage

Primary Adrenal Insufficiency Serious infection Acute stress in previously undiagnosed cases No extra glucocorticoid therapy during infection

Secondary adrenocortical insufficiency -Abrupt withdrawal from glucocorticoids

Catastrophic HPA axis failure Head trauma Hemorrhage of pituitary adenoma Post-partum herniation (Sheehan syndrome)

Kronenberg - Williams Textbook of Endocrinology 11th ed

DIAGNOSIS

Immediate Laboratory Work up Electrolytes, glucose Baseline Cortisol and ACTH

Send immediately (before steroid administration) Send in early morning sample if pt is stable with

suspected chronic adrenal insufficiency Can measure urinary 17-OHCS

Renin(+/- aldosterone- less sensitive)Random Cortisol

Kronenberg - Williams Textbook of Endocrinology 11th ed

ACTH STIMULATION TESTS

Cosyntropin(synthetic ACTH) stimulation test is (used in all patients in whom adrenal insufficiency is being considered Cortisol levels measured at 0 and 30 minutes following

cosyntropin administration Normal response is defined by a peak plasma cortisol of

> 20mcg/dl. Primary adrenal insufficiency has a low or no rise in

cortisol following ACTH stimulation Secondary or Tertiary causes due to deficient

endogenous ACTH have an increase in cortisol (sub sub-normal) following ACTH stimulation.

Kronenberg - Williams Textbook of Endocrinology 11th ed

OTHER DIAGNOSTIC TESTS Imaging:

Abdominal CT to evaluate for adrenal findings (ie. Infection, calcification, hemorrhage)

Head CT or MRI if secondary AI is diagnosed Other Labs:

Anti-adrenal antibodies VLCFA (very long chain fatty acids)(acids) Especially in young males with negative antibodies

Evaluate for other autoimmune disorders as indicated PPD if TB is suspected Metyrapone test: : ↓↓cortisol synthesis, should see

a resultant ↑↑in ACTH. Used to diagnosis partial ACTHKronenberg - Williams Textbook of Endocrinology 11th ed

MANAGEMENT OF ADRENAL CRISIS EMERGENCY MEASURES1) Establish intravenous access with a large-gauge needle2) Draw blood for stat serum electrolytes and glucose and

routine measurement of plasma cortisol and ACTH. Do not wait for laboratory results.

3) Infuse 2 to 3 L of 0.9% saline solution or 5% dextrose in 0.9% saline solution as quickly as possible. Monitor for signs of fluid overload by measuring central or peripheral venous pressure and listening for pulmonary rales. Reduce infusion rate if indicated.

4) Inject intravenous hydrocortisone (100 mg immediately and every 6 hr)

5) Use supportive measures as needed. Kronenberg - Williams Textbook of Endocrinology 11th ed

SUBACUTE MEASURES AFTER STABILIZATION OF THE PATIENT

Continue iv 0.9% saline solution at a slower rate for next 24 to 48 hr

Search for and treat possible infectious precipitating causes of the adrenal crisis

Perform a short ACTH stimulation test to confirm the diagnosis of adrenal insufficiency, if he is not a known case .

Determine the type of adrenal insufficiency and its cause if not already known.

Taper glucocorticoids to maintenance dosage over 1 to 3 days, if precipitating or complicating illness permits.

Begin mineralocorticoid replacement with fludrocortisone (0.1 mg by mouth daily) when saline infusion is stopped.

Kronenberg - Williams Textbook of Endocrinology 11th ed

CHRONIC TREATMENT

Glucocorticoid replacement Dexamethasone or prednisone (longer-acting or once daily acting)

daily Alternative therapy with hydrocortisone BID -TID

Mineralocorticoid replacement Fludrocortisone Liberal salt intake

Patient Education Recognition and treatment of minor and major stress/ illness Instructions to triple the dose of steroid in the event of an

intercurrent illness, accident or mental stress Emergency precautions

Medic-alert bracelet, pre alert pre--filled dexamethasone syringesKronenberg - Williams Textbook of Endocrinology 11th ed

ADDISONIAN CRISIS AND TUBERCULOSIS

Tuberculosis is known to affect adrenal glands directly. Adrenal destruction by tuberculosis may lead to overt

or subclinical adrenal insufficiency In India it is the most common cause of Addison’s disease . CT abdomen shows typically shrunken and calcified

adrenals in chronic stage and enlarged in the active stage. Confirmed by FNAC

ATT increase the degratdation of corticosteroids, may precipitate the adrenal crisis & has been reported with rifampicin therapy

INT. J. DIAB. DEV. COUNTRIES (1999), VOL. 19

TAKE HOME MESSAGE

“Unexplained hyponatremia and hyperkalemia in the setting of hypotension unresponsive to catecholamine and fluid administration… should receive 100mg hydrocortisone intravenously.”

Prevention through careful titration of steroids Patient, family, friends aware of signs/symptoms of

crisis

Thank You