What is the management of an Addisonian crisis, characterized by hyperpigmentation of knuckles and elbows, hypotension, and dehydration?

Management of Addisonian Crisis with Hyperpigmentation, Hypotension, and Dehydration

Immediate treatment of Addisonian crisis requires prompt administration of hydrocortisone 100 mg IV bolus, without delaying for diagnostic procedures, followed by aggressive fluid resuscitation with 0.9% saline. 1

Emergency Management Algorithm

• Administer hydrocortisone 100 mg IV bolus immediately upon suspicion of adrenal crisis 1
• Begin fluid resuscitation with 0.9% saline 1L over the first hour, followed by 3-4L over 24-48 hours with frequent hemodynamic monitoring 1
• Continue glucocorticoid administration with hydrocortisone 100-300 mg/day, either as continuous IV infusion or divided IV/IM boluses every 6 hours 1
• Obtain laboratory assessment including serum cortisol, ACTH, electrolytes, creatinine, urea, and glucose 1
• Consider ICU/high-dependency unit admission, gastric stress ulcer prophylaxis, and antibiotic treatment if infection is suspected 1
• Evaluate for precipitating causes of crisis such as infection, trauma, or surgery 2

Clinical Recognition

• Hyperpigmentation of knuckles and elbows is a classic sign of primary adrenal insufficiency due to elevated ACTH levels 2
• Hypotension and dehydration are cardinal manifestations of adrenal crisis requiring immediate intervention 1
• Other common symptoms include malaise, fatigue, nausea, vomiting, abdominal pain, and muscle pain/cramps 1
• Laboratory findings typically include hyponatremia, hyperkalemia, increased creatinine, and hypoglycemia 1

Transition to Maintenance Therapy

• Taper parenteral glucocorticoids over 1-3 days to oral maintenance therapy once the patient is stabilized 1, 3
• For maintenance therapy, hydrocortisone is the preferred glucocorticoid at a total daily dose of 15-25 mg divided into 2-3 doses 3
• Common dosing schedules include 10 mg in the morning, 5 mg at midday, and 2.5 mg in the afternoon 3
• Add fludrocortisone 0.1 mg daily (range 0.05-0.2 mg) for mineralocorticoid replacement in primary adrenal insufficiency 4

Prevention of Future Crises

• Provide comprehensive patient education on stress dosing during illness or stress 2, 3
• Instruct patients to double or triple their glucocorticoid dose during minor illness and to use parenteral hydrocortisone during severe illness 1, 3
• Ensure patients have emergency supplies including injectable hydrocortisone 1
• Recommend medical identification such as medical alert jewelry and emergency steroid card 2, 1
• Educate patients, family members, and primary care providers on recognition and management of adrenal crisis 5

Common Pitfalls and Caveats

• Delaying treatment while waiting for diagnostic confirmation can be fatal - treat first, diagnose later 1, 6
• Insufficient dose adjustment during intercurrent illness is a common cause of preventable mortality 5, 6
• Inadequate duration of stress dosing can lead to recurrence of crisis, as seen in cases where hydrocortisone was reduced too quickly 7
• Patients often need to advocate for themselves in emergency settings - proper medical identification and education can help overcome this barrier 2
• Monitoring should continue even after apparent stabilization, as electrolyte abnormalities may persist, particularly hyponatremia 8