What is the recommended treatment for a patient with lymphocytosis, high IgA, abdominal pain, bone pain, tachycardia, erythema in the atrium, adrenal insufficiency, and labile TSH levels?

Immediate Diagnostic and Treatment Approach

This patient requires urgent endocrine evaluation for suspected hypophysitis with multiple pituitary hormone deficiencies, and immediate initiation of stress-dose hydrocortisone must precede any thyroid hormone replacement to prevent precipitating adrenal crisis. 1

Critical First Steps

Immediate Laboratory Assessment

• Draw morning (8 AM) ACTH, cortisol, free T4, LH, FSH, and electrolytes before initiating any treatment 1, 2
• The combination of consistently low cortisol with TSH of 82 suggests central adrenal insufficiency with concurrent primary hypothyroidism, or more likely hypophysitis affecting multiple pituitary axes 1
• Order MRI of the brain with pituitary/sellar cuts immediately given multiple endocrine abnormalities, as this is diagnostic for hypophysitis 1

Distinguishing Primary vs. Secondary Adrenal Insufficiency

• If ACTH is low with low cortisol, this confirms secondary (central) adrenal insufficiency from hypophysitis 1, 2
• If ACTH is high with low cortisol, this indicates primary adrenal insufficiency—consider adrenal lymphoma given the lymphocytosis, high IgA, and bone pain 3
• The labile TSH reaching 82 suggests primary thyroid failure rather than central hypothyroidism, which would show low or normal TSH with low free T4 1

Treatment Algorithm Based on Symptom Severity

For Moderate Symptoms (Abdominal Pain, Bone Pain, Tachycardia Present)

Start hydrocortisone 10-20 mg orally in the morning and 5-10 mg in early afternoon immediately 1

• Do not wait for confirmatory testing if clinical suspicion is high and patient is symptomatic 1, 2
• Critically important: Start corticosteroids several days before initiating levothyroxine to prevent precipitating adrenal crisis 1
• After 3-5 days of hydrocortisone, begin levothyroxine based on weight (typically 1.6 mcg/kg/day) 1

If Severe Symptoms Develop (Hypotension, Altered Mental Status, Vomiting)

Administer IV hydrocortisone 100 mg bolus immediately, followed by 100 mg IV every 6 hours 1

• Infuse 0.9% normal saline at 1 L/hour initially, then continue at slower rate for 24-48 hours 1, 2
• Never delay treatment for diagnostic procedures if adrenal crisis is suspected 1, 2, 4
• If diagnosis uncertain and you need to perform ACTH stimulation testing later, use dexamethasone 4 mg IV instead of hydrocortisone 2

Addressing the Underlying Diagnosis

Hypophysitis Workup

• The combination of low cortisol, extremely elevated TSH (suggesting concurrent autoimmune thyroiditis), and systemic symptoms points toward autoimmune hypophysitis or infiltrative disease 1
• Obtain endocrinology consultation urgently for management of multiple hormone deficiencies 1
• Consider checking anti-pituitary antibodies if available 1

Lymphoma Consideration

The constellation of lymphocytosis, high IgA, bone pain, and adrenal insufficiency raises concern for adrenal lymphoma 3

• If ACTH is elevated (indicating primary adrenal insufficiency), obtain CT abdomen to evaluate adrenal glands for masses 2, 3
• Primary adrenal lymphoma can present with bilateral adrenal masses causing acute adrenal insufficiency 3
• Consider hematology consultation and bone marrow biopsy if lymphoproliferative disorder suspected 3

Gastric Findings

• The erythema in the gastric atrium may represent autoimmune gastritis, which commonly coexists with autoimmune adrenal insufficiency 2
• Check anti-parietal cell antibodies, vitamin B12, and methylmalonic acid levels 2

Maintenance Therapy After Stabilization

Glucocorticoid Replacement

• Hydrocortisone 15-25 mg daily in divided doses (typically 10-20 mg morning, 5-10 mg early afternoon) 1, 5, 6
• If primary adrenal insufficiency confirmed, add fludrocortisone 0.05-0.2 mg daily for mineralocorticoid replacement 5, 6
• Titrate fludrocortisone to maintain normotension, normokalemia, and plasma renin in upper normal range 5

Thyroid Hormone Replacement

• Monitor free T4 for dose titration, not TSH, as TSH is unreliable in central hypothyroidism 1
• If both primary hypothyroidism and adrenal insufficiency present, ensure adequate corticosteroid coverage before starting levothyroxine 1

Critical Patient Education and Safety Measures

Stress Dosing Protocol

• All patients must receive education on doubling glucocorticoid doses during illness, fever, or stress 1, 4
• Provide injectable hydrocortisone 100 mg for home use in case of vomiting or inability to take oral medications 4
• Issue medical alert bracelet stating "adrenal insufficiency" to trigger stress-dose corticosteroids by emergency medical services 1

Surgical/Procedural Planning

• Endocrine consultation required before any surgery or invasive procedure for stress-dose planning 1
• Major surgery requires 100 mg hydrocortisone IM before anesthesia, then 100 mg IV every 6 hours until able to take oral medications 1

Common Pitfalls to Avoid

• Never start thyroid hormone replacement before ensuring adequate glucocorticoid coverage—this can precipitate life-threatening adrenal crisis 1
• Do not rely on presence of hyperkalemia to diagnose adrenal insufficiency—it occurs in only 50% of cases 2, 4
• Do not delay treatment waiting for ACTH stimulation test results if patient is clinically unstable 1, 2, 4
• Recognize that patients on corticosteroids for other conditions will have suppressed cortisol from iatrogenic secondary adrenal insufficiency 1