Rapid Improvement of Adrenal Function in a Subject with Acute Adrenal Insufficiency after Bilateral Adrenal Hemorrhage as Suggested by a Repeated ACTH-Stimulation Test

Introduction:
Antiphospholipid syndrome (APLS) is a prothrombotic autoimmune disorder that can be managed with anticoagulation (AC) for primary thrombosis prevention. Adrenal gland involvement is rare, but both APLS and AC can cause adrenal hemorrhage. Adrenal insufficiency (AI) due to bilateral adrenal hemorrhage is uncommon and recovery of adrenal function is not typically observed.

Case Description:
52 y/o woman presented to the hospital with abdominal pain worsening over 3 days associated with vomiting and fullness. Medical history was significant for cerebrovascular accident and APLS controlled with warfarin. Vital signs were normal, but on exam she had abdominal tenderness. Laboratory tests showed hyponatremia. CT abdomen with IV contrast revealed left adrenal hemorrhage. Hematology advised to hold AC until she could follow-up in clinic. Her labs remained stable and she was discharged home.

Two days later she returned to the hospital with abdominal pain and nausea. Abdominal exam was notable for diffuse tenderness. Laboratory tests still demonstrated hyponatremia. CT abdomen with IV contrast showed a left adrenal hemorrhage with new, right adrenal gland hemorrhage. Morning cortisol was 12.4 mcg/dL. Endocrinology recommended ACTH-stimulation test: ACTH 134 (n: 6-50 pg/mL), 60 mins post-stimulation cortisol was close to normal at 17.8 (n: ≥18 mcg/dL). Due to high suspicion for evolving primary AI, physiologic doses of hydrocortisone and fludrocortisone were started. Her nausea resolved and hyponatremia improved.

She followed at the Endocrine clinic feeling well with glucocorticoid and mineralocorticoid replacements. ACTH-stim test was repeated with a normal 60 mins post-stimulation cortisol level (>18 mcg/dL) and ACTH level of 69 pg/mL. Due to concerns of partial AI, it was decided to continue glucocorticoid and mineralocorticoid replacement with plan to repeat ACTH-stim test soon to reassess discontinuation of steroids replacement.

Discussion:
Bilateral adrenal hemorrhage is a rare cause of AI and an uncommon complication of AC. Most frequent clinical presentation is pain in the abdomen or back. Nausea and hypotension may occur. Some laboratory findings that may be present are hyponatremia, hyperkalemia, and hypoglycemia. An ACTH-stim test can help confirm the diagnosis. CT, MRI, and ultrasound are useful in identifying the hemorrhage. Immediate treatment with glucocorticoids should be initiated rather than waiting for laboratory tests in highly suspicious cases. Adrenalectomy or embolization are rarely required to treat hemorrhage. While AI is usually irreversible, adrenocortical function may recover in some cases as suggested by the repeated ACTH-stim test in our patient.