SAT-428 - Adrenal Crisis in APS-1 During Acute Pancreatitis: A Sepsis Mimic

Background:
Autoimmune polyglandular syndrome type 1 (APS-1) can present with overlapping endocrine and metabolic emergencies, complicating early recognition of adrenal crisis. Diagnostic uncertainty is heightened when acute pancreatitis, shock, and ketoacidosis coexist and mimic sepsis.

Clinical

Case:
A 27-year-old man with known APS-1 manifested by primary adrenal insufficiency and hypoparathyroidism, with concomitant type 1 diabetes mellitus and autoimmune hypothyroidism, presented after one day of emesis and was minimally responsive on arrival. He was febrile to 39.3°C, tachycardic, hypoxemic, and profoundly hypotensive, requiring intubation, norepinephrine, and vasopressin. Initial studies showed sodium 129 mmol/L (135-145), pH 7.10 (7.35-7.45), bicarbonate 13 mmol/L (22-29), anion gap 29 (7-17), lactate 15.9 mmol/L (0.5-2.0), glucose 197 mg/dL (70-99), beta-hydroxybutyrate 4.1 mmol/L ( < 0.4), and lipase 4487 U/L (13-60). CT abdomen/pelvis showed acute interstitial pancreatitis without biliary obstruction or walled-off collection. Empiric broad-spectrum antimicrobials were started, but blood, urine, and cerebrospinal fluid cultures remained negative.

He was treated with stress-dose hydrocortisone (100 mg intravenously then 50 mg every 6 hours), aggressive intravenous fluids, vasopressors, and an insulin infusion for glycemic control during steroid therapy. Point-of-care glucose later peaked at 447 mg/dL after treatment initiation. Endocrinology was consulted and favored adrenal crisis precipitated by acute pancreatitis. Given recent alcohol intake and markedly elevated phosphatidylethanol (PEth) 16:0/18:1 of 1894 ng/mL during hospitalization, endocrinology and gastroenterology favored alcohol-associated pancreatitis. Ketoacidosis was considered multifactorial rather than isolated classic diabetic ketoacidosis, with contributions from recent alcohol use, poor intake, severe lactic acidosis, stress, and glucocorticoid exposure. Vasopressors were discontinued by hospital day 4, he was extubated on day 5, and he was discharged on hydrocortisone, fludrocortisone, insulin, levothyroxine, and calcitriol.

Clinical Lessons:
In patients with APS-1, acute pancreatitis may precipitate adrenal crisis and present as a sepsis mimic. Appropriate level of care is essential when multiple endocrine emergencies coexist, allowing timely vasopressor support, stress-dose glucocorticoids, and insulin infusion to control steroid-associated hyperglycemia and mitigate risk of progression to diabetic ketoacidosis. Prompt empiric stress-dose hydrocortisone is essential in known or suspected primary adrenal insufficiency while infectious evaluation is ongoing.

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