(1687) A Tale of Two Shocks: Hypotension due to Cardiac Tamponade in Autoimmune Polyglandular Syndrome-2

Introduction: Autoimmune polyglandular syndrome-2 (APS-2) involves two of the following: primary adrenal insufficiency, thyroid dysfunction, and type 1 diabetes. Cardiac tamponade is a rare complication of APS-2, with only half of the cases presenting with shock. Differentiating between cardiogenic and distributive shock in APS-2 patients is challenging.

Description: A 30-year-old female with APS-2, including hypothyroidism and primary adrenal insufficiency, on hormone replacement, was brought to the hospital with decreased alertness and lethargy. Vitals were 66/40 mmHg, 121 bpm, 22 breaths/min, and 95% on room air. Workup showed leukocytosis to 13.27 X 109 /L, acute kidney injury to 1.8 mg/dL, metabolic acidosis to 14 mmol/L, elevated troponin of 0.068 ng/mL, lactic acid of 8.2 mmol/L, and procalcitonin of 57.2 ng/mL. An electrocardiogram showed sinus tachycardia and low-voltage QRS. A bedside transthoracic echocardiogram (TTE) revealed a pericardial effusion with cardiac tamponade. She underwent emergent pericardiocentesis, draining 80 ml of fluid, but remained in refractory shock requiring norepinephrine, epinephrine, and dobutamine. In the intensive care unit, weaning down the vasopressors was difficult during the initial days. The cause of her shock—whether cardiogenic from tamponade or distributive from adrenal insufficiency—was unclear. A complete TTE did not show any remaining fluid. Further workup showed a normal TSH, an elevated adrenocorticotropic hormone level of 577 pg/mL, and a urinary tract infection. She was treated with antibiotics and high-dose steroids. Vasopressors were tapered down by day 4, and steroids were reduced. Discharged on day 8 with hydrocortisone, fludrocortisone, and levothyroxine, she recovered at her one-month follow-up with a normal repeat TTE.

Discussion: APS-2, an autoimmune syndrome, is associated with pericardial fluid accumulation, causing tamponade and cardiogenic shock. Reported studies showed that a small amount of fluid is enough to trigger severe shock. APS-2 patients may also experience Addisonian crises, leading to distributive shock and hemodynamic instability. In critically ill patients, like our case, differentiating between both shocks requires a multidisciplinary approach for accurate diagnosis and treatment.