Cardiac Tamponade as the Inaugural Manifestation of Type 2 Autoimmune Polyglandular Syndrome

Document Type

Conference Proceeding

Publication Date

10-2025

Publication Title

Chest

Abstract

INTRODUCTION: Autoimmune Polyglandular Syndrome (APS) Type 2 is characterized by a combination of adrenal insufficiency, autoimmune thyroid disease, and Type 1 diabetes. Its incidence ranges from 1.4 to 4.5 per 100,000 individuals and is associated with HLA DR3 and DR4. Target antigens include 21-hydroxylase (adrenal insufficiency), thyroid peroxidase (Hashimoto's thyroiditis), and glutamic acid decarboxylase (Type 1 diabetes). Pericardial effusion in APS Type 2 may result from pericardial sac inflammation, possibly triggered by antibody formation or impaired T-cell regulation. CASE PRESENTATION: A 32-year-old male with a recent diagnosis of hypothyroidism, initiated on levothyroxine every other day two weeks earlier, presented to the emergency department with progressive fatigue and shortness of breath over the past week. Ultrasonography revealed a significant pericardial effusion. Laboratory findings included a markedly elevated thyroid-stimulating hormone (TSH) of 148 mU/L, anti-thyroid peroxidase (anti-TPO) antibodies >1000 IU/mL, thyroglobulin antibodies (TG ab) 851 IU/mL, and free thyroxine (FT4) of 0.7 ng/dL. Additionally, he had hypokalemia (K 3.1 mmol/L), elevated creatinine (3.12 mg/dL), and significantly elevated liver enzymes (AST 2000 IU/L, ALT 1707 IU/L), bilirubin 3 mg/dL, and ferritin 40,000 ng/mL. Echocardiography revealed a reduced ejection fraction (EF 30%) with global hypokinesis, and troponin was mildly elevated at 0.9 ng/mL, without ischemic changes on EKG. The patient was transferred to the ICU for hypotension and started on intravenous levothyroxine, stress-dose steroids, as well as vasopressor support with dobutamine. Pericardial effusion was drained, with fluid analysis showing transudative characteristics and >2000 cells, predominantly neutrophils. Cultures were negative for infection. The patient was weaned off pressors, and his TSH improved (down to 23 mU/L), liver function tests normalized, creatinine improved, and EF recovered. Cosyntropin test was positive on subsequent hospitalization and patient was diagnosed with APS type 2 DISCUSSION: Pericardial effusion in Type 2 APS can present as recurrent effusion or cardiac tamponade, typically diagnosed in patients aged 20-30 years. This delayed diagnosis is especially common in this age group. Hypothyroidism is often detected in outpatient visits, but thyroid supplementation can exacerbate adrenal insufficiency. Adrenal insufficiency worsens pericardial effusion through hypovolemia from a hypoaldosterone state, reducing cardiac pressures. Additionally, decreased alpha-1 receptor potentiation increases vascular permeability, further aggravating the pericardial effusion. CONCLUSIONS: Assessing adrenal function in patients with significant fatigue and newly diagnosed hypothyroidism is crucial in this age cohort.

Volume

168

Issue

4S

First Page

768A

Comments

American College of Chest Physicians CHEST Annual Meeting, October 19-22, 2025, Chicago, IL

Last Page

768A

DOI

10.1016/j.chest.2025.07.454

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