IMPENDING PERICARDIAL TAMPONADE AS THE INITIAL PRESENTATION OF AUTOIMMUNE POLYGLANDULAR SYNDROME 3B

Abstract

TOPIC: Critical Care TYPE: Fellow Case Reports INTRODUCTION: Autoimmune polyglandular syndromes (APS) are rare polyendocrinopathies characterized by loss of immune tolerance to self-antigens leading to autoimmune destruction of endocrine glands and other tissues. APS is classified into APS types 1, 2, 3 and 4. APS Type 3 is recognized by absence of adrenal insufficiency. APS type 3b (APS3b) specifically comprises of the association of autoimmune thyroid disease with pernicious anemia. CASE PRESENTATION: A 66-year-old female with history of pernicious anemia presented to the emergency department with shortness of breath and lethargy which started on day of arrival. She was diagnosed with pernicious anemia 2 years ago and was receiving intramuscular cyanocobalamin injections. In the emergency department, she was found to be hypoxic with oxygen saturation of 90%. Her physical exam was notable for muffled heart sounds. Her labs on admission were: Hb 11.8 g/dl, HCT 35.8 %, WBC 6.3 K/ul, PLT 166 K/ul, Glucose 106 mg/dl, Sodium 143 mmol/l, BUN 13 mg/dl, Creatinine 1.13 mg/dl, pro-BNP 171 pg/ml, TSH 44.6 UIU/ml, Free T4 0.2 ng/dl, Free T3 0.5 pg/ml and positive thyroid peroxidase antibody >1300 u/ml. Serial troponins were unremarkable. Chest x-ray showed an enlarged cardiac silhouette concerning for pericardial effusion. EKG showed normal sinus rhythm with low voltage QRS complexes. 2D Echo showed a large circumferential pericardial effusion with end diastolic RV collapse suggestive of impending pericardial tamponade. LV ejection fraction was 60%. Pericardiocentesis drained 1700 cc of fluid with relief of patient's symptoms. This was followed by placement of pericardial drain. Her pericardial effusion was attributed to her new diagnosis of hypothyroidism. She was started on low dose levothyroxine given her age above 60 with plan for slowly increasing her dose with close outpatient follow up. DISCUSSION: Pernicious anemia is the most common association with thyroid autoimmune disease in APS type 3 seen in 39% of cases. APS3 is more commonly seen in females. The diagnosis of this condition requires the presence of multiple autoimmune diseases in the same individual, diagnosed in any order and at any age. CONCLUSIONS: A high index of suspicion is required whenever one autoimmune disease is diagnosed to prevent morbidity and mortality from other associated conditions. Here, we describe a patient with known history of pernicious anemia presenting with impending tamponade who was subsequently diagnosed with Hashimoto's thyroiditis. The constellation of these findings represent APS Type 3b. REFERENCE #1: Betterle, Corrado et al. "A rare combination of type 3 autoimmune polyendocrine syndrome (APS-3) or multiple autoimmune syndrome (MAS-3)." Auto- immunity highlights vol. 5,1 27-31. 11 Feb. 2014, doi:10.1007/s13317-013-0055-6 REFERENCE #2: Lahner E, Centanni M, Agnello G, et al. Occurrence and risk factors for autoimmune thyroid disease in patients with atrophic body gastritis. Am J Med. 2008;121(2):136-141. doi:10.1016/j.amjmed.2007.09.025 DISCLOSURES: No relevant relationships by Sana Fatima, source=Web Response No relevant relationships by Shumail Syed, source=Web Response