8746 Primary Prevention and Early Intervention for Cardiovascular Disease in Polyglandular Autoimmune Syndrome Type 2
Authors: Flores-Robles Valerie Nicole , Fuentes-Rosa Yatzel , Sanchez-Cordero Milaris , Lopez David A , Rivera Melanie Caban , Carrero-Quinones Milton 
Published: October 2024

Disclosure: V.N. Flores-Robles: None. Y. Fuentes-Rosa: None. M. Sanchez-Cordero: None. D.A. Lopez: None. M. Carrero-Quinones: None.

Introduction: Polyglandular autoimmune syndrome type 2 (PAS-2) is an autoimmune syndrome that is known for lymphocytic infiltration causing organ-specific damage. It can include primary adrenal insufficiency (Addison's disease), type 1 diabetes mellitus (T1DM), autoimmune thyroid disease-causing Grave’s disease, or hypothyroidism, and can be diagnosed when two out of these three pathologies are present. Carpenter syndrome, a type of PAS-2, is described as the triad of Addison's disease, autoimmune thyroid disease, and T1DM. Its initial manifestation can be associated with non-endocrine and endocrine disorders as in autoimmune thyroid disease, T1DM, primary hypogonadism, and diabetes insipidus. It is well documented that uncontrolled diabetes mellitus can accelerate the progression of cardiovascular disease of co-existing coronary artery disease, but documentation that Addison’s disease may accelerate the progression is scant with only ten cases documented. On the other hand, there are no documented cases of the presence of coronary artery disease in patients with PAS-2. Case report: This case involves a 66-year-old Hispanic male with a past medical history of hypertension, and PAS-2, presenting with Addison’s disease, type 1 diabetes mellitus, and hypothyroidism. The initial presentation at the hospital was that of a complete atrioventricular block that required pacemaker placement. Cardiology services were consulted as the patient also had a positive stress test and eventually cardiac catheterization was done as further workup. Cardiac catheterization showed obstruction of the left main coronary artery of 80% and left circumflex with mild disease, that required coronary artery bypass (CABG). A 2D ECHO was done for further evaluation of cardiac function and findings were pertinent for an ejection fraction of 65-70%, mild concentric hypertrophy of the left ventricular wall, and left ventricular diastolic function with impaired relaxation pattern. The patient underwent the CABG procedure without any complications. He has been followed up outpatient and remains without any symptoms or pertinent presentation. Early intervention in this patient with PAS-2 prevented any further cardiovascular complications. Conclusion: Patients with PAS-2 may have a predisposition to developing coronary artery disease due to concomitant endocrinopathies that independently increase the risk of cardiovascular disease. This promotes further investigation into the primary prevention of cardiovascular disease in patients presenting with PAS-2. Taking into consideration the increased risk and/or progression of cardiovascular disease in these patients, early intervention and evaluation by cardiology services should be suggested as it may decrease their morbidity or mortality.

Presentation: 6/2/2024