287 Aortitis and Aortic Valvulitis from Autoimmune Non-Necrotising Vasculitis

Abstract

A 45-year-old female presented with Acute pulmonary oedema associated with Severe Aortic regurgitation (AR) . Past medical history included long term immunosuppression with Hydroxychloroquine and Prednisolone for the management of Discoid Lupus with no previous systemic involvement. Trans-oesophageal Echocardiogram demonstrated thickened, fibrotic, tented aortic valve leaflets without central coaptation causing severe AR . There were no lesions typical of Libman-Sacks endocarditis and no appreciable masses or vegetations. Computed tomography coronary angiogram showed thickening around the aortic root with stenotic lesions in the coronary arteries and a zero-calcium score. Coronary angiogram confirmed these stenotic lesions. Fluorodeoxyglucose - Positron emission tomography showed increased uptake within the aortic root, ascending aorta, and proximal head and neck vessels .The findings were suggestive of vasculitis, however, a complete vasculitis screen was non-contributory. Mechanical Aortic Valve Replacement and Coronary Artery Bypass Grafting to the Right Coronary Artery and Left Anterior Descending Coronary Artery was performed. Histopathology of the aortic root/valve revealed non-necrotising aortitis (NNA), most likely representative of autoimmune associated aortitis. NNA is associated with autoimmune disease in approximately 50% of the cases with a slight female predominance and a typical age of presentation between 60-90 years. Differentials include Takayasu Aortitis, IgG4-related Aortitis, Syphilitic Aortitis, Systemic Lupus Erythematosus, and Isolated Idiopathic Aortitis. In this case, biopsy showed non-specific chronic aortitis with negative spirochete immunohistochemistry, scant IgG4 plasma cells, and no giant cells, with considerable overlap between various aetiologies. The history of immunosuppression treatment may have contributed to the modified histological appearance of aortitis.