Master of Disguise: Atypical Presentation of Aortitis

By Namdarzadeh, B., Mouton, S., Ruban, A., Tadi, S., Vance, J., Baghdadi, O.
Cardiology Interest Group, American University of the Caribbean School of Medicine, Cupecoy, Sint Maarten

Aortitis is a general term to classify aortic wall inflammation. Aortitis can be divided into infectious and non-infectious with isolated cases of idiopathic aortitis. The most common causes of non-infectious aortitis are the large-vessel vasculitis, such as giant cell arteritis (GCA) and Takayasu’s arteritis. Early diagnosis of large-vessel vasculitis is difficult and needs a high level of suspicion. Along with imaging, a biopsy and serologic tests are often needed to help diagnose or exclude different vasculitis. Regardless of the etiology, aortitis frequently presents with dilation of the aortic root, thickening of the vessel wall, aortic aneurysm, aortic insufficiency, and back pain. In rare cases, isolated idiopathic aortitis can be disguised as another condition such as pericardial effusion. Aortitis can appear with a variety of symptoms and often patients with early stage are asymptomatic. Identified risk factors for aortitis include advanced age, history of connective tissue disease, diabetes mellitus, and heart valve pathology. The purpose of this study is to show the difficulty in diagnosing rare aortitis that can be initially hidden by other more pronounced complications.

Case Presentation: A 43-year-old female with a past medical history of asthma and uterine fibroidspresented in clinic complaining of dyspnea with associated chest tightness. Patient states she was woken from sleep by the sudden onset of chest pain with radiation down her left arm. Patient denies any prior history of cardiac diseases. Patient also reports a recent history of fever with a productive cough. Viral pericarditis was initially diagnosed due to 3 days of viral prodrome. A follow up echocardiogram 1 week later showed decreased pericardial effusion without steroid therapy and the patient was discharged. One year later the patient came back with progressive chest pain, dyspnea, lower extremity swelling, and decreased exercise tolerance. Patient started 20mg oral prednisone and her symptoms improved. Further imaging and lab tests were performed to reach the final diagnosis of idiopathic aortitis. Patients with this diagnosis require frequent follow ups due to the increased risk of aneurysm and relapse is very common. Patient was initially on prednisone as its the gold standard for aortitis but was then shifted to azathioprine to achieve better treatment goals. The patient must be observed regularly to avoid adverse effects and to determine if the therapeutic goals are achieved. Early and prompt recognition is critical for treatment, reducing the severity of symptoms, and preventing the need for surgery.

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