Abstract 10845: Spontaneous Coronary Artery Dissection as the Primary Presentation of Ana-Negative Lupus

Abstract

Introduction: Spontaneous coronary artery dissection (SCAD) is a rare cause of myocardial infarction that most often occurs in middle-aged women with minimal classic risk factors and has been associated with hormonal imbalance, pregnancy, fibromuscular dysplasia, and connective tissue disease. SCAD has been seldom associated with systemic lupus erythematosus (SLE). SLE patients typically exhibit positive ANA titers. Here, we present the first known case report of a patient with SCAD as the primary presentation of ANA-negative SLE. Case Presentation: A 55-year-old female with migraines presented for elective kidney donation. Surgery was complicated by hemodynamic instability and NSTEMI. Transthoracic echocardiogram (TTE) showed preserved ejection fraction and hypokinesis of the basal to mid lateral and septal walls. Coronary angiogram was suspicious for type 3 SCAD of the left anterior descending (LAD) coronary artery, and given the clinical course, a coronary wire was advanced to perform intracoronary hemodynamic assessment, which was negative for atherosclerotic disease. Upon retraction of the wire, there was no flow in the LAD, consistent with propagated SCAD, leading to PEA arrest. After resuscitation, the LAD was stented. Repeat TTE showed preserved ejection fraction and normal wall motion. She was discharged on aspirin and clopidogrel. At cardiology outpatient follow up, the patient described symptoms consistent with Raynaud’s phenomenon and was referred to Rheumatology. She ultimately also developed hair loss, oral ulcers, joint pain, photosensitivity, malar erythema, and Sicca symptoms. Her anti-double stranded DNA antibodies were strongly positive twice, but remarkably, ANA screening was repeatedly negative. However, taken together, she met classification criteria for SLE diagnosis. Conclusions: SLE can present in highly atypical fashion, clinically and serologically, as with this case. SCAD is rare, but this case highlights the importance of keeping it on the differential for myocardial infarction, particularly in female patients with non-traditional risk factors or rheumatological disease. This case also exemplifies there should be a low clinical threshold for rheumatological workup in patients diagnosed with SCAD.