Background Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic autoimmune small- to medium-vessel vasculitis. Reports of rapidly progressive left main/left anterior descending (LM/LAD) disease under normolipidemic conditions with parallel coronary–aortic progression are scarce. Case presentation We report a case of perinuclear antineutrophil cytoplasmic antibody (p-ANCA) and myeloperoxidase (MPO)–positive ANCA-associated vasculitis (AAV) in which, despite persistently normal lipid levels, the coronary arteries developed rapidly progressive stenosis and occlusion within one year. Intravascular ultrasound (IVUS) revealed concentric thickening of the coronary arterial wall characterized predominantly by soft plaques with minimal calcification, which was consistent with an inflammation-driven remodeling phenotype. Because the admission electrocardiogram (ECG) revealed an ST-segment elevation myocardial infarction (STEMI)-equivalent pattern (aVR ST elevation with diffuse ST depression), the patient was treated for acute myocardial infarction, and urgent LM-to-LAD drug-eluting stent (DES) implantation was performed. During a four-month follow-up after discharge, no recurrent angina or ischemic events were reported. Conclusion In a p-ANCA/MPO-positive AAV patient with sustained normolipidemia, we observed rapidly progressive LM/LAD disease occurring in parallel with aortic involvement, which was consistent with an inflammation-driven coronary phenotype that precipitated acute coronary syndrome. Integrating sequential coronary–aortic imaging with inflammatory biomarker surveillance and individualized immunomodulatory and antithrombotic strategies may enable earlier risk detection and optimize outcomes in patients with AAV.
Lu et al. (Thu,) studied this question.