Reports of rapidly progressive left main/left anterior descending (LM/LAD) disease under normolipidemic conditions with parallel coronary-aortic progression are scarce.<h4>Case presentation</h4>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. Here, MPO is linked to anti-neutrophil cytoplasmic antibody-associated vasculitis.