Multiple systemic arterial aneurysms in large-vessel vasculitis

Abstract

Large-vessel vasculitis (LVVs) is a rare inflammatory disease that predominantly affects young females' aorta and its main branches, coronary arteries, and pulmonary arteries. Clinical manifestations result from vascular stenosis, occlusion, and dilation, sometimes complicated by aneurysm rupture or dissection. Early diagnosis and treatment of LVVs are paramount to reducing the risk of ischemic complications such as visual loss and strokes, vascular stenosis and occlusion, and aortic aneurysm formation. The diagnosis of LVVs is often challenging because the presenting clinical features are nonspecific in many cases and are often shared by different types of autoimmune and inflammatory diseases including other systemic vasculitides. Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. We report a rare case of the LVVs with multisystemic vascular aneurysms and catastrophic complications of ruptured abdominal aortic aneurysm. Our case highlights that an early diagnosis of the diverse manifestations of vasculitis and a high index of clinical suspicion is essential to avoid delays in disease recognition that may result in permanent or life-threatening morbidity.