Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Aortitis Presenting With Acute Dissection: Case Report and Comprehensive Literature Review.

Description Vasculitides are a group of diseases that produces vasculitis, which is characterized by inflammatory infiltrates within blood vessel walls and results in intimal injury as well as progressive mural destruction. Infiltrates are characterized per the Chapel Hill classification, into large, medium, and small-vessel vasculitides. ANCA-associated vasculitis (AAV) is a disease that has been described as involving small-sized vessels. However, some cases of large vessel disease involvement have been documented. ANCA-associated aortitis is a rare entity poorly described in the literature. Due to the rarity of this pathology, there is no Level I evidence available regarding diagnosis and treatment. We present the rare case of an 80-year-old male presenting with ANCA-associated aortitis complicated by acute dissection of the left common iliac artery. His case was successfully managed by corticosteroid therapy and endovascular stenting of the involved iliac artery. ANCA-associated aortitis is a rare entity that has not been described well in the current literature. We believe this case to be the first involving ANCA-associated aortitis presenting with an acute dissection.