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Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a type of small-vessel vasculitis. It is unusual for ANCA to involve aorta. However, multiple cases have been found where ANCA involved large vessels, particularly the aorta. Among vasculitides, aortic vasculitis is a part of Takayasu arteritis (TAK). In this review article, we tried to find the mechanism behind the aortic involvement in AAV. PubMed was used as a primary search engine, and all the available cases of aortic, as well as large-vessel involvement in ANCA-associated vasculitis, were thoroughly reviewed. Very limited data was available that could provide the mechanism behind this involvement. It is observed that ANCA-associated aortitis is more common in immunocompromised people; however, cases in previously healthy individuals have also been found. Pathogenesis of ANCA-related aortitis is different from Takayasu arteritis and is more close to ANCA-associated small vasculitis. ANCA-related aortitis involves the aorta through the same mechanism as it uses to involve small vessels. This rare manifestation of ANCA-associated vasculitis could be life-threatening but has a good prognosis if timely diagnosed and treated. ANCA-associated vasculitis must be considered as a differential diagnosis while treating a case of aortitis. We believe that there is a need to revise the classification of different types of vasculitides, and physicians should be aware of the possible overlap between different forms of vasculitides.

Aortic Involvement in Antineutrophil Cytoplasmic Antibodies Vasculitis, a Coincidence or a Real Association?