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

Abstract

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.