p-ANCA-associated periaortitis with histological proof of Wegener's granulomatosis: case report

We describe a 63-year-old man who presented with an inflammatory aortic aneurysm. The patient had p-ANCA antibodies directed against myeloperoxidase. A diagnosis of idiopathic periaortitis was made. Seven years later, he was rehospitalized because of fever, weight loss, and polyneuropathy. After revision of the aortic biopsy, which showed necrotizing vasculitis with palisading granuloma, a diagnosis of Wegener's granulomatosis (WG) was made. This case report illustrates an unusual disease course in WG, resembling large vessel vasculitis, and we discuss the possible mechanisms of large vessel involvement in this form of vasculitis.