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.
Carels
T
T
Department of Internal Medicine, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium.
Verbeken
E
E
Blockmans
D
D
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging / Q000473:pathology*; D017544:Aortic Aneurysm, Abdominal / Q000150:complications / Q000175:diagnosis; D015415:Biomarkers; D001706:Biopsy; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D005455:Fluorescent Antibody Technique; D005500:Follow-Up Studies; D013700:Giant Cell Arteritis / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011860:Radiography, Abdominal; D014057:Tomography, X-Ray Computed
D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers