Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Complicated by Periaortitis and Cranial Hypertrophic Pachymeningitis: A Report of an Autopsy Case

• Published in: Internal Medicine Vol. 57; no. 1; pp. 107 - 113
• Main Authors: Kaga, Hajime, Komatsuda, Atsushi, Saito, Masaya, Nara, Mizuho, Omokawa, Ayumi, Togashi, Masaru, Okuyama, Shin, Wakui, Hideki, Takahashi, Naoto
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01-01-2018; Japan Science and Technology Agency
• Subjects: anti-neutrophil cytoplasmic antibody-associated vasculitis; Aorta; Autopsies; Autopsy; Case Report; cranial hypertrophic pachymeningitis; Eutrophication; Geriatrics; Giant cells; Granulomatosis; granulomatosis with polyangiitis; Inflammatory diseases; Internal medicine; Kidneys; Lesions; Neutrophils; Nose; Older people; periaortitis; Skull; Vasculitis; granulomatosis with polyangiitis; anti-neutrophil cytoplasmic antibody-associated vasculitis; periaortitis; cranial hypertrophic pachymeningitis
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.8751-16

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic inflammatory disorder categorized as small-vessel vasculitis. We herein report an elderly Japanese man with AAV (granulomatosis with polyangiitis affecting the eyes, nose, lungs, and kidneys) who also showed periaortitis at the diagnosis and developed cranial hypertrophic pachymeningitis (HP) during steroid maintenance therapy. His consciousness disturbance caused by HP improved after steroid pulse therapy, but he died of aspiration pneumonia. Autopsy findings showed giant cells in the thickened pachymeninges and obsolete inflammatory lesions in the aortic adventitia and renal tubulointerstitium. This is the first case of AAV complicated by periaortitis and cranial HP.