Kaga Hajime, Komatsuda Atsushi, Saito Masaya, Nara Mizuho, Omokawa Ayumi, Togashi Masaru, Okuyama Shin, Wakui Hideki, Takahashi Naoto
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan.
Department of Life Science, Akita University Graduate School of Engineering Science, Japan.
Intern Med. 2018 Jan 1;57(1):107-113. doi: 10.2169/internalmedicine.8751-16. Epub 2017 Oct 11.
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.
抗中性粒细胞胞浆抗体相关性血管炎（AAV）是一种归类为小血管炎的全身性炎症性疾病。我们在此报告一名患有AAV（累及眼、鼻、肺和肾的肉芽肿性多血管炎）的老年日本男性，其在诊断时还表现为主动脉周炎，并在类固醇维持治疗期间发展为颅骨肥厚性硬脑膜炎（HP）。他因HP引起的意识障碍在类固醇冲击治疗后有所改善，但最终死于吸入性肺炎。尸检结果显示增厚的硬脑膜中有巨细胞，主动脉外膜和肾小管间质中有陈旧性炎症病变。这是首例并发主动脉周炎和颅骨HP的AAV病例。
