Autopsy findings showed giant cells in the thickened pachymeninges and obsolete inflammatory lesions in the aortic adventitia and renal tubulointerstitium. Conference Nomenclature of Vasculitides, anti-neutrophil cytoplasmic antibody (ANCA)-connected vasculitis (AAV) is definitely classified as small-vessel vasculitis (1). The three major clinicopathologic variants of AAV are microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) (previously known as Wegener’s granulomatosis), and eosinophilic granulomatosis with polyangiitis (1). In Japanese individuals with AAV, myeloperoxidase (MPO)-ANCA-positive MPA/renal-limited vasculitis is the most common form of AAV, and approximately half of individuals with GPA are positive for MPO-ANCA or proteinase 3 (PR3)-ANCA (2). These medical features in Japanese individuals contrast markedly with those in individuals from European countries and the United States (2). Although AAV is definitely characterized by small-vessel swelling, large-vessel involvement MAP2K2 can rarely happen (3-13). For example, in 2004, Chirinos et al. (3) reported a case of fatal aortitis in a patient with MPA and examined 13 reported instances of large-vessel involvement in AAV since 1990. Thereafter, related cases have been reported (4-13). Cranial and spinal hypertrophic pachymeningitis (HP) is definitely a rare inflammatory disorder characterized by localized or diffuse thickening of the dura mater, causing intracranial hypertension, cranial nerve palsy, and spinal cord dysfunction (14). A nationwide survey of HP in Japan exposed that ANCA-related HP is the most frequent form of this disease (14). Yokoseki et al. (15) recently reported the medical significance of MPO-ANCA in HP. According to a recent review of published case reports of HP associated with ANCA since 2000, approximately half of individuals were Japanese (16). To our knowledge, there have been three case reports of AAV complicated by large-vessel involvement and dural/epidural swelling of the spinal cord (5,6,10). We herein statement the 1st case of AAV (MPO-ANCA-positive GPA) complicated by large-vessel involvement (periaortitis) and intracranial HP. Our patient died of aspiration pneumonia during steroid therapy. We also describe the autopsy findings. Case Statement A 69-year-old Japanese man having a 2-12 Dehydrocholic acid months history of refractory uveitis was admitted because of progressive visual disturbance. Contrast-enhanced computed tomography (CT) of the head and chest exposed remaining orbital tumor and mucosal thickening of the nose cavity (Fig. 1A), several patchy pulmonary shadows (Fig. 1B), and wall thickening of the ascending aorta and aortic arch (Fig. 1C and D). A dipstick urinalysis showed no proteinuria or hematuria, but elevated levels of 2-microglobulin (2,244 g/L) and N-acetyl–D-glucosaminidase (12.7 U/L) were observed. Blood urea nitrogen was 11.7 mg/dL, and serum creatinine was 0.86 mg/dL. Serologic checks revealed an elevated level of serum C-reactive protein (CRP) (7.51 mg/dL), a normal level of serum IgG4 (96.4 mg/dL) (normal 135 mg/dL), positivity for MPO-ANCA (38 EU) (normal 20 EU), and negativity for PR3-ANCA ( 10 EU) (normal 10 EU) and antinuclear antibodies. A biopsy of the remaining orbital mass showed necrotizing granuloma surrounded by fibrosis with epithelioid cells, multinucleated huge cells, and leukocyte infiltration (Fig. 2A). A renal biopsy exposed small-sized necrotizing arteritis and tubulointerstitial nephritis with multinucleated huge cell formation Dehydrocholic acid (Fig. 2B and C). Based on these findings, he was diagnosed with GPA complicated by periaortitis. After treatment with prednisolone (PSL) (40 mg/day time for 4 weeks), his inflammatory symptoms were improved, and the irregular CT findings in the lungs and aortic arch were resolved (Fig. 3). At that time, serum MPO-ANCA titer was normalized. Two months later, the PSL dose was gradually tapered, and he Dehydrocholic acid was discharged. Thereafter, he was treated with 7.5 mg/day of PSL in our outpatient clinic with normalized serum MPO-ANCA titers. However, he became completely blind two years later on due to accompanying central retinal artery occlusion. Open in a separate window Number 1. CT findings at the 1st admission. CT of the head and chest shows a contrast-enhanced remaining orbital mass and mucosal thickening of the nose cavity (A), patchy shadows within the lungs (B), and contrast-enhanced smooth tissue shadow round the ascending aorta and aortic arch (C, D)..