PMID- 29021484 OWN - NLM STAT- MEDLINE DCOM- 20180618 LR - 20221207 IS - 1349-7235 (Electronic) IS - 0918-2918 (Print) IS - 0918-2918 (Linking) VI - 57 IP - 1 DP - 2018 Jan 1 TI - Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis Complicated by Periaortitis and Cranial Hypertrophic Pachymeningitis: A Report of an Autopsy Case. PG - 107-113 LID - 10.2169/internalmedicine.8751-16 [doi] AB - 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. FAU - Kaga, Hajime AU - Kaga H AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. FAU - Komatsuda, Atsushi AU - Komatsuda A AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. FAU - Saito, Masaya AU - Saito M AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. FAU - Nara, Mizuho AU - Nara M AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. FAU - Omokawa, Ayumi AU - Omokawa A AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. FAU - Togashi, Masaru AU - Togashi M AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. FAU - Okuyama, Shin AU - Okuyama S AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. FAU - Wakui, Hideki AU - Wakui H AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. AD - Department of Life Science, Akita University Graduate School of Engineering Science, Japan. FAU - Takahashi, Naoto AU - Takahashi N AD - Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20171011 PL - Japan TA - Intern Med JT - Internal medicine (Tokyo, Japan) JID - 9204241 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Biomarkers) SB - IM MH - Adrenal Cortex Hormones/*therapeutic use MH - Aged MH - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*complications/diagnostic imaging/*drug therapy MH - Antibodies, Antineutrophil Cytoplasmic/*blood MH - Asian People MH - Autopsy MH - Biomarkers/blood MH - Dura Mater/physiopathology MH - Fatal Outcome MH - Granulomatosis with Polyangiitis/complications/diagnosis/*drug therapy MH - Humans MH - Hypertrophy/complications/*drug therapy MH - Male MH - Meningitis/diagnosis/diagnostic imaging/*therapy PMC - PMC5799067 OTO - NOTNLM OT - anti-neutrophil cytoplasmic antibody-associated vasculitis OT - cranial hypertrophic pachymeningitis OT - granulomatosis with polyangiitis OT - periaortitis EDAT- 2017/10/13 06:00 MHDA- 2018/06/19 06:00 PMCR- 2018/01/01 CRDT- 2017/10/13 06:00 PHST- 2017/10/13 06:00 [pubmed] PHST- 2018/06/19 06:00 [medline] PHST- 2017/10/13 06:00 [entrez] PHST- 2018/01/01 00:00 [pmc-release] AID - 10.2169/internalmedicine.8751-16 [doi] PST - ppublish SO - Intern Med. 2018 Jan 1;57(1):107-113. doi: 10.2169/internalmedicine.8751-16. Epub 2017 Oct 11.