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KMID : 0911820180190010025
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Korean Journal of Headache
2018 Volume.19 No. 1 p.25 ~ p.28
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Antineutrophil Cytoplasmic Antibody Related Hypertrophic Pachymeningitis
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Park Yong-Hee
Ko Jeong-Min
Ha Joon-Young
Lee Ji-Yong
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Abstract
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Hypertrophic pachymeningitis is a rare disease, characterized by the inflammatory thickening of the dura mater, mostly around the brain or rarely the spinal cord and can be classified based on its etiology, including idiopathic or secondary hypertrophic pachymeningitis due to autoimmune diseases, IgG4-related disease, infections, trauma and malignancies. Recent several studies have indicated a close relationship between antineutrophil cytoplasmic antibody (ANCA) and hypertrophic pachymeningitis and have revealed that the presence of ANCA is a useful indicator of ANCA-associated vasculitis as the cause without biopsy. We herein report the case of a man with ANCA related hypertrophic cranial pachymeningitis which was improved by intravenous cyclophosphamide.
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KEYWORD
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Hypertrophic pachymeningitis, Antineutrophil cytoplasmic antibody, Cyclophosphamide
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