High PR3-ANCA positivity in a patient with chronic inflammatory demyelinating polyneuropathy

Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient respon...

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Bibliographic Details
Main Authors: Masanori Kurihara, Yu Kurata, Izumi Sugimoto, Yuki Hatanaka, Yasuhisa Sakurai
Format: Article
Language:English
Published: Elsevier 2017-03-01
Series:eNeurologicalSci
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2405650216300314
Description
Summary:Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis.
ISSN:2405-6502