High PR3-ANCA positivity in a patient with chronic inflammatory demyelinating polyneuropathy
Masanori Kurihara, Yu Kurata, Izumi Sugimoto, Yuki Hatanaka, Yasuhisa Sakurai, Masanori Kurihara, Yu Kurata, Izumi Sugimoto, Yuki Hatanaka, Yasuhisa Sakurai
Abstract
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.
Keywords: Chronic inflammatory demyelinating polyneuropathy (CIDP); PR3-anti-neutrophil cytoplasmic antibody (PR3-ANCA); Vasculitic neuropathy.
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Source: PubMed