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Anti-Neutrophil Cytoplasmic Antibody-Negative Central Nervous System Granulomatosis With Polyangiitis and Its Clinical Characteristics

Abstract Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions. Address correspondence and reprint requests to Dr Yongming Qiu, MD, PhD, Department of Neurosurgery, Ren Ji Hospital, School of Medicine, Shanghai JiaoTong University, Shanghai 200127, China; E-mail: qiuzhoub@126.com; Dr Longtian Chen, MD, Department of Rheumatology, Longyan First Hospital Affiliated to Fujian Medical University, Fujian Province, China; E-mail: LT_chen@163.com Received 20 July, 2017 Accepted 24 August, 2017 The authors report no conflicts of interest. © 2017 by Mutaz B. Habal, MD.

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