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<h4>Objective</h4>Reported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN.<h4>Methods</h4>Data of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Associations with other organ manifestations were compared in patients with and without VN.<h4>Results</h4>Nine hundred fifty-five patients (mean age 57 years, range 18-91 years, 51% female) were identified. Of these, 572 had granulomatosis with polyangiitis (GPA), 218 microscopic polyangiitis (MPA), and 165 eosinophilic granulomatosis with polyangiitis (EGPA). The prevalence of VN was 65% in EGPA, 23% in MPA, and 19% in GPA. Nerve biopsy was performed in 32/269 (12%) patients, demonstrating definite vasculitis in 17/32 (53%) of patients. VN was associated with myeloperoxidase-ANCA positivity (<i>p =</i> 0.004) and skin (<i>p</i> < 0.001), musculoskeletal, (<i>p</i> < 0.001) and cardiovascular (<i>p =</i> 0.005) involvement. Patients with VN were less likely to have renal (<i>p</i> < 0.001), eye (<i>p</i> < 0.001), and gastrointestinal (<i>p =</i> 0.023) involvement.<h4>Conclusions</h4>Our study provides comprehensive insights into the prevalence and organ associations of VN in a large, systematically collected AAV cohort. VN is most commonly associated with skin, musculoskeletal, and cardiovascular manifestations. In routine clinical practice, diagnosis of VN is infrequently confirmed by the gold standard of nerve biopsy but rather supported by the clinical setting of active systemic AAV.

Original publication




Journal article


Neurology(R) neuroimmunology & neuroinflammation

Publication Date





From the Immunology Clinic and Department of Neurology (A.B.), Departments of Medicine, Biomedicine and Clinical Research, University Hospital Basel; Department of Rheumatology (V.K.J., T.D.), University Hospital Basel, Switzerland; Department of Neurology (R.D.M.H.), King's College Hospital, London; Nuffield Department of Orthopedics (R.A.L., A.C.), Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, University of Oxford, United Kingdom; Department of Neurology (A.-K.P.), University Hospital Basel & Clinical Neuroimmunology; Department of Biomedicine (A.-K.P.), University of Basel, Switzerland; Division of Rheumatology and Department of Biostatistics, Epidemiology, and Informatics (P.A.M.), University of Pennsylvania, Philadelphia; Department of Rheumatology (R.S.), Auckland District Health Board, New Zealand; and Department of Neurology (M.P.C.), Medical College of Wisconsin, Milwaukee, WI.


Humans, Peripheral Nervous System Diseases, Churg-Strauss Syndrome, Prevalence, Adolescent, Adult, Aged, Aged, 80 and over, Middle Aged, Female, Male, Young Adult, Microscopic Polyangiitis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Granulomatosis with Polyangiitis