AB0621 Characteristics of Anti-Neutrophil Cytoplasmic Antibody Associated Vasculitis With Severe Peripheral Neuropathy.

Abstract

BackgroundPeripheral neuropathy is one of major manifestations of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and has a significant impingement on patients’ quality of life.1,2ObjectivesThe aim of this study was to clarify clinical features of AAV patients with peripheral neuropathy and identify risk factors for severe motor neuropathy.MethodsAll consecutive patients with active, treatment-naïve AAV who were admitted to our hospital between April, 2012 and October, 2021 were reviewed. Patients were divided into two groups according to the presence or absence of peripheral neuropathy, and their baseline clinical characteristics were compared.ResultsA total of 76 patients (microscopic polyangiitis, MPA, n=37, granulomatosis with poly angiitis, GPA, n=24, eosinophilic GPA, EGPA, n=15) were included in the study. Among them, 28 patients (36.8%) had peripheral neuropathy including 12 with MPA, 4 with GPA, and 12 with EGPA. The clinical characteristics of all patients with peripheral neuropathy were younger age (66.0 years versus 74.5 years, p<0.05) and showed higher white blood cell counts (11500/μL versus 8800/μL, p<0.05), higher blood eosinophil counts (788.5/μL versus 139.0/μL, p<0.05), higher creatinine clearance (77.3 mL/min versus 56.3 mL/min, p<0.05), higher levels of serum immunoglobulin (Ig)E (660 IU/mL versus 125 IU/mL, p<0.05), and IgG4 (361.5 mg/dL versus 84.0 mg/dL, p <0.05). In patients with EGPA, the most susceptible type to peripheral neuropathy, 80% of the patients had peripheral neuropathy. Comparison between patients with EGPA with peripheral neuropathy and those without demonstrated significantly younger age (56.0 years versus 80.0 years, p<0.05) and higher blood eosinophil counts (7832/μL versus 2340/μL, p<0.05) were characteristic for the presence of peripheral neuropathy. Patients with EGPA with motor neuropathy (n=8) showed higher white blood cell counts (26850//μL versus 8650//μL, p <0.05) and higher blood eosinophil counts (13134//μL versus 3436//μL, p<0.05) compared with those with only sensory neuropathy (n=4).ConclusionOur current study has shown that patients with EGPA are more prone to peripheral neuropathy than patients with MPA or GPA. Severe motor neuropathy was observed only in patients with EGPA and associated with more intense eosinophilic inflammation. Our results suggest that molecular targeted therapy that improves eosinophilic inflammation such as anti-IL-5 therapy is beneficial for peripheral neuropathy.References[1]RUTGERS, Abraham; KALLENBERG, Cees GM. Peripheral neuropathy in AAV—when vasculitis hits a nerve. Nature Reviews Rheumatology, 2012, 8.3: 127-128.[2]NAKAZAWA, Daigo, et al. Pathogenesis and therapeutic interventions for ANCA-associated vasculitis. Nature Reviews Rheumatology, 2019, 15.2: 91-101.Disclosure of InterestsNone declared