Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides
Open Access
- 1 November 2019
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology Neuroimmunology & Neuroinflammation
Abstract
Objective 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. Methods 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. Results 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 (p = 0.004) and skin (p < 0.001), musculoskeletal, (p < 0.001) and cardiovascular (p = 0.005) involvement. Patients with VN were less likely to have renal (p < 0.001), eye (p < 0.001), and gastrointestinal (p = 0.023) involvement. Conclusions 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.Keywords
This publication has 18 references indexed in Scilit:
- Lumbosacral radiculoplexus neuropathyNeurology, 2019
- Vasculitic peripheral neuropathy: Case definition and guidelines for collection, analysis, and presentation of immunisation safety dataVaccine, 2017
- Cellular infiltrates in skin and sural nerve of patients with polyneuropathiesMuscle & Nerve, 2016
- The Neuropathies of VasculitisNeurologic Clinics, 2013
- Eosinophilic granulomatosis with polyangiitis (Churg‐Strauss): Clinical characteristics and long‐term followup of the 383 patients enrolled in the French Vasculitis Study Group cohortArthritis & Rheumatism, 2012
- A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patientsAnnals Of The Rheumatic Diseases, 2012
- Peripheral neuropathy in ANCA-associated vasculitis: outcomes from the European Vasculitis Study Group trialsRheumatology, 2011
- Combined nerve and muscle biopsy in the diagnosis of vasculitic neuropathy. A 16‐year retrospective study of 202 casesJournal of the Peripheral Nervous System, 2006
- Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: A comparative studyJournal of the Neurological Sciences, 2006
- Development and initial validation of the vasculitis damage index for the standardized clinical assessment of damage in the systemic vasculitidesArthritis & Rheumatism, 1997