Central Nervous System Involvement in ANCA-Associated Vasculitis: What Neurologists Need to Know
Open Access
- 10 January 2019
- journal article
- review article
- Published by Frontiers Media SA in Frontiers in Neurology
- Vol. 9, 1166
- https://doi.org/10.3389/fneur.2018.01166
Abstract
Objective: To provide a comprehensive review of the central nervous system (CNS) involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including the pathogenesis, clinical manifestations, ancillary investigations, differential diagnosis, and treatment. Particular emphasis is placed on the clinical spectrum and diagnostic testing of AAV. Recent Findings: AAV is a pauci-immune small-vessel vasculitis characterized by neutrophil-mediated vasculitis and granulomatousis. Hypertrophic pachymeninges is the most frequent CNS presentation. Cerebrovascular events, hypophysitis, posterior reversible encephalopathy syndrome (PRES) or isolated mass lesions may occur as well. Spinal cord is rarely involved. In addition, ear, nose and throat (ENT), kidney and lung involvement often accompany or precede the CNS manifestations. Positive ANCA testing is highly suggestive of the diagnosis, with each ANCA serotype representing different groups of AAV patients. Pathological evidence is the gold standard but not necessary. Once diagnosed, prompt initiation of induction therapy, including steroid and other immunosuppressants, can greatly mitigate the disease progression. Conclusions and Relevance: Early recognition of AAV as the underlying cause for various CNS disorders is important for neurologists. Ancillary investigations especially the ANCA testing can provide useful information for diagnosis. Future studies are needed to better delineate the clinical spectrum of CNS involvement in AAV and the utility of ANCA serotype to classify those patients. Evidence Review: We searched Pubmed for relevant case reports, case series, original research and reviews in English published between Sep 1st, 2001 and Sep 1st, 2018. The following search terms were used alone or in various combinations: “ANCA,” “proteinase 3/PR3-ANCA,” “myeloperoxidase/MPO-ANCA,” “ANCA-associated vasculitis,” “Wegener's granulomatosis,” “microscopic polyangiitis,” “Central nervous system,” “brain” and “spinal cord”. All articles identified were full-text papers.Keywords
This publication has 83 references indexed in Scilit:
- The Role of the Innate Immune System in Granulomatous DisordersFrontiers in Immunology, 2013
- Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: a cluster analysisAnnals Of The Rheumatic Diseases, 2012
- Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis--a meta-analysisRheumatology, 2011
- Rituximab as Maintenance Therapy for Anti-Neutrophil Cytoplasmic Antibody-Associated VasculitisClinical Journal of the American Society of Nephrology, 2010
- Rituximab versus Cyclophosphamide for ANCA-Associated VasculitisNew England Journal of Medicine, 2010
- Patients with Wegener’s granulomatosis demonstrate a relative deficiency and functional impairment of T‐regulatory cellsImmunology, 2010
- Review: Diagnosis and treatment of cerebral vasculitisTherapeutic Advances in Neurological Disorders, 2009
- EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitisAnnals Of The Rheumatic Diseases, 2007
- Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studiesAnnals Of The Rheumatic Diseases, 2006
- Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosisClinical Rheumatology, 2005