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Open Access 01-03-2022 | ANCA-Associated Vasculitis | Review

ANCA-Associated Vasculitic Neuropathies: A Review

Authors: Haruki Koike, Ryoji Nishi, Ken Ohyama, Saori Morozumi, Yuichi Kawagashira, Soma Furukawa, Naohiro Mouri, Yuki Fukami, Masahiro Iijima, Gen Sobue, Masahisa Katsuno

Published in: Neurology and Therapy | Issue 1/2022

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic disorder that frequently affects the peripheral nervous system and consists of three distinct conditions: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, previously Wegener’s granulomatosis), and eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg-Strauss syndrome). The neuropathic features associated with this condition usually include mononeuritis multiplex, which reflects the locality of lesions. Findings suggestive of vasculitis are usually found in the epineurium and occur diffusely throughout the nerve trunk. Nerve fiber degeneration resulting from ischemia is sometimes focal or asymmetric and tends to become conspicuous at the middle portion of the nerve trunk. The attachment of neutrophils to endothelial cells in the epineurial vessels is frequently observed in patients with ANCA-associated vasculitis; neutrophils play an important role in vascular inflammation by binding of ANCA. The positivity rate of ANCA in EGPA is lower than that in MPA and GPA, and intravascular and tissue eosinophils appear to participate in neuropathy. Immunotherapy for ANCA-associated vasculitis involves the induction and maintenance of remission to prevent the relapse of the disease. A combination of glucocorticoids along with cyclophosphamide, rituximab, methotrexate, or mycophenolate mofetil is considered depending on the severity of the condition of the organ to induce remission. A combination of low-dose glucocorticoids and azathioprine, rituximab, methotrexate, or mycophenolate mofetil is recommended to maintain remission. The efficacy of anti-interleukin-5 therapy (i.e., mepolizumab) was demonstrated in the case of refractory or relapsing EGPA. Several other new agents, including avacopan, vilobelimab, and abatacept, are under development for the treatment of ANCA-associated vasculitis. Multidisciplinary approaches are required for the diagnosis and management of the disorder because of its systemic nature. Furthermore, active participation of neurologists is required because the associated neuropathic symptoms can significantly disrupt the day-to-day functioning and quality of life of patients with ANCA-associated vasculitis.

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Title: ANCA-Associated Vasculitic Neuropathies: A Review
Authors: Haruki Koike
Ryoji Nishi
Ken Ohyama
Saori Morozumi
Yuichi Kawagashira
Soma Furukawa
Naohiro Mouri
Yuki Fukami
Masahiro Iijima
Gen Sobue
Masahisa Katsuno
Publication date: 01-03-2022
Publisher: Springer Healthcare
Keywords: ANCA-Associated Vasculitis
Vasculitis
ANCA-Associated Vasculitis
Vasculitis
Granulomatosis with Polyangiitis
Churg-Strauss Syndrome
Churg-Strauss Syndrome
Granulomatosis with Polyangiitis
Published in: Neurology and Therapy / Issue 1/2022
Print ISSN: 2193-8253
Electronic ISSN: 2193-6536
DOI: https://doi.org/10.1007/s40120-021-00315-7

At a glance: The STEP trials

Springer Medicine

ANCA-Associated Vasculitic Neuropathies: A Review

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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