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Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis pp 193–237Cite as

Peripheral Nervous System Involvement

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Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Vasculitis is an uncommon but treatable and thus important cause of neuropathy. Vasculitic neuropathy can occur as an isolated entity (nonsystemic vasculitic neuropathy) but more frequently evolves in the setting of a systemic vasculitis. Polyarteritis nodosa, anti-neutrophil antibody-associated vasculitides (especially microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis), rheumatoid vasculitis, and cryoglobulinemic vasculitis are the systemic disorders most commonly associated with neuropathy. Both systemic vasculitic and nonsystemic vasculitic neuropathy usually present with a subacutely progressive, asymmetric/multifocal, painful, distal-predominant, axonal, sensory-motor phenotype. Available evidence suggests that nonsystemic and most systemic vasculitic neuropathies are mediated primarily by T-cell mechanisms, but immune complexes probably contribute. Evaluation of patients with suspected vasculitic neuropathy usually includes EMG, laboratory studies, and nerve/muscle biopsy. In patients lacking definite vasculitis in nerve biopsy, clinically probable vasculitic neuropathy can be diagnosed by recourse to supportive clinicopathologic findings. Vasculitic neuropathies occurring in the ANCA-associated vasculitides are treated in accordance with the underlying systemic vasculitis. About 50% of neuropathy-predominant primary systemic vasculitides are not controlled by initial corticosteroid monotherapy. IVIg or rituximab can induce remission in refractory or relapsing patients. Long-term outcome is reasonably good.

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Abbreviations

AAV:

ANCA-associated vasculitides

ACR:

American College of Rheumatology

AIF:

Allograft inflammatory factor

ANA:

Antinuclear antibody

ANCA:

Anti-neutrophil cytoplasmic antibody

AZA:

Azathioprine

CHCC:

Chapel Hill Consensus Conference

CIDP:

Chronic inflammatory demyelinating polyneuropathy

CMV:

Cytomegalovirus

CS:

Corticosteroids

CTD:

Connective tissue disease

CTL:

Cytotoxic T lymphocyte

CYC:

Cyclophosphamide

DCVAS:

Diagnostic and Classification Criteria in Vasculitis Study

DIF:

Direct immunofluorescence

EGPA:

Eosinophilic granulomatosis with polyangiitis

EMG:

Electromyography

ESR:

Erythrocyte sedimentation rate

EULAR:

European League Against Rheumatism

FVSG:

French Vasculitis Study Group

GCA:

Giant cell arteritis

GPA:

Granulomatosis with polyangiitis

HNPP:

Hereditary neuropathy with liability to pressure palsies

ICOS:

Inducible costimulator

IHC:

Immunohistochemical

IVIg:

Intravenous immunoglobulins

MMF:

Mycophenolate mofetil

MMP:

Matrix metalloproteinase

MPA:

Microscopic polyangiitis

MPO:

Myeloperoxidase

MTX:

Methotrexate

NCS:

Nerve conduction studies

NSVN:

Nonsystemic vasculitic neuropathy

PAN:

Polyarteritis nodosa

PE:

Plasma exchange

PR3:

Proteinase 3

RCT:

Randomized controlled trial

RF:

Rheumatoid factor

SVN:

Systemic vasculitic neuropathy

VEGF:

Vascular endothelial growth factor

VN:

Vasculitic neuropathy

VZV:

Varicella zoster virus

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Authors and Affiliations

  1. Medical College of Wisconsin, Milwaukee, WI, USA

    Michael P. Collins

  2. Mayo Clinic, Rochester, MN, USA

    P. James B. Dyck

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  1. Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Monza e Brianza, Italy

    Renato Alberto Sinico

  2. Department of Medicine, Paris Descartes University Hospital Cochin, Paris, France

    Loïc Guillevin

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Collins, M.P., Dyck, P.J.B. (2020). Peripheral Nervous System Involvement. In: Sinico, R., Guillevin, L. (eds) Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-02239-6_12

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  • DOI: https://doi.org/10.1007/978-3-030-02239-6_12

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-030-02239-6

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