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Peripheral Nervous System Involvement

  • Michael P. CollinsEmail author
  • P. James B. Dyck
Chapter
Part of the Rare Diseases of the Immune System book series (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.

Keywords

Vasculitis Neuropathy Antineutrophil cytoplasmic antibodies Nerve biopsy Pathology 

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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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Medical College of WisconsinMilwaukeeUSA
  2. 2.Mayo ClinicRochesterUSA

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