Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary

Non‐systemic vasculitic neuropathy (NSVN) is routinely considered in the differential diagnosis of progressive axonal neuropathies, especially those with asymmetric or multifocal features. Diagnostic criteria for vasculitic neuropathy, classification criteria for NSVN, and therapeutic approaches to...

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Bibliographic Details
Published in:Journal of the peripheral nervous system 2010-09, Vol.15 (3), p.176-184
Main Authors: Collins, Michael P., Dyck, P. James B., Gronseth, Gary S., Guillevin, Loïc, Hadden, Robert D. M., Heuss, Dieter, Léger, Jean-Marc, Notermans, N.C., Pollard, John D., Said, Gérard, Sobue, Gen, Vrancken, A.F.J.E., Kissel, John T.
Format: Article
Language:English
Subjects:
antineutrophil cytoplasmic antibodies
azathioprine
Biopsy
Classification
Clinical trials
Corticoids
Cryoglobulins
Cyclophosphamide
diagnosis
Differential diagnosis
Drugs
Evidence-Based Medicine
Fibrinogen
guidelines
Humans
Immunofluorescence
Immunoglobulin M
Immunosuppression - methods
Immunosuppressive agents
Inflammation
Methotrexate
Muscles
Necrosis
Neurodegeneration
Neuropathy
Pain
Peripheral nerves
Peripheral Nervous System Diseases - classification
Peripheral Nervous System Diseases - complications
Peripheral Nervous System Diseases - diagnosis
Peripheral Nervous System Diseases - therapy
Regeneration
Remission
Sedimentation
Side effects
Systemic vasculitis
treatment
United States
vasculitic neuropathy
Vasculitis
Vasculitis - classification
Vasculitis - complications
Vasculitis - diagnosis
Vasculitis - therapy
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Summary:Non‐systemic vasculitic neuropathy (NSVN) is routinely considered in the differential diagnosis of progressive axonal neuropathies, especially those with asymmetric or multifocal features. Diagnostic criteria for vasculitic neuropathy, classification criteria for NSVN, and therapeutic approaches to NSVN are not standardized. The aim of this guideline was to derive recommendations on the classification, diagnosis, investigation, and treatment of NSVN based on the available evidence and, where evidence was not available, expert consensus. Experts on vasculitis, vasculitic neuropathy, and methodology systematically reviewed the literature for articles addressing diagnostic issues concerning vasculitic neuropathy and NSVN as well as treatment of NSVN and the small‐to‐medium vessel primary systemic vasculitides using MEDLINE, EMBASE, and the Cochrane Library. The selected articles were analyzed and classified. The group initially reached consensus on a classification of vasculitides associated with neuropathy. Non‐diabetic radiculoplexus neuropathy was incorporated within NSVN. The consensus definition of pathologically definite vasculitic neuropathy required that vessel wall inflammation be accompanied by vascular damage. Diagnostic criteria for pathologically probable vasculitic neuropathy included five predictors of definite vasculitic neuropathy: vascular deposits of IgM, C3, or fibrinogen by direct immunofluorescence; hemosiderin deposits; asymmetric nerve fiber loss; prominent active axonal degeneration; and myofiber necrosis, regeneration, or infarcts in peroneus brevis muscle biopsy (Good Practice Points from class II/III evidence). A case definition of clinically probable vasculitic neuropathy in patients lacking biopsy proof incorporated clinical features typical of vasculitic neuropathy: sensory or sensory‐motor involvement, asymmetric/multifocal pattern, lower‐limb predominance, distal‐predominance, pain, acute relapsing course, and non‐demyelinating electrodiagnostic features (Good Practice Points from class II/III evidence). Proposed exclusionary criteria for NSVN – favoring the alternate diagnosis of systemic vasculitic neuropathy – were clinicopathologic evidence of other‐organ involvement; anti‐neutrophil cytoplasmic antibody (ANCAs); cryoglobulins; sedimentation rate ≥100 mm/h; and medical condition/drug predisposing to systemic vasculitis (Good Practice Points supported by class III evidence). Three class III studies on treatment of NSVN were
ISSN:1085-9489
1529-8027
DOI:10.1111/j.1529-8027.2010.00281.x