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Peripheral neuropathy in systemic vasculitides.
Christian Pagnoux1, Loïc Guillevin
1Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris V, Paris, France. christian.pagnoux@psl.ap-hop-paris.fr
Current Opinion in Rheumatology
|December 18, 2004
Summary
Peripheral nervous system (PNS) involvement is common in systemic vasculitides, offering diagnostic clues without indicating a poor prognosis. Recent research clarifies the pathogenesis and treatment of vasculitic neuropathy.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Peripheral nervous system (PNS) involvement is frequent and often an early sign in systemic vasculitides.
- Despite a blood-nerve barrier, PNS damage occurs, though it does not predict a worse prognosis.
Purpose of the Study:
- To review recent advances in understanding the pathogenesis of PNS involvement in systemic vasculitides.
- To summarize current clinical findings and therapeutic strategies for vasculitic neuropathy.
Main Methods:
- Review of recent scientific literature on vasculitic neuropathy.
- Analysis of pathogenetic mechanisms, including blood-nerve barrier function and inflammatory processes.
- Evaluation of diagnostic and therapeutic approaches.
Main Results:
- The blood-nerve barrier is less effective than the blood-brain barrier, allowing inflammatory infiltration and ischemic axonal injury.
- Proinflammatory cytokines and other factors breach the barrier, contributing to nerve damage.
- Current animal models do not support a role for antineutrophil cytoplasmic autoantibodies in PNS involvement.
Conclusions:
- PNS involvement is rarely the only sign of systemic necrotizing vasculitis; other manifestations often suggest the diagnosis.
- Recent advances have improved understanding of vasculitic neuropathy pathogenesis and treatment.
- Isolated PNS involvement may be treated with corticosteroids as a first-line therapy.