Elsevier

The Lancet Neurology

Volume 4, Issue 12, December 2005, Pages 853-865
The Lancet Neurology

Review
An update on the classification and treatment of vasculitic neuropathy

https://doi.org/10.1016/S1474-4422(05)70249-0Get rights and content

Summary

Vasculitic neuropathy usually presents with painful mononeuropathies or an asymmetric polyneuropathy of acute or subacute onset. The disorder should be classified as being systemic or non-systemic. Systemic vasculitis should be further classified into one of the primary and secondary forms. Although specific treatment regimens vary among neurologists, basic principles can be applied. Corticosteroids and cytotoxic drugs have been the mainstay of treatment for most forms of vasculitic neuropathy. Here we discuss dosing, potential side-effects, and management recommendations of conventional treatments. New treatments showing promise include intravenous immunoglobulin and biological agents and trials of the newest treatments are being reviewed. Future trials should compare commonly used treatment regimens and better establish the efficacy of newer, potentially safer, treatments.

Introduction

The various forms of vasculitis consist of a heterogeneous group of disorders that can affect different organ systems and blood vessel diameters. Many forms of vasculitis commonly affect the peripheral nervous system. There are several schemes aimed at classifying the vasculitides, and different therapeutic strategies are currently in use. The purpose of this review is to bring the neurologist up-to-date with current classification and treatment of the most common forms of vasculitic neuropathy, and to discuss future potential targets in the inflammatory pathway.

Section snippets

Classification

Classification of the vasculitides has become increasingly complex over the past half-century.1, 2, 3 The systemic vasculitides are divided into primary systemic disorders, for which there is no known cause, and secondary systemic disorders, for which a virus, drug, or connective-tissue disease causes inflammation of the vessel wall.4 Although the exact triggering events in primary vasculitis are unknown, altered expression and function of adhesion molecules and leucocyte and endothelial cell

Clinical and diagnostic characteristics

The clinical characteristics of systemic and non-systemic vasculitic neuropathies tend to be consistent with the pathophysiology—ie, acute to subacute onset of painful sensory or sensorimotor deficits. The most common presentations are of multiple mononeuropathies or asymmetric polyneuropathy. Occasionally, the progression of mononeuropathies is so rapid that on presentation the deficits can be confused with those of polyneuropathies.19 For this reason, it is imperative that patients are asked

Treatment

An important role of neurologists in the management of systemic vasculitic neuropathy is the assessment of clinical response, especially in terms of neuropathic impairment. Treatment decisions should be made in consultation with rheumatologists or physicians, and are based in part on the form of systemic vasculitis, extent and degree of organ involvement, prior responsiveness to any treatments, and presence or absence of viral infection. For example, immunosuppressive drugs, which can be

Conclusion

Because neurologists play an integral part in the diagnosis and management of patients with vasculitis affecting the peripheral nerves, they need to understand the classification and treatment options and indications for these diseases. Provision of care for patients with vasculitic neuropathy also includes appropriate surveillance of potential complications of treatments. Although corticosteroids remain the mainstay of treatment for non-viral systemic vasculitic neuropathy, current treatments

Search strategy and selection criteria

References for this review were identified by searches of MEDLINE up until September, 2005, and references from relevant articles. Many articles were also identified through searches of the extensive files of the authors. The search terms “vasculitis”, “vasculitic neuropathy”, “polyarteritis nodosa”, “Wegener's granulomatosis”, “Churg-Strauss syndrome”, “microscopic polyangiitis”, “hepatitis”, “cryoglobulinemia”, “diabetic amyotrophy”, and “diabetic lumbosacral radiculoplexus neuropathy”

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      Treatment should be initiated rapidly with high dose IV and oral corticosteroids (e.g. 1 mg/kg/day) followed by the addition of a steroid sparing agent (e.g. cyclophosphamide oral or IV) [191,195]. Azathioprine and methotrexate may be use as maintenance therapy or in patients who develop toxicity with cyclophosphamide [195,196]. Although not useful for most SS-related neuropathies, IV rituximab showed benefit in 11 of 17 patients enrolled in a prospective cohort registry study in France [513].

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