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”
ReviewAn update on the classification and treatment of vasculitic neuropathy
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
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2019, Clinical ImmunologyCitation Excerpt :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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