Non-systemic vasculitic neuropathy ('isolated vasculitic neuropathy') is a rare disease causing progressive, disabling, painful loss of sensation with muscle weakness in arms and legs that is often asymmetrical. The diagnosis is made through the finding of inflammation of blood vessels confined to nerve or muscle tissue, or both, and by excluding other conditions that could be the cause, such as systemic vasculitis (inflammation of blood vessels affecting multiple organs in the body), rheumatic diseases, or any other cause for the neuropathy. This is an updated review. No randomised controlled clinical trials have been performed on which to base immunosuppressive treatment for non-systemic vasculitic neuropathy. Blinded randomised trials of adding other immunosuppressive agents to corticosteroids are needed. Such trials will need sufficiently long follow-up periods to evaluate treatment efficacy with disability outcome measures designed and validated for assessing change in immune-mediated inflammatory neuropathies.
Read the full abstract
Non-systemic vasculitic neuropathy is a rare disabling disease that usually has a subacute onset of progressive or relapsing-remitting sensory or sensorimotor deficits. Asymmetry, pain and weakness are key features. The diagnosis can only be made by exclusion of other causes, the absence of systemic vasculitis or other rheumatic diseases, and the demonstration of vasculitis in a nerve or a combined nerve and muscle biopsy. There is a need for efficacious therapy to prevent disease progression and improve prognosis. This is an updated review.
Objectives
To assess if immunosuppressive treatment in non-systemic vasculitic neuropathy reduces disability, and ameliorates neurological symptoms, and if such therapy can be given safely.
Search strategy
The Cochrane Neuromuscular Disease Group Specialized Register was searched again in May 2011, The Cochrane Central Register of Controlled Trials CENTRAL in The Cochrane Library, Pubmed, EMBASE and Web of Science/ISI Web of Knowledge were searched again in March 2011. Because non-systemic vascular neuropathy was first recognised in the 1980s, the searches were done from January 1980 onwards. In addition, the reference lists of retrieved articles relevant to and cited in this review were handsearched.
Selection criteria
All randomised or quasi-randomised trials that examined the efficacy of immunosuppressive treatment for non-systemic vasculitic neuropathy at least one year after the onset of therapy were sought. Participants had to fulfil the following criteria: absence of systemic or neurological disease, exclusion of any recognised cause of the neuropathy by appropriate clinical or laboratory investigations, electrophysiological studies in agreement with axonal neuropathy, confirmation of vasculitis in a nerve or a combined nerve and muscle biopsy. The primary outcome was to be improvement in disability. Secondary outcomes were to be change in the mean disability score, change in muscle strength measured with the Medical Research Council sum score, change in pain or other positive sensory symptoms, number of relapses, and adverse events.
Data collection and analysis
Two authors independently reviewed and extracted details of all potentially relevant trials. For included studies pooled risk ratios and pooled standardised mean differences were to be calculated to assess treatment efficacy.
Main results
Thirty studies were identified and assessed for possible inclusion in the review, but all were excluded because of insufficient quality or lack of relevance.
Authors' conclusions
No adequate randomised or quasi-randomised controlled trials have been performed on which to base treatment for non-systemic vasculitic neuropathy. Randomised trials are needed, especially of adding other immunosuppressive agents to corticosteroids.
