Nonsystemic vasculitic neuropathy: insights from a clinical cohort. Neurology 2003 Sep 09;61(5):623-30
Date
09/10/2003Pubmed ID
12963752DOI
10.1212/01.wnl.0000082715.48844.3eScopus ID
2-s2.0-0042836678 (requires institutional sign-in at Scopus site) 155 CitationsAbstract
BACKGROUND: Nonsystemic vasculitic neuropathy (NSVN) is an uncommon disorder. Few series with small numbers of patients have been reported. The prognosis and treatment of patients presenting with NSVN remain uninvestigated. The authors sought to address these issues by assembling a large retrospective cohort with extended follow-up.
METHODS: All nerve biopsies performed over 20 years were reviewed; cases with definite, probable, or possible vasculitis were segregated for clinical correlation. Patients satisfying clinical criteria for NSVN at presentation were selected. Clinicopathologic, treatment, and outcome measures were analyzed in patients followed for > or = 6 months.
RESULTS: A total of 48 patients (30 women, 18 men) with a median of 63 months of follow-up were identified. Most patients (85%) had extensive, overlapping involvement of multiple nerves. Only one had a symmetric polyneuropathy. Most neuropathies (96%) were painful. In 96%, nerve damage was distally accentuated, but most had concurrent proximal weakness. Diagnostic sensitivity was 58% for superficial peroneal nerve/peroneus brevis muscle biopsy and 47% for sural nerve biopsy. Combination corticosteroid/cytotoxic therapy was more effective than corticosteroid monotherapy in inducing remission and improving disability, with trends toward reduced relapses and chronic pain. Treatment with cyclophosphamide for >6 months decreased the relapse rate, which was 46% for all patients. Disease/treatment-related mortality was 10%. Six percent developed cutaneous involvement. Although chronic pain persisted in 60% of survivors, 80% had good outcomes.
CONCLUSIONS: NSVN nearly always presents as an asymmetric, distally accentuated, painful, sensorimotor polyneuropathy. Risks for systemic spread and death are small, and, aside from pain, neurologic prognosis is unexpectedly good. Although this was not a randomized controlled trial, combination therapy produced the best outcome in this cohort.
Author List
Collins MP, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JTAuthor
Michael P. Collins MD Professor in the Neurology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultAged
Cohort Studies
Drug Therapy, Combination
Female
Follow-Up Studies
Humans
Male
Methylprednisolone
Middle Aged
Polyneuropathies
Prognosis
Recurrence
Retrospective Studies
Treatment Outcome
Vasculitis
Weight Loss
