BACKGROUND: @entity16 ( @entity16 ) is an uncommon disorder. Few series with small numbers of @entity1 have been reported. The prognosis and treatment of @entity1 presenting with @entity16 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 @entity1333 were segregated for clinical correlation. @entity1 satisfying clinical criteria for @entity16 at presentation were selected. Clinicopathologic, treatment, and outcome measures were analyzed in @entity1 followed for > or = 6 months. RESULTS: A total of 48 @entity1 (30 @entity1 , 18 @entity1 ) with a median of 63 months of follow-up were identified. Most @entity1 (85%) had extensive, overlapping involvement of multiple nerves. Only one had a symmetric @entity305 . Most @entity16 (96%) were @entity158 . In 96%, @entity16 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 @entity228 monotherapy in inducing remission and improving disability, with trends toward reduced relapses and @entity1118 . Treatment with @entity786 for >6 months decreased the relapse rate, which was 46% for all @entity1 . Disease/treatment-related mortality was 10%. Six percent developed cutaneous involvement. Although @entity1118 persisted in 60% of survivors, 80% had good outcomes. CONCLUSIONS: @entity16 nearly always presents as an asymmetric, distally accentuated, @entity158 , @entity305 . Risks for systemic spread and death are small, and, aside from @entity158 , neurologic prognosis is unexpectedly good. Although this was not a randomized controlled trial, combination therapy produced the best outcome in this cohort.

XXXX : insights from a clinical cohort.

@entity1 :: ('9606', 'Species') :: ['women', 'Patients', 'patients', 'men'] @entity1333 :: ('MESH:D014657', 'Disease') :: ['vasculitis'] @entity16 :: ('MESH:D009422', 'Disease') :: ['Nonsystemic vasculitic neuropathy', 'nerve damage', 'neuropathies', 'NSVN'] @entity228 :: ('CHEBI:50858', 'Chemical') :: ['corticosteroid'] @entity305 :: ('MESH:D011115', 'Disease') :: ['polyneuropathy', 'sensorimotor polyneuropathy'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide'] @entity158 :: ('MESH:D010146', 'Disease') :: ['pain', 'painful'] @entity1118 :: ('MESH:D059350', 'Disease') :: ['chronic pain']
@entity16 :: (MESH:D009422,Disease) :: ['Nonsystemic vasculitic neuropathy']
