Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: A comparative study

Sugiura, Makoto; Koike, Haruki; Iijima, Masahiro; Mori, Keiko; Hattori, Naoki; Katsuno, Masahisa; Tanaka, Fumiaki; Sobue, Gen

doi:10.1016/j.jns.2005.10.018

Cited by 65 publications

(44 citation statements)

References 29 publications

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“…PNS microvasculitis with vascular damage is characteristic of the diabetic radiculoplexus neuropathies 11 . However, data from at least four studies that evaluated the size of vessels involved in NSVN [12][13][14][15] indicate that NSVN is not, in most circumstances, a microvasculitis.…”

Section: Classification Of the Vasculitidesmentioning

confidence: 99%

“…Approximately 5-10% of patients present with acute, rapidly progressive deficits and are diagnosed within 1 month, but most patients progress subacutely or chronically 30,31,[35][36][37] . Median duration of symptoms at diagnosis is 2.5-12 months 13,19,31,[35][36][37][38] . In one study, the median delay to diagnosis was 2 years; combined with the relatively mild neurological involvement in this cohort, this delay suggests a bias against inclusion of more severely affected patients 30 .…”

Section: Classification Of the Vasculitidesmentioning

confidence: 99%

“…On the basis of data from multiple studies, the estimated sensitivities of sural nerve biopsy alone and superficial peroneal nerve biopsy combined with peroneus brevis muscle biopsy for definite NSVN are both ~50% 3,13,14,19,[35][36][37][116][117][118][119] .…”

Section: Brighton Collaboration Case Definition Of Vasculitic Neuropamentioning

confidence: 99%

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The nonsystemic vasculitic neuropathies

2017

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The vasculitides are a clinically diverse group of diseases with the histopathological signature of blood vessel-centred inflammation that results in vascular damage and ischaemic injury to the affected tissues 1 . Vasculitis can be caused by drugs, infections and cancers, but diagnostic workup reveals no trigger in most patients. Autoimmune mechanisms are active in all vasculitides, except those caused by direct infection of vessel walls. Most vasculitides are systemic and involve multiple organs and tissues. Those affecting small-to medium-sized vessels often manifest with a vasculitic neuropathy. For example, neuropathies occur in 60-70% of patients with polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis and cryoglobulinaemic vasculitis, and 40-50% of those with microscopic polyangiitis and rheumatoid vasculitis 2 .Vasculitic neuropathy can also occur without systemic vasculitis. This single-organ vasculitis of the PNS has commonly been referred to as nonsystemic vasculitic neuropathy (NSVN), but other forms of clinically isolated PNS vasculitis are now recognized and can be considered variants of NSVN 3 . Although NSVN is classically an acute, relapsing, multifocal neuropathy, it can present as a slowly progressive neuropathy without distinct asymmetries. As such, it should be considered as a possible cause of any progressive axonal neur opathy. NSVN can be diagnosed only with a nerve biopsy, with or without a concomitant muscle or skin biopsy; as nerve biopsies are seldom performed, the condition is under-recognized. The disorder must be distinguished from many other causes of multifocal neuropathy, the definition of which is itself not standardized and requires clarification.A Peripheral Nerve Society guideline group reviewed the literature on vasculitic neuropathy and published consensus recommendations on the classification, diagnosis and immunosuppressive treatment of NSVN in 2010 (REF. 4) Abstract | Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and -arguably -neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard defi...

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Section: Classification Of the Vasculitidesmentioning

confidence: 99%

Section: Classification Of the Vasculitidesmentioning

confidence: 99%

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The nonsystemic vasculitic neuropathies

2017

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“…This observation suggests that functional disabilities in patients with NSVN were attributed mainly to motor deficits. A higher number of NSVN patients presented sensory disturbance as an initial symptom [1], followed later by motor dysfunction. The reason why patients who presented foot drop before treatment were less responsive to therapy may be due to the fact that their neuropathies were too advanced to be reversible when motor deficits manifested.…”

Section: Discussionmentioning

confidence: 99%

“…Systemic vasculitis can be classified into two categories - primary disorders including polyarteritis nodosa, Churg-Strauss syndrome and granulomatosis with polyangiitis (Wegener's), and disorders involving a secondary process including systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, infection, malignant neoplasia, and cryoglobulinemia [1]. As Kernohan and Woltman [2] reported in 1938, some patients develop vasculitis that is clinically restricted to the peripheral nervous system.…”

Section: Introductionmentioning

confidence: 99%

Gait Disturbance due to Foot Drop Is Refractory to Treatment in Nonsystemic Vasculitic Neuropathy

et al. 2014

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Background: Nonsystemic vasculitic neuropathy (NSVN) is a vasculitis syndrome clinically restricted to the peripheral nervous system. Although treatment may improve prognosis, daily activities of such patients after treatment have not been well studied. Methods: We evaluated clinical features, laboratory data, nerve conduction, and sural nerve biopsy findings for 16 unbiased consecutive patients with NSVN. Results: Initial symptoms included neuropathic pain (31%) and lower limb sensory disturbance (19%). The mean duration between disease onset and initial treatment was 4.1 ± 4.8 months. Mean modified Rankin scale scores were 3.13 at hospital admission and 2.69 at final follow-up. The poor outcome group had significantly decreased compound muscle action potentials of peroneal nerves and significantly more patients presenting with foot drop compared with the good outcome group. No other significant differences were found. Conclusion: Pretreatment foot drop signaled poor outcome in daily activities of patients with NSVN, and earlier treatment may be critical for these patients.

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