Epineurial microvasculitis in proximal diabetic neuropathy

Llewelyn, J. G.; Thomas, P. K.; King, R. H. M.

doi:10.1007/s004150050197

Cited by 108 publications

(51 citation statements)

References 31 publications

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“…Unlike NSVN with proximal involvement, the diabetic radiculoplexus neuropathies are always self-limited and develop in a stereotypical manner, commencing in one region and spreading to another, even as the first site is resolving 11,[49][50][51][52][53][54][55][56][57][58] . The mechanisms responsible for this distinctive behaviour of diabetesrelated vasculitis are poorly understood.…”

Section: Variantsmentioning

confidence: 99%

“…Diabetic radiculoplexus neuropathy has a propensity to affect lumbosacral nerve roots and peripheral nerve trunks and is then labelled as diabetic lumbosacral radiculoplexus neuropathy (DLSRPN) 11,[49][50][51][52][53][54][55][56][57][58] . DLSRPN affects 1% of patients with diabetes mellitus 59 , typically type 2.…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

“…ESR is elevated in 20% of patients, and CSF protein levels are high in 85%. Symptoms progress for 1 week to 3 years (median four months), then recede over many months (median 15 months) 11,[49][50][51][52][53][54][55][56][57][58] . Residual weakness persists, especially distally, in more than 50% of patients, and 10-15% of patients relapse.…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

“…Residual weakness persists, especially distally, in more than 50% of patients, and 10-15% of patients relapse. Nerve biopsies reveal T-cell predominant perivascular or vascular inflammation of epineurial microvessels and histopathological alterations associated with vasculitic neuropathy, such as asymmetric fibre loss, active axonal degeneration, haemosiderin, and complement deposits in vessel walls; necrotizing vasculitis, however, is rare 11,49,[53][54][55][56][57]60 . Thus, DLSRPN seems to be a PNS microvasculitis.…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

“…Pain is a key feature of DLSRPN, but 5-10% of patients described in the literature had no pain 11,[49][50][51][52][53][54][55][56][57][58] . In a 2011 study of 23 patients with painless DLSRPN (also known as painless diabetic motor neuropathy), almost all had type 2 rather than type 1 diabetes mellitus 62 .…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

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: Variantsmentioning

confidence: 99%

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

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The nonsystemic vasculitic neuropathies

2017

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Skin Denervation in Vasculitic Neuropathy

Lee¹,

Shun²,

Hsieh³

et al. 2005

Arch Neurol

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Background: Skin denervation in vasculitic neuropathy has rarely been documented despite frequent manifestations of small-fiber neuropathy including reduced sensitivity and neuropathic pain. Recently, skin biopsy has been established as a new approach to diagnose smallfiber sensory neuropathy. Objectives: To investigate the pathologic features of cutaneous nerves and to evaluate inflammatory vasculopathy in the skin of patients with vasculitis.

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