Idiopathic hypocomplementemic urticarial vasculitis-linked neuropathy

Filosto, Massimiliano; Cavallaro, Tiziana; Pasolini, G.; Broglio, Laura; Tentorio, Marta; Cotelli, Maria Sofia; Ferrari, Sergio; Padovani, Alessandro

doi:10.1016/j.jns.2009.03.027

Cited by 12 publications

(5 citation statements)

References 17 publications

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“…Hypocomplementemic urticarial vasculitis (HUV) is a kind of vasculitis accompanied by urticaria and hypocomplementemia affecting cutaneous small vessels and is associated with anti-C1q antibodies, commonly causing urticaria and multiorgan involvement such as glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation (10). HUV may be induced by connective tissue diseases, infections (such as hepatitis B, hepatitis C and infectious mononucleosis), neoplasms and drugs; however, most cases of HUV are idiopathic (88). Neurological involvement has been occasionally reported as pseudotumor cerebri (89), lower cranial nerve (VIII, IX, and X) palsies (90) and peripheral nerve involvement such as asymmetrical multifocal axonal sensory neuropathy.…”

Section: Medium-vessel Vasculitismentioning

confidence: 99%

Neurological Involvement in Primary Systemic Vasculitis

2019

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Primary systemic vasculitis can affect every structure in both the central and peripheral nervous system, causing varied neurological manifestations of neurological dysfunction. Early recognition of the underlying causes of the neurological symptoms can facilitate timely treatment and improve the prognosis. This review highlights the clinical manifestations of primary systemic vasculitis in the nervous system.

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Section: Medium-vessel Vasculitismentioning

confidence: 99%

Neurological Involvement in Primary Systemic Vasculitis

2019

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“…Systemic vasculitic neuropathies (SVNs) occur with varying degrees of frequency in numerous vasculitides 2,[5][6][7][8] . Histopathologically proven vasculitic neuropathy has not been reported in Kawasaki disease, Takayasu arteritis, anti-glomerular basement membrane disease or Cogan syndrome 4 .…”

Section: Classification Of the Vasculitidesmentioning

confidence: 99%

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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“…The disease is categorized according to levels of complement as either normo- or hypocomplementemic. Both subtypes may be idiopathic, but the hypocomplementemic variant tend to have a more severe course and is often associated to systemic inflammatory disease ( 9 ). In particular, an association between HUV and systemic lupus erythematosus has frequently been reported ( 8 , 10 , 11 ).…”

Section: Discussionmentioning

confidence: 99%