Peripheral neuropathy in systemic vasculitides

Pagnoux, Christian; Guillevin, Loı̈c

doi:10.1097/01.bor.0000145518.91595.2f

Cited by 76 publications

(47 citation statements)

References 71 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Prednisone has been the accepted initial therapy for SVN and NSVN for decades but has never been subjected to a randomized controlled trial [5,8,28,30,31,33••, 53,54, Class III]. Most patients observe improved strength and less pain after several weeks to months.…”

Section: Prednisonementioning

confidence: 96%

Therapy for Vasculitic Neuropathies

Gorson

2006

Curr Treat Options Neurol

View full text Add to dashboard Cite

The term vasculitis refers to a pathologic condition defined by inflammatory cell infiltration and destruction of blood vessels. Systemic vasculitis is classified as primary (eg, polyarteritis nodosa, Churg-Strauss syndrome) or secondary, the latter associated with connective tissue disorders, infections, medications, and rarely, as a paraneoplastic phenomenon. Neuropathy is a common complication of systemic vasculitis and is related to ischemic nerve fiber damage with axon loss. Peripheral neuropathy may be the sole manifestation of vasculitis, a condition termed nonsystemic vasculitic neuropathy (NSVN). Treatment of vasculitic neuropathy requires long-term immunosuppressive therapies with potential side effects. The diagnosis of vasculitis should be established by tissue (preferably nerve) biopsy. High-dose prednisone is the standard platform therapy for patients with systemic and NSVN; for those with systemic vasculitis, at least 3 to 12 months of treatment with cyclophosphamide (monthly intravenous pulse or daily oral therapy) is also necessary to sustain remission and allow successful prednisone tapering. The use of cyclophosphamide in patients with NSVN is controversial, but recent retrospective data suggest that those treated with prednisone and cyclophosphamide from the outset fare better than those initially treated only with prednisone. If prednisone is administered as monotherapy, cyclophosphamide should be added after several months if there is no improvement or relapse occurs with tapering of prednisone. Intravenous pulse and daily oral cyclophosphamide probably offer similar efficacy, although the risk of complications is greater with oral therapy. Azathioprine can be safely substituted for cyclophosphamide after 3 months without an increased relapse rate. Azathioprine, methotrexate, intravenous immune globulin, mycophenolate mofetil, plasma exchange, and rituximab can be offered to patients who are intolerant or have a contraindication to cyclophosphamide. However, efficacy is unproven for any of these therapies. Interferon-alpha, sometimes combined with plasma exchange, is used to treat vasculitis associated with hepatitis B infection. Some patients also may improve with corticosteroids. The classification of diabetic lumbosacral radiculoplexus neuropathy as a vasculitic disorder remains controversial. However, there is compelling pathological evidence that this condition represents a T-cell-mediated microvasculitis. Some patients treated with intravenous corticosteroids may have greater recovery and improved pain control.

show abstract

Section: Prednisonementioning

confidence: 96%

Therapy for Vasculitic Neuropathies

Gorson

2006

Curr Treat Options Neurol

View full text Add to dashboard Cite

show abstract

“…Infiltration of the vascular wall and resulting damage facilitates thrombosis and subsequent ischemia. Damage to the blood-nerve-barrier is induced by pro-inflammatory cytokines, oxidative stress-derived molecules, or metallo-proteinases [7]. Altered expression and function of adhesion molecules or leukocytes (primary T-cell) and endothelial cell activation play a key role in the pathogenesis [8,9].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of Henoch Schönlein Purpura Presenting as Mononeuritis Multiplex

Goyal¹,

Mohan²,

Sehgal³

2016

Arc Cas Rep CMed

View full text Add to dashboard Cite

IgA vasculitis [Henoch Schönlein Purpura (HSP)] is characterized by leukocytoclastic vasculitis involving the small vessels with deposition of immune complexes that contain IgA. Clinical signs include purpura, arthralgia, glomerulonephritis and gastrointestinal involvement. HSP with nervous system involvement is very uncommon. We report a 12 year old boy with IgA vasculitis who presented with typical rash and Mononeuritis Multiplex and responded well to steroid therapy. HSP presenting as Mononeuritis Multiplex has rarely been reported in children. Clinicians must know of this rare complication of HSP which, when present, may be well managed with steroids.

show abstract

“…Coronary lesions, in particular aneurysms resulting in myocardial infarction, sudden death or ischemic heart disease, are the main cause of mortality, and their prevention is the most important goal of therapy. Extreme irritability, rather frequently occurring and more severe than in other febrile illnesses may indicate CNS Frequencies of neurological involvement according to [9,31,71,84]. Suggested potential mechanisms of vessel damage are listed [88]: TC/GF pathogenic T lymphocyte responses and granuloma formation; ICF pathogenic immune complex formation and/or deposition a small, medium and large vessels; b may be higher; c frequent overlap of small-and medium-sized blood-vessel involvement; d earlier designations: hypersensitivity vasculitis or cutaneous leucocytoclastic vasculitis; e vasculitis represents only one (infrequent) mechanism 68,89,93].…”

Section: Ivig In Vasculitis ■ Kawasaki Diseasementioning

confidence: 99%

Intravenous immunoglobulin treatment in vasculitis and connective tissue disorders

Steinbrecher

Berlit

2006

J Neurol

View full text Add to dashboard Cite

Vasculitis syndromes and connective tissue disorders are heterogeneous and mostly rare multisystem disorders with various autoimmune mechanisms driving tissue inflammation and remodeling, ischemic and hemorrhagic tissue damage. While the nervous system can be affected by most of these diseases, the pathogenesis for neural involvement is often ambiguous and elusive for the clinician. Intravenous immunoglobulins (IVIG) have been used for the treatment of most of these disorders. However, a thorough review of the literature indicates that the role for IVIG has to be discussed for individual entities, has often only anecdotal evidence, and is particularly hard to define with respect to neurological manifestations. This review gathers the available evidence on the efficacy of IVIG in neurologically relevant rheumatic diseases, leading to recommendations for their clinical use.

show abstract

Peripheral neuropathy in systemic vasculitides

Cited by 76 publications

References 71 publications

Therapy for Vasculitic Neuropathies

Therapy for Vasculitic Neuropathies

An Unusual Case of Henoch Schönlein Purpura Presenting as Mononeuritis Multiplex

Intravenous immunoglobulin treatment in vasculitis and connective tissue disorders

Contact Info

Product

Resources

About