Immunosuppressive treatment for non-systemic vasculitic neuropathy

Vrancken, AFJE; Hughes, R. A. C.; Saïd, Gérard; Wokke, J.H.J.; Notermans, N. C.

doi:10.1002/14651858.cd006050

Cited by 5 publications

(6 citation statements)

References 70 publications

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“…No head-to-head studies are available with regard to the efficacy of different immunosuppressive and immunomodulatory drugs [5,26]. Current treatments were derived from the treatment regimens of SV or of other immune-mediated disorders such as myasthenia gravis or chronic inflammatory polyneuropathy [11] where corticosteroids and immunosuppressive drugs are the mainstay of treatment [6].…”

Section: Discussionmentioning

confidence: 99%

“…Although reliable epidemiologic data on the prevalence and incidence of NSVN are not available, NSVN seems to be a rare disease, which may be one reason for the lack of randomized controlled treatment trials [26]. Current guidelines recommend corticosteroid monotherapy as firstline, and immunosuppressive combination treatments for progressive cases.…”

Section: Introductionmentioning

confidence: 99%

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Non-systemic vasculitic neuropathy: single-center follow-up of 60 patients

et al. 2015

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The objective of this study is to report the clinical presentation and long-term outcome of patients with non-systemic vasculitic neuropathy (NSVN) seen at our neuromuscular center. In this retrospective analysis, we assessed medical records of 60 patients with biopsy-proven NSVN (39 men, 21 women; median age: 64 years, 24-80), who were seen at our department between 1999 and 2008 and were followed up until 2014. The initial neurological findings, laboratory and neurophysiological data, treatment regimens, and outcome were analyzed in all patients. NSVN was mostly asymmetric (48/60, 80%), sensorimotor (45/60, 75%), and painful (38/60, 63%), with walking impairment as one major sign (51/60, 85%). No compound action potentials could be recorded in 29/60 (48%) sural nerves (later biopsied side) and in 6/60 (10%) tibial (motor) nerves. Pathology of sural nerve was informative in all cases irrespective of neurophysiological findings and prior immunosuppression. After initial treatment with i.v. methylprednisolone, all patients reported overall improvement. Of the 46 patients who were followed for >1 year, those with mild to moderate affliction were stable with azathioprine (19/46, 41%), while 18/46 (39%) patients were treated with cyclophosphamide and other immunosuppressants due to progression or relapse. At 4 years, 24/46 (52%) patients had either discontinued (n = 21) or had primarily refused immunosuppressive treatment (n = 3) without relapse. Age younger than the group median of 64 years was associated with better outcome. No patient evolved to systemic vasculitis. NSVN is a potentially treatable disorder of the peripheral nervous system.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Non-systemic vasculitic neuropathy: single-center follow-up of 60 patients

et al. 2015

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“…Vasculitic neuropathies are believed to respond to immunosuppressive interventions, but this impression has never been subjected to a systematic analysis of all classes of evidence. For NSVN, a recent Cochrane Systematic Review found no adequate randomized controlled trials of immunosuppressive therapy ( Vrancken et al, 2007 ) . Although many controlled trials of therapy in primary systemic vasculitides have been reported, the relevance of these studies to vasculitic neuropathy is unclear.…”

Section: Introductionmentioning

confidence: 99%

Peripheral Nerve Society Guideline* on the classification, diagnosis, investigation, and immunosuppressive therapy of non‐systemic vasculitic neuropathy: executive summary

Collins

Dyck²,

Gronseth³

et al. 2010

J Peripheral Nervous Sys

240

256

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Non-systemic vasculitic neuropathy (NSVN) is routinely considered in the differential diagnosis of progressive axonal neuropathies, especially those with asymmetric or multifocal features. Diagnostic criteria for vasculitic neuropathy, classification criteria for NSVN, and therapeutic approaches to NSVN are not standardized. The aim of this guideline was to derive recommendations on the classification, diagnosis, investigation, and treatment of NSVN based on the available evidence and, where evidence was not available, expert consensus. Experts on vasculitis, vasculitic neuropathy, and methodology systematically reviewed the literature for articles addressing diagnostic issues concerning vasculitic neuropathy and NSVN as well as treatment of NSVN and the small-to-medium vessel primary systemic vasculitides using MEDLINE, EMBASE, and the Cochrane Library. The selected articles were analyzed and classified. The group initially reached consensus on a classification of vasculitides associated with neuropathy. Non-diabetic radiculoplexus neuropathy was incorporated within NSVN. The consensus definition of pathologically definite vasculitic neuropathy required that vessel wall inflammation be accompanied by vascular damage. Diagnostic criteria for pathologically probable vasculitic neuropathy included five predictors of definite vasculitic neuropathy: vascular deposits of IgM, C3, or fibrinogen by direct immunofluorescence; hemosiderin deposits; asymmetric nerve fiber loss; prominent active axonal degeneration; and myofiber necrosis, regeneration, or infarcts in peroneus brevis muscle biopsy (Good Practice Points from class II/III evidence). A case definition of clinically probable vasculitic neuropathy in patients lacking biopsy proof incorporated clinical features typical of vasculitic neuropathy: sensory or sensory-motor involvement, asymmetric/multifocal pattern, lower-limb predominance, distal-predominance, pain, acute relapsing course, and non-demyelinating electrodiagnostic features (Good Practice Points from class II/III evidence). Proposed exclusionary criteria for NSVN--favoring the alternate diagnosis of systemic vasculitic neuropathy--were clinicopathologic evidence of other-organ involvement; anti-neutrophil cytoplasmic antibody (ANCAs); cryoglobulins; sedimentation rate ≥100 mm/h; and medical condition/drug predisposing to systemic vasculitis (Good Practice Points supported by class III evidence). Three class III studies on treatment of NSVN were identified, which were insufficient to permit a level C recommendation. Therefore, the group reviewed the literature on treatment of primary small-to-medium vessel systemic vasculitides prior to deriving Good Practice Points on treatment of NSVN. Principal treatment recommendations were: (1) corticosteroid (CS) monotherapy for at least 6 months is considered first-line; (2) combination therapy should be used for rapidly progressive NSVN and patients who progress on CS monotherapy; (3) immunosuppressive options include cyclophosphamide, azathioprine, and methotrex...

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“…The optimal treatment of vasculitic neuropathy is not well defined,123 132 and there are no RCTs to guide the clinician 140. Treatment can often be decided in conjunction with specialists with expertise in the management of vasculitis in other systems.…”

Section: Vasculitic Neuropathymentioning

confidence: 99%

Diagnosis and treatment in inflammatory neuropathies

Lunn

Willison

2009

Postgraduate Medical Journal

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The inflammatory neuropathies are a large diverse group of immune-mediated neuropathies that are amenable to treatment and may be reversible. Their accurate diagnosis is essential for informing the patient of the likely course and prognosis of the disease, informing the treating physician of the appropriate therapy and informing the scientific community of the results of well-targeted, designed and performed clinical trials. With the advent of biological therapies able to manipulate the immune response more specifically, an understanding of the pathogenesis of these conditions is increasingly important. This review presents a broad overview of the pathogenesis, diagnosis and therapy of inflammatory neuropathies, concentrating on the most commonly encountered conditions.

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Immunosuppressive treatment for non-systemic vasculitic neuropathy

Cited by 5 publications

References 70 publications

Non-systemic vasculitic neuropathy: single-center follow-up of 60 patients

Non-systemic vasculitic neuropathy: single-center follow-up of 60 patients

Peripheral Nerve Society Guideline* on the classification, diagnosis, investigation, and immunosuppressive therapy of non‐systemic vasculitic neuropathy: executive summary

Diagnosis and treatment in inflammatory neuropathies

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