Immunosuppressive treatment for non-systemic vasculitic neuropathy

Vrancken, Alexander F.J.E.; Hughes, Richard; Saïd, Gérard; Wokke, John H. J.; Notermans, Nicolette C.

doi:10.1002/14651858.cd006050.pub2

Cited by 18 publications

(3 citation statements)

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“…Similarly, a 2007 Cochrane review revealed no randomized controlled clinical trials have been performed on which to base immunosuppressive treatment for non-systemic vasculitic neuropathy. 67 In the largest observational study in this population (48 patients followed for at least 6 months), 61% of patients treated with corticosteroids monotherapy had a long term response whereas 95% of patients treated with corticosteroids in combination with another immunosuppressive agent, usually cyclophosphamide, had a long term response. 4 Further high quality treatment trials are needed to determine the appropriate initial and long term therapeutic strategy in patients with systemic and non-sytemic vasculitic neuropathy.…”

Section: Disease Modifying Therapymentioning

confidence: 94%

The Importance of Rare Subtypes in Diagnosis and Treatment of Peripheral Neuropathy

et al. 2015

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Importance-Peripheral neuropathy is a prevalent condition that usually warrants a thorough history and examination, but limited diagnostic evaluation. Rare localizations of peripheral neuropathy, however, often require more extensive diagnostic testing and different treatments.Objective-To describe rare localizations of peripheral neuropathy, including the appropriate diagnostic evaluation and available treatments.Evidence Review-References were identified from PubMed searches with an emphasis on systematic reviews and randomized clinical trials. Articles were also identified through the use of the author's own files. Search terms included common rare neuropathy localizations and their causes, as well as epidemiology, pathophysiology, diagnosis, and treatment.Findings-Diffuse, non-length dependent neuropathies, multiple mononeuropathies, polyradiculopathies, plexopathies, and radiculoplexus neuropathies are rare peripheral neuropathy localizations that often require extensive diagnostic testing. Atypical neuropathy features, such as acute/subacute onset, asymmetry, and/or motor predominant signs, are frequently present. The most common diffuse, non-length dependent neuropathies are Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and amyotrophic lateral sclerosis (ALS). Effective disease modifying therapies exist for many diffuse, non-length dependent neuropathies including GBS, CIDP, MMN, and some paraprotein-associated demyelinating neuropathies. Vasculitic neuropathy (multiple mononeuropathy) also has efficacious treatment options, but definitive evidence of a treatment effect for IgM anti-MAG neuropathy and diabetic amyoptrophy (radiculoplexus neuropathy) is lacking.Conclusions and Relevance-Recognition of rare localizations of periperhal neuropathy is essential given the implications for diagnostic testing and treatment. Electrodiagnostic studies are

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Section: Disease Modifying Therapymentioning

confidence: 94%

The Importance of Rare Subtypes in Diagnosis and Treatment of Peripheral Neuropathy

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

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240

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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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“…Vaskulitische Neuropathien kommen als Organmanifestation einer systemischen Vaskulitis vor oder als "nicht systemische Vaskulitis des peripheren Nerven" (NSVN), wobei im letzteren Fall Haut und Muskel mitbetroffen sein können. Zur Therapie der NSVN liegen keine RCTs vor (Collins et al 2010;Vrancken et al 2007). Die wenigen kleinen Studien zur Cryoglobulinassoziierten Vaskulitis ließen keine Aussage zur Therapie zu (Benstead et al 2014).…”

Section: Vaskulitische Neuropathienunclassified