Nonsystemic necrotizing vasculitis of the peripheral nervous system

Rosenbaum, J. T.; Bell, L. A.; Rosenbaum, Richard B.

doi:10.1002/1529-0131(199807)41:7<1324::aid-art27>3.0.co;2-c

Cited by 10 publications

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“…Vasculitic neuropathy is routinely considered in the differential diagnosis of cryptogenic axonal neuropathies with asymmetric, multifocal, and progressive features ( Vrancken et al, 2004 ) . While the disorder is typically subacute in evolution, some patients are symptomatic for many years prior to diagnosis ( Rosenbaum et al, 1998 ; Collins et al, 2003 ) . Although most vasculitic neuropathies exhibit a stepwise clinical course, one‐third of patients present in a progressive fashion ( Collins et al, 2000 ; Bennett et al, 2008 ) .…”

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

242

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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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

242

256

View full text Add to dashboard Cite

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Necrotizing vasculitis confined to the nerves: Comment on the concise communication by Rosenbaum et al

Handa

Aggarwal

Wali

1999

Arthritis & Rheumatism

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LETTERS Necrotizing vasculitis confined to the nerves: comment on the concise communication by Rosenbaum et alTo the Editor: We read with interest the concise communication by Rosenbaum et al (1) on nonsystemic necrotizing vasculitis of the peripheral nervous system (PNS). While agreeing that vasculitis limited to the PNS has been reported mainly in the neurology literature, we would like to draw the authors' attention to a report in the rheumatology literature about acute necrotizing vasculitis confined to the nerve, with spontaneous recovery (2). A 65-year-old man had presented with leg pains, fever, arthralgias, and weight loss. Muscle enzyme levels were normal. Electrophysiologic studies revealed a symmetric and diffuse polyneuropathy of all 4 limbs, with moderate slowing of motor nerve conduction velocity. A peroneal nerve biopsy revealed a necrotizing vasculitis, while muscle biopsy findings were normal. Interestingly, the patient recovered spontaneously in a few weeks without the use of corticosteroids or other immunosuppressive therapy.We also take this opportunity to describe a similar 21-year-old man we encountered recently. This patient presented with bilateral wasting of the hand muscles and fever. Antinuclear antibodies, rheumatoid factor, antineutrophil cytoplasmic antibodies, cryoglobulins, and hepatitis B and C serology findings were negative. Urinalysis results were normal, and magnetic resonance imaging of the cervical spine was unremarkable. Electrophysiologic studies revealed asymmetric sensorimotor neuropathy of the mixed axonal and demyelinating type. Sural nerve biopsy showed necrotizing vasculitis. Findings of angiography of the renal, celiac, and superior mesenteric arteries were normal. The patient made a significant recovery when treated with oral corticosteroids and was doing well at 6-month followup.Although muscle biopsy is said to be superior to nerve biopsy for diagnosing isolated neuropathy from necrotizing vasculitis (3), the muscle biopsy findings in the patient described by de la Sayette et al (2) were normal while the peroneal nerve biopsy results confirmed the presence of vasculitis. Our own patient was found to have necrotizing vasculitis by sural nerve biopsy; muscle biopsy was not performed.In addition to vasculitis confined to the skin and nerves, polyarteritis confined to the calf muscles has also been reported (4,5). As of 1993, 9 such patients had been described in the literature (5).Awareness of the existence of limited forms of systemic necrotizing vasculitides helps avoid unnecessary immunosuppressive therapy in such patients.

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Necrotizing vasculitis of the peripheral nervous system: Nonsystemic or clinically undetectable?

Puéchal

Saïd

1999

Arthritis & Rheumatism

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LETTERS Necrotizing vasculitis confined to the nerves: comment on the concise communication by Rosenbaum et al To the Editor:We read with interest the concise communication by Rosenbaum et al (1) on nonsystemic necrotizing vasculitis of the peripheral nervous system (PNS). While agreeing that vasculitis limited to the PNS has been reported mainly in the neurology literature, we would like to draw the authors' attention to a report in the rheumatology literature about acute necrotizing vasculitis confined to the nerve, with spontaneous recovery (2). A 65-year-old man had presented with leg pains, fever, arthralgias, and weight loss. Muscle enzyme levels were normal. Electrophysiologic studies revealed a symmetric and diffuse polyneuropathy of all 4 limbs, with moderate slowing of motor nerve conduction velocity. A peroneal nerve biopsy revealed a necrotizing vasculitis, while muscle biopsy findings were normal. Interestingly, the patient recovered spontaneously in a few weeks without the use of corticosteroids or other immunosuppressive therapy.We also take this opportunity to describe a similar 21-year-old man we encountered recently. This patient presented with bilateral wasting of the hand muscles and fever. Antinuclear antibodies, rheumatoid factor, antineutrophil cytoplasmic antibodies, cryoglobulins, and hepatitis B and C serology findings were negative. Urinalysis results were normal, and magnetic resonance imaging of the cervical spine was unremarkable. Electrophysiologic studies revealed asymmetric sensorimotor neuropathy of the mixed axonal and demyelinating type. Sural nerve biopsy showed necrotizing vasculitis. Findings of angiography of the renal, celiac, and superior mesenteric arteries were normal. The patient made a significant recovery when treated with oral corticosteroids and was doing well at 6-month followup.Although muscle biopsy is said to be superior to nerve biopsy for diagnosing isolated neuropathy from necrotizing vasculitis (3), the muscle biopsy findings in the patient described by de la Sayette et al (2) were normal while the peroneal nerve biopsy results confirmed the presence of vasculitis. Our own patient was found to have necrotizing vasculitis by sural nerve biopsy; muscle biopsy was not performed.In addition to vasculitis confined to the skin and nerves, polyarteritis confined to the calf muscles has also been reported (4,5). As of 1993, 9 such patients had been described in the literature (5).Awareness of the existence of limited forms of systemic necrotizing vasculitides helps avoid unnecessary immunosuppressive therapy in such patients.

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Nonsystemic necrotizing vasculitis of the peripheral nervous system

Cited by 10 publications

References 10 publications

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

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

Necrotizing vasculitis confined to the nerves: Comment on the concise communication by Rosenbaum et al

Necrotizing vasculitis of the peripheral nervous system: Nonsystemic or clinically undetectable?

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