NECROTIZING PERIPHERAL NERVE VASCULITIS

doi:10.1016/S0733-8619(05)70350-9

Neurologic Clinics

Volume 15, Issue 4, 1 November 1997, Pages 835-848

https://doi.org/10.1016/S0733-8619(05)70350-9 Get rights and content

Necrotizing vasculitis, especially that of the type observed in poly-arteritis nodosa (PAN), affects arteries of the epineurium and induces nerve ischemia when vasa nervorum are occluded. Necrotizing arteritis is associated with symptomatic and asymptomatic nerve lesions owing to ischemia.1, 24, 27 It also occurs secondarily in the course of neuropathy associated with infection, malignancy, and focal diabetic neuropathy. It further causes ischemic nerve lesions on preexisting ones.

The classification of vasculitis is still a matter of controversy because it has often been based on the size of the vessels predominantly affected,⁴⁶ even though overlaps occur. Recent attempts to modify the classification of systemic vasculitides²² have not achieved further clarification. Large vessel vasculitis, which includes giant cell arteritis and Takayasu's arteritis, is seldom associated with peripheral neuropathy, and further, neuropathy is rare in cranial (giant cell) arteritis, and when present, is usually due to lesions of epineurial vessels of the type observed in PAN.5, 36 Of the medium-sized vessel vasculitic disorders, which include PAN and Kawasaki's disease, neuropathy occurs only in PAN. The group of small vessel vasculitis generally includes Wegener's granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis (with involvement of capillaries), which actually in many cases, overlaps with PAN. The nomenclature of the systemic vasculitides remains enigmatic²⁵ owing to the lack of specific biologic markers, except for Wegener's granulomatosis in which the presence of antineutrophil cytoplasmic antibodies (c-ANCA) can be of value.

Section snippets

PERIPHERAL NEUROPATHY OF NECROTIZING ARTERITIS

Necrotizing arteritis is the most common primary vasculitis responsible for vasculitic neuropathy and encompasses local and systemic mechanisms of vascular inflammation that involve large or small vessels; it leads to vessel and tissue necrosis with typical leucocytic infiltration, leucocytoclasia, hemorrhage, and fibrinoid change.⁴⁶ Clinical neuropathy occurs in 50% to 75% of patients with systemic vasculitis of the PAN group.17, 29 Subclinical lesions are probably much more common. A similar

EVOLUTION OF ISCHEMIC NEUROPATHY

Under treatment, mainly with long-term corticosteroids, recovery of motor deficits because of ischemic neuropathy takes months, because the lesions are axonal. In many patients, there is no residual deficit after 6 to 12 months. Sensory loss gradually recedes, but is often incomplete. Residual pain is common and may be difficult to differentiate from a relapse of the neuropathy, especially in patients who lack general manifestations or elevated biological markers of inflammation.

When neuropathy

NEUROPATHY IN WEGENER'S GRANULOMATOSIS

The neurologic manifestations of Wegener's granulomatosis were reviewed retrospectively in 324 patients at the Mayo Clinic.³¹ Overall, 109 patients (33.6%) had neurologic manifestations, including 53 with peripheral neuropathy. A multifocal neuropathy was seen in 42 patients, a distal symmetric polyneuropathy in 6, and an unclassified peripheral neuropathy in 5. Neuropathy was a major presenting symptoms in 8 of 22 patients with mononeuropathy multiplex who underwent detailed evaluation. The

NECROTIZING VASCULITIS IN NEUROPATHY OF OTHER ORIGINS

A secondary vasculitis neuropathy with lymphocytic inflammation occurs in (1) infections, such as retroviral infection, leprosy, and Lyme disease³⁵; (2) the delayed type hypersensitivity reactions of tuberculoid leprosy and reversal reactions in lepromatous neuropathy; (3) lymphoproliferative disorders and other cancers; and (4) occasionally in multifocal diabetic neuropathy.

INDICATION OF NERVE BIOPSY FOR DIAGNOSIS OF VASCULITIS

Vasculitis is one of the most treatable forms of neuropathy, and its diagnosis should not be missed. It is unmistakable when acute or subacute multifocal neuropathy occurs in the context of multisystem involvement, including renal insufficiency, ischemic lesions of the skin, especially of the lower extremities, asthma, or eosinophilia. In such patients the diagnosis of necrotizing vasculitis is easily suspected clinically and confirmed by nerve and muscle biopsy. In older patients with

TREATMENT

With the use of corticosteroids in treatment of PAN, the 5-year survival decreased from 13% in the control group to 48% in treated patients.¹⁵ It is widely agreed that one can start with prednisone at 1 mg/kg/d, although some start with 2 mg/kg/d. High doses of corticosteroids may not be well tolerated in the elderly. Simultaneous treatment with oral cyclophosphamide, 2mg/kg/d, or by monthly pulses of 1g i.v. may help to reduce the doses of corticosteroids. In cases with severe general

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Polyarteritis nodosa is a necrotizing systemic vasculitis of medium caliber arteries whose cause, most often unknown, may be hepatitis B virus. It is a serious condition diagnosed on the basis of well-defined criteria. Clinically, it is manifested by signs of kidney, skin, digestive, cardiac, joint or even vascular damage. The neurological manifestations of polyarteritis nodosa are frequent and make the disease serious. Peripheral neuropathy is the most frequent and earliest symptom. Central nervous system manifestations are less frequent and usually occur at a late stage. The objective of this review article is to describe the clinical manifestations of polyarteritis nodosa with a focus on damage to the central and peripheral nervous system.
La polyartérite noueuse est une vascularite systémique nécrosante des artères de moyen calibre dont la cause, le plus souvent inconnue, peut être le virus de l’hépatite B. Il s’agit d’une affection grave dont le diagnostic est établi sur la base de critères bien définis. Cliniquement, elle se traduit par des signes d’atteinte rénale, cutanée, digestive, cardiaque, articulaire ou encore vasculaire. Les manifestations neurologiques de la polyartérite noueuse sont fréquentes et font toute la gravité de la maladie. La neuropathie périphérique en constitue le symptôme le plus fréquent et le plus précoce. Les manifestations du système nerveux central sont moins fréquentes et surviennent en général à un stade tardif. Cette mise au point a pour objectif de décrire les manifestations cliniques de la polyartérite noueuse en se focalisant sur les atteintes du système nerveux central et périphérique.
Polyarteritis Nodosa Neurologic Manifestations
2019, Neurologic Clinics
Citation Excerpt :
However, that “an isolated vasculitis is a limited expression of a systemic vasculitis does not imply that the vasculitis will or will not subsequently evolve into systemic disease.”10 Indeed, Said32 showed that 37% of the patients with initially isolated peripheral nerve vasculitis subsequently developed systemic vasculitis manifestations and that 24% of them experienced neuropathy relapses. Although some sporadic cases reported had poor outcomes,34 most nonsystemic vasculitides, clearly restricted to peripheral nerves, respond favorably to treatment (mainly corticosteroids and immunosuppressants)35,36; however, improvement may occur very slowly and sometimes only partially.
Rehabilitation of Patients With Neuropathies
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Peripheral neuropathies (PNs) are prevalent and detrimental to a patient's functional level. As the motor system is involved at the early stage of the disease, the influenced limb shows decreased maximal strength, endurance, and power, which clinically mimics the normal aging process or general fatigue. As the sensory system is predominantly affected, patients may have difficulties participating in multitask activities, such as driving, when patients need to control hands and feet through proprioception. The rehabilitation process could be unsuccessful if PNs are undiagnosed and neglected. This chapter provides an understanding of PNs to allow physiatrists to diagnose these conditions, offer a prognosis, and provide rehabilitative therapies.
Autoimmune neuropathies associated to rheumatic diseases
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Just as in EGPA, PNS impairment represents one of the major diagnostic criteria [89]. Once again, MM is the most frequent PNS phenotype accounting for more than half of all affected subjects [50,89]. Disease onset is around 50 years and PNS symptoms are already present by the time of diagnosis in at least 50% of all patients [62].
Systemic manifestations are frequent in autoimmune rheumatic diseases and include peripheral nervous system damage. Neuron cell body, axons and myelin sheath may all be affected in this context. This involvement results in severe and sometimes disabling symptoms. Sensory, motor and autonomic features may be present in different patterns that emerge as peculiar clinical pictures. Prompt recognition of these neuropathies is pivotal to guide treatment and reduce the risks of long term disability. In this review, we aim to describe the main immune-mediated neuropathies associated to rheumatic diseases: sensory neuronopathies, multiple mononeuropathies and chronic inflammatory demyelinating polyradiculoneuropathy, with an emphasis on clinical features and therapeutic options.
Vasculitic neuropathy
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Vasculitis is a primary phenomenon in autoimmune diseases such as polyarteritis nodosa, Wegener’s granulomatosis, Churg–Strauss syndrome, microscopic polyangiitis, and essential mixed cryoglobulinemia. As a secondary feature vasculitis may complicate, for example, connective tissue diseases, infections, malignancies, and diabetes. Vasculitic neuropathy is a consequence of destruction of the vessel wall and occlusion of the vessel lumen of small epineurial arteries. Sometimes patients present with nonsystemic vasculitic neuropathy, i.e., vasculitis limited to peripheral nerves and muscles with no evidence of further systemic involvement. Treatment with corticosteroids, sometimes in combination with other immunosuppressants, is required to control the inflammatory process and prevent further ischemic nerve damage.
Neurophysiological approach to disorders of peripheral nerve
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Citation Excerpt :
EMG and NCS should include bilateral examination of several nerves and muscles to demonstrate chronic or acute lesions or both and whether other nerves are normal. The underlying disorder may be systemic disease (polyarteritis nodosa, Churg–Strauss syndrome, Wegener granulomatosis (Fig. 6.17)) or occur as a nonsystemic vasculitic neuropathy (NSVN), i.e., a disease clinically and electrophysiologically affecting the PNS only although histopathological examination usually shows vasculitis in muscle as well (Said et al., 1988; Said, 1997). The neuropathy may also be secondary to infection (cytomegalovirus, HIV, hepatitis B and C and others), malignant disease, or medical treatment/drug abuse (Said and Lacroix, 2005).
Disorders of the peripheral nerve system (PNS) are heterogeneous and may involve motor fibers, sensory fibers, small myelinated and unmyelinated fibers and autonomic nerve fibers, with variable anatomical distribution (single nerves, several different nerves, symmetrical affection of all nerves, plexus, or root lesions). Furthermore pathological processes may result in either demyelination, axonal degeneration or both. In order to reach an exact diagnosis of any neuropathy electrophysiological studies are crucial to obtain information about these variables.
Conventional electrophysiological methods including nerve conduction studies and electromyography used in the study of patients suspected of having a neuropathy and the significance of the findings are discussed in detail and more novel and experimental methods are mentioned. Diagnostic considerations are based on a flow chart classifying neuropathies into eight categories based on mode of onset, distribution, and electrophysiological findings, and the electrophysiological characteristics in each type of neuropathy are discussed.

View all citing articles on Scopus

: Address Reprint Requests to Professor Girard Said, Service de Neurologie, CH de Bicêtre, 78, rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France

^*: From the Service de Neurologie, Hôpital de Bicêtre, Université Paris Sud, Paris, France

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NECROTIZING PERIPHERAL NERVE VASCULITIS

Section snippets

PERIPHERAL NEUROPATHY OF NECROTIZING ARTERITIS

EVOLUTION OF ISCHEMIC NEUROPATHY

NEUROPATHY IN WEGENER'S GRANULOMATOSIS

NECROTIZING VASCULITIS IN NEUROPATHY OF OTHER ORIGINS

INDICATION OF NERVE BIOPSY FOR DIAGNOSIS OF VASCULITIS

TREATMENT

Am J Med

Mayo Clin Proc

Lancet

Hum Pathol

Mayo Clin Proc

J Neurol Sci

Am J Med

Vasculitic neuropathy

Peripheral neuropathy associated with essential mixed cryoglobulinaemia: A role for hepatitis C virus infection?

J Neurol Neurosurg Psychiatry

Peripheral neuropathy in systemic vasculitis: Clinical and electrophysiologic study of 22 patients

Neurology

Systemic vasculitis in association with human immunodeficiency virus infection

Arthritis Rheumatism

Neurologic disease in biopsy-proven giant cell (temporal) arteritis

Neurology

Contribution of nerve biopsy findings to the diagnosis of disabling neuropathy in the elderly. A retrospective review of 100 consecutive patients

Brain

Allergic granulomatosis and angiitis (Churg-Strauss syndrome)

Proc Mayo Clin

Allergic granulomatosis, allergic angeitis, and periarteritis nodosa

Am J Pathol

Detection of autoantibodies against myeloid lysosomal enzymes: A useful adjunct to classification of patients with biopsy-proven necrotizing arteritis

Am J Med

Neurologic manifestations of systemic vasculitides

Rheum Dis Clin North Am

Cutaneous periarteritis nodosa: A study of 33 cases

Nonsystemic vasculitic neuropathy

Brain

The spectrum of vasculitis

Ann Intern Med

Long-term follow up study of periarteritis nodosa

Am J Med

Vulnerability of nerve fibers to ischaemia

Brain

Périartérite noueuse systémique