Elsevier

The Lancet Neurology

Volume 13, Issue 1, January 2014, Pages 67-82
The Lancet Neurology

Review
Vasculitic neuropathies

https://doi.org/10.1016/S1474-4422(13)70236-9Get rights and content

Summary

The vasculitic neuropathies are a diverse group of disorders characterised by the acute-to-subacute onset of painful sensory and motor deficits that result from inflammatory destruction of nerve blood vessels and subsequent ischaemic injury. They are common in patients with primary systemic vasculitis and are seen in vasculitis secondary to disorders such as rheumatoid arthritis, viral infections, and diabetic inflammatory neuropathies. It is imperative that neurologists recognise these disorders to initiate treatment promptly and thereby prevent morbidity and mortality. To simplify the approach to patients with vasculitis of the peripheral nerves, a straightforward, dichotomous classification scheme can be used in which the vasculitic neuropathies are divided into two groups—nerve large arteriole vasculitis and nerve microvasculitis—on the basis of the size of the involved vessels. The size of the affected blood vessels correlates with the clinical course and prognosis in patients with vasculitic neuropathy.

Introduction

The vasculitides are systemic or localised disorders in which blood vessel walls are infiltrated and damaged by inflammatory cells, with secondary ischaemic injury. Inflammation can affect blood vessels of any size anywhere in the body. Many forms of vasculitis affect the peripheral nervous system (PNS).1, 2 Peripheral nerve involvement of vasculitis has been reported in up to 60–70% of patients with systemic vasculitis.3, 4, 5 Vasculitis can occur as a primary process or a secondary phenomenon related to various disorders, ranging from rheumatological conditions to viral infections.2 More recently, diabetic lumbosacral radiculoplexus neuropathy (DLRPN) and non-diabetic lumbosacral radiculoplexus neuropathy (LRPN) have been classified as vasculitic neuropathies.6 In this Review, we propose a straightforward, dichotomous classification scheme in which we divide the vasculitic neuropathies into two groups on the basis of the size of the involved vessels: nerve large arteriole vasculitis and nerve microvasculitis. We summarise the diagnosis, clinical features, and treatment of the common primary and secondary systemic vasculitic neuropathies (SVNs), non-systemic vasculitic neuropathy (NSVN), DLRPN, and LRPN. And we discuss painless diabetic motor neuropathy—a condition seen by neurologists that shares many clinical and pathological features with LRPN—which might be another vasculitic neuropathy.

Section snippets

Classification

The classification of vasculitic neuropathy has become increasingly complex over the past half century, in large part because of an improved understanding of vasculitis and the incremental addition of other disorders under the rubric of vasculitis.7, 8, 9, 10, 11 The vasculitic neuropathies can be classified in terms of clinical characteristics (eg, organ involvement, disease association), histopathological features (eg, size of involved vessels), and underlying mechanisms. There are several

Presentation

Nerve large arteriole vasculitis classically presents with the acute-to-subacute onset of painful sensory or sensorimotor deficits.1, 7, 17 Patients often first note the sudden development of proximal deep aching pain in the limb followed by burning cutaneous pain and focal weakness in the territory of a single nerve.18 Within days to weeks, other nerves become involved, resulting in the classic mononeuritis multiplex or what we prefer to call multiple mononeuropathy. Although multiple

Diagnosis

The diagnostic approach is the same for both nerve large arteriole vasculitis and nerve microvasculitis and is not tailored to the specific, suspected type of vasculitic neuropathy except as noted below.

Electrodiagnostic studies in nerve large arteriole vasculitis—as well as nerve microvasculitis—characteristically show acute-to-subacute axonal loss of sensory and motor fibres, frequently in a patchy, multifocal distribution.7 Electrodiagnostic findings that are most supportive of a diagnosis

Treatment

The treatment approach for patients with nerve large arteriole vasculitis is essentially the same for all patients with the notable exceptions of virus-associated vasculitic neuropathies. Patients with nerve microvasculitis are managed slightly differently, as discussed below in the NSVN, DLRPN, and LRPN sections. One responsibility of the neurologist caring for a patient with vasculitic neuropathy is to assess the clinical response to therapy by using predetermined neurological endpoints,

Conclusions

Vasculitis affecting the peripheral nerves is commonly seen in patients with primary systemic vasculitis and can also result from vasculitis secondary to connective tissue diseases, viral infections, or malignancies. Not uncommonly (30% of reported cases), vasculitis is confined to the nerves and possibly also the adjacent muscles, an entity termed NSVN. DLRPN, LRPN, DCRPN, and painless diabetic motor neuropathy are emerging as additional non-systemic vasculitic neuropathies with predominant

Search strategy and selection criteria

References for this Review were identified by searches of PubMed between 1950 and Aug 16, 2013, and references from relevant articles. The search terms were “vasculitic neuropathy”, “nerve large arteriole”, “microvasculitis”, “microscopic polyangiitis”, “polyarteritis nodosa”, “Churg-Strauss syndrome”, “Wegener's granulomatosis”, “rheumatoid vasculitis”, “Sjögren's syndrome”, “hepatitis C vasculitic neuropathy”, “cryoglobulinemia”, “HIV-associated vasculitic neuropathy”, “paraneoplastic

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