Neuropathies with Systemic Vasculitis

Collins, Michael; Kissel, John T.

doi:10.1016/b978-0-7216-9491-7.50108-3

Cited by 26 publications

(34 citation statements)

References 491 publications

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“…Systemic vasculitis is an important cause of neuropathy, and the typical disorders are microscopic polyangitis (MPA), Churg-Strauss syndrome (CSS), and Wegener's granulomatosis (WG) [1][2][3][4][5]. Peripheral neuropathy often appears as an early symptom of MPA and CSS.…”

Section: Introductionmentioning

confidence: 99%

Two subtypes of Churg–Strauss syndrome with neuropathy: the roles of eosinophils and ANCA

Oka¹,

Kawasaki

Matsui

et al. 2010

Mod Rheumatol

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The aim of this study was to clarify the differences in the pathogenesis of neuropathy between myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive and -negative patients with Churg-Strauss syndrome (CSS). Eight MPO-ANCA-positive and 14 MPO-ANCA-negative patients were included. In addition to the standard histology, nerve biopsies were examined, employing immunohistochemistry for eosinophil major basic protein and electron microscopy. The groups did not differ significantly in clinical profiles, including the peak disability score and number of blood eosinophils. In nerve biopsies, necrotizing vasculitis was found in 63% (5/8) of the ANCA-positive and 21% (3/14) of the ANCA-negative patients. Fibrinoid necrosis of vessel walls was noted in 4 ANCA-positive patients (50%), and in one ANCA-negative patient (p = 0039). In contrast, a large number of eosinophilic infiltrations in the epineurium was shown in 36% (5/14) of the ANCA-negative patients, with no eosinophilic infiltrations shown in ANCA-positive patients. In 3 ANCA-negative patients, endoneurial eosinophils were seen where focal axonal loss and capillary dilatation were occasionally noted. There may be 2 pathogenetic mechanisms of neuropathy with CSS: ANCA-related vascular fibrinoid necrosis, and a toxic eosinophilic effect on nerve fibers which is independent of ANCA. Therapy targeting activated eosinophils may be a possible treatment for intractable neuropathy of CSS.

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Section: Introductionmentioning

confidence: 99%

Two subtypes of Churg–Strauss syndrome with neuropathy: the roles of eosinophils and ANCA

Oka¹,

Kawasaki

Matsui

et al. 2010

Mod Rheumatol

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show abstract

“…We observed an acute, distal, relatively symmetrical presentation of vasculitic neuropathy resembling GBS, presumably due to bilateral sciatic nerve involvement at the popliteal fossa. Such clinical presentation is extremely rare in the context of peripheral nerve vasculitis, while mononeuritis multiplex is considered the classical presentation [3]. Early electrodiagnostic studies can sometimes show "pseudo-conduction blocks", that is, nerve conduction features consistent with conduction block electrodiagnostic criteria [7], but not confirmed by subsequent serial nerve conduction studies.…”

Section: Nilo Riva Federica Cerri Calogera Butera Stefano Amadio Angementioning

confidence: 98%

Churg Strauss syndrome presenting as acute neuropathy resembling Guillain Barré syndrome

et al. 2008

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“…Vasculitis is histologically defined as inflammatory cell infiltration and destruction of blood vessels and is classified as primary, when idiopathic, or secondary when it is a manifestation of other conditions like connective tissue diseases, infections, adverse drug effects or paraneoplastic syndromes [1][2][3]. It has a wide spectrum of severity ranging from skin-limited diseases to systemic involvement [1][2][3].…”

Section: Introductionmentioning

confidence: 99%

“…Nerve involvement in vasculitides is well characterized [1][2][3]. Infection-related vasculitides, systemic necrotizing vasculitides (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, connective tissue diseases) and hypersensitivity vasculitides (cryoglobulinemia, malignancy) may be complicated by nerve damage [1][2][3].…”

Section: Introductionmentioning

confidence: 99%