Vasculitic Neuropathy

05 November 2004

Term confined to the PNS

Combination of PNS & CNS involvement only reported in context of a recognized multisystem connective tissue disease such as Wegener’s, SLE

Aetiology of vasculitic neuropathy

32% PAN. Churg Strauss

24% undifferentiated connective (multisystem) tissue disease

20% rheumatoid arthritis

isolated peripheral nervous system vasculitis

SLE

Wegener’s granulomatosis

CLL with cryoglobulinemia

Primary Sjogren’s syndrome

Infectious: leprosy, HIV

Paraneoplastic

(Diabetes)

Investigations:

Serology:          ANA, ENA, ACE, ANCA, hep B&C, cryoglobulins, EPG, ACE, ESR,

                        C3, C4, CH50, LAC, ACLA

Evidence of system disease elsewhere: CXR, urine

EMG/NCS: 100% patients had abnormalities

Nerve biopsy:   30-60% sensitivity for vasculitis

                        Affects epineural arterioles 50-300 micrometres in diameter

                        Gold std is vessel wall infarction (strictest definition)

                        Probable if prominent inflammatory cell infiltrate with devastating acute

axonal death or teased fibre with >40% axonal degeneration

85% cases IF evidence of immune complex deposition

Muscle biopsy: increases sensitivity for vasculitis a further 20-25%

(LP for patient with flow cytometry re ?lymphoma)

Prognosis

13% 5 yr survival for untreated neuropathy associated with systemic vasculitis

57% 5 yr survival for treated neuropathy associated with systemic vasculitis

good prognosis with isolated PNS vasculitis (96% 3 yr survival)

Treatment

75% response with steroids +/- immunosuppresives (cyclophosphamide)