Peripheral neuropathy in ANCA-associated vasculitis: outcomes from the European Vasculitis Study Group trials

Rheumatology (Oxford). 2011 Dec;50(12):2214-22. doi: 10.1093/rheumatology/ker266. Epub 2011 Sep 2.


Objectives: To describe the incidence and prevalence of peripheral neuropathy in ANCA-associated vasculitis (AAV); to evaluate the correlation of neuropathy with other clinical manifestations; and to review the long-term outcome of treated neuropathy.

Methods: Presence of neuropathy was determined using items from the BVAS and vasculitis damage index (VDI) during 5 years from enrollment into clinical trials conducted by the European Vasculitis Study Group (EUVAS).

Results: Forty (8%) of 506 patients had vasculitic neuropathy at baseline. Incidence of vasculitic motor-involving neuropathy was identical between microscopic polyangiitis (MPA) [16 (7%) out of 237] and granulomatosis with polyangiitis (Wegener's) [19 (7%) out of 269], P = 0.94. Pure sensory neuropathy was reported in 5 (2%) out of 269 patients with granulomatosis with polyangiitis, but not in patients with MPA, P = 0.065. Vasculitic neuropathy at baseline was associated with systemic [odds ratio (OR) = 1.81], cutaneous (OR = 1.29), mucous membranes (OR = 1.21) and ENT (OR = 1.14) manifestations of vasculitis (P < 0.05 for all). There was no association between neuropathy and renal, chest, cardiovascular or abdominal vasculitis or with overall mortality. Of the 40 patients with vasculitic neuropathy at baseline, 35% had complete resolution within 6 months. The cumulative prevalence of chronic neuropathy at any time up to 5 years was 15% (75 of 506). Chronic neuropathy was associated with older age [hazard ratio (HR) = 1.03], higher BVAS (HR = 1.07) and lower baseline creatinine (HR = 0.82) (P < 0.01 for all).

Conclusion: Peripheral neuropathy is an occasional accompaniment of AAV that typically remits in concert with non-neuropathic manifestations, usually involves motor nerves, often produces long-lasting symptoms and is not associated with life-threatening organ involvement.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / complications*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / epidemiology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / therapy
  • Chronic Disease
  • Clinical Trials as Topic
  • Europe / epidemiology
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Peripheral Nervous System Diseases / complications*
  • Peripheral Nervous System Diseases / epidemiology
  • Peripheral Nervous System Diseases / therapy
  • Prevalence
  • Treatment Outcome