title: Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: a comparative study.
abstract: OBJECTIVE
  To compare clinicopathologic findings in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis-associated neuropathy (MPAN).
  
  
  METHODS
  Patients clinicopathologically confirmed to have NSVN (n=23) or MPAN (n=40) were compared with respect to clinical, electrophysiologic, and histopathologic features.
  
  
  RESULTS
  Clinical features of neuropathy such as initial symptoms, progression, and distribution of sensory and motor deficits were similar in both groups, while functional compromise was greater in MPAN than NSVN. Abnormalities of laboratory data including those reflecting severity and extent of inflammation such as C-reactive protein were more conspicuous in MPAN than NSVN. Perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were positive in two-thirds of patients with MPAN but negative in all NSVN. Electrophysiologic and histopathologic findings indicated axonal neuropathy in both groups, whereas the reduction of compound muscle action potentials in the tibial nerve and sensory nerve action potentials in the median nerve was significantly more profound in MPAN than NSVN. As for the epineurial perivascular infiltration, frequencies of cell-specific markers for T lymphocytes, macrophages, and B lymphocytes among cells infiltrating the vasculitic lesions were essentially similar between groups.
  
  
  CONCLUSIONS
  Clinicopathologic profiles and vascular pathology were similar between NSVN and MPAN but the age at onset, severity, and presence of p-ANCA were clearly different. Further study is needed to clarify the pathogenesis of NSVN and its place in the vasculitic spectrum of diseases.
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Sugiura
authors.forename: H
authors.initials: H
authors.lastname: Koike
authors.forename: M
authors.initials: M
authors.lastname: Iijima
authors.forename: K
authors.initials: K
authors.lastname: Mori
authors.forename: N
authors.initials: N
authors.lastname: Hattori
authors.forename: M
authors.initials: M
authors.lastname: Katsuno
authors.forename: F
authors.initials: F
authors.lastname: Tanaka
authors.forename: G
authors.initials: G
authors.lastname: Sobue
mesh_terms: D000200:Action Potentials / Q000502:physiology; D017668:Age of Onset; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism; D003937:Diagnosis, Differential; D005260:Female; D005921:Glomerulonephritis / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology; D010523:Peripheral Nervous System Diseases / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology*; D011115:Polyneuropathies / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology*; D018709:Statistics, Nonparametric; D013497:Sural Nerve / Q000503:physiopathology; D014657:Vasculitis / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic