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Vasculitis and its Renal Manifestation with Special Reference
to Anti-Neutrophil Cytoplasmic Antibody
Seiji MINOTA, MD*
The nomenclature and classification of vasculitis have been problematic,
with each researcher proposing their own system. Since the discovery of
anti-neutrophil cytoplasmic antibody and the consensus conference on the
nomenclature of vasculitis in Chapel Hill, order has been brought to the
chaos to some extent. Measurement of P-ANCA and C-ANCA in patients' sera
has exerted an immeasurable influence on the clinical aspects of vasculitis,
since they are shown to be valuable for the diagnosis and assessment of
the vasculitic activity in most cases.
Pathogenetic significance is also given to ANCA. ANCA has been reported
to release myeloperoxidase(MPO) and proteinase-3 antigens from neutrophils
activated by inflammatory cytokines. When both MPO antigen in serum as
well as ANCA were measured serially in the same patients, however, clinical
condition was better reflected by the amount of serum MPO than the titer
of ANCA in some patients. Thus, the amount of serum MPO increased without
concomitant rise in ANCA titer in exacerbation of vasculitis in one patient.
In two more, high serum titers of ANCA and low MPO antigen in the active
phase of vasculitis were reversed when pulmonary hemorrhage occurred, and
fatal outcome ensued.
It could be speculated from these observations that some ANCA might be directed against the active sites of MPO and even suppress the vasculitic activity through blocking MPO. Since definitive evidence has not been obtained so far on the pathogenetic role of ANCA, the clinical significance of ANCA should be addressed at the same level as anti-dsDNA antibody in systemic lupus erythematosus: aid for diagnosis and disease activity.
[Rinsho Byori 51 : 231`238, 2003]
*Division of Clinical Immunology, Jichi Medical School, Kawachi-gun, Tochigi-pref. 329-0498
yKey Wordszanti-neutrophil cytoplasmic antibodyFANCA(RD
ΧEΏRΜ)CMPO(~GyILV_[[)Cproteinase 3FPR-3CChapel Hill Jt@X
*©‘γΘεwAM[EE}`Θ(§329-0498 ΘΨ§ΝΰSμΝΰ¬ςt3311-1)
E-mail :sminota@jichi.ac.jp