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Clinicoepidemiological manifestations of RPGN and ANCA-associated vasculitides: an 11-year retrospective hospital-based study in Japan
Modern Rheumatology, 10/14/09

Suzuki Y FI et al. – Most patients treated with immunosuppressants showed severe prognosis because of frequent recurrences of vasculitis and infectious episodes after repeated and prolonged treatments with immunosuppressants. Present analysis further confirms the epidemiological and serological differences in ANCA-related RPGN between Japan and Europe, and reinforced the fact that ANCA-associated vasculitis is the most serious causal disease for RPGN.

• Retrospectively analyzed 27 RPGN patients who were admitted in hospital over past 11 years and who could be basically followed for more than 1 year
• Incidence of ANCA-related vasculitis, the presence of (MPO)/proteinase 3 (PR3)-ANCA and clinical outcomes followed
• As there were no PR3-ANCA single positive and/or WG patients, all patients serologically divided into 4 groups
• Group I: MPO-ANCA single-positive patients (N = 11)
• Group II: MPO-ANCA and PR3-ANCA double-positive patients (N = 3)
• Group III: antiglomerular basement membrane antibody (anti-GBM Ab)-positive patients (N = 6)
Group IV: all negative patients (N = 7)

• Groups II/III showed more severe manifestation at admission
• Group I, only 36.3% patients avoided death and/or dialysis-dependent end-stage renal disease
• Most in Group IV were women (85.7%), and 50% were diagnosed with rheumatic diseases
• Every patient in Groups I-III treated with oral corticosteroid and/or methylprednisolone pulse therapy

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