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Classification of clinical subtypes, patient survival, kidney prognosis, and relapse in patients with MPO-ANCA-associated vasculitis: A single-center experience
Modern Rheumatology, 06/19/09

Nakabayashi K et al. – Data suggest that pulmo-renal type as well as pulmonary type have a high chance to progress to renal failure or systemic type, and they were fairly commonly associated with vasculitic or infectious death. Therefore, classification of clinical subtypes of MPO-ANCA associated vasculitis at the initial time and on admission is meaningful to some extent for predicting patient survival, kidney prognosis, and relapse, in addition to indicating the appropriate treatment regimen.

• Aim was to investigate the relationship between the initial organ involvement and the eventual clinical course of MPO-ANCA-associated vasculitis
• 30 pts categorized into 10 clinical subtypes based on organ involvement
• Relationship of these subtypes to development of clinical features, patient survival, kidney prognosis, and relapse were evaluated over 4.3 yrs

• Most common clinical features were lung and kidney involvement
• 21 pts manifested with clinical features at time of admission did not present new symptoms as long as they were receiving the treatment
• For pulmonary involvement type, those not being treated for vasculitis, 7/12 pts progressed to pulmo-renal involvement and 5 of them went onto renal failure
• Progression to renal failure also occurred frequently in pts with pulmo-renal type manifesting
• 13 pts died, including 3 due to vasculitis of systemic type, 7 due to infections, and 3 due to malignancy
• Death due to vasculitis occurred in the early phase of treatment and was associated with either pulmonary hemorrhage or gastrointestinal bleeding
• Infectious death occurred throughout the entire course of treatment, mostly in pts with pulmo-renal or pulmonary type; associated with opportunistic organisms
• Death with malignancy was observed after several years of treatment
• Regarding renal prognosis:
  • 10 pts underwent hemodialysis
  • At initiation of hemodialysis, 9 pts had pulmo-renal type
  • 1 had renal type
  • A relapse was observed in 10 pts, mainly in patients with pulmo-renal or pulmonary type, and it occurred after about 2.7 years, even with treatment
  • Such relapses manifested in a similar manner to their initial clinical subtypes

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