Table 4.

Summary of studies on renal and patient survival in patients with AAV based on ANCA serotype (C-/PR3-ANCA and P-/MPO-ANCA).

Study (reference)YearNo. PatientsRenal InvolvementSpecialtyComparisonCase IdentificationESRDDeath
Geffriaud-Ricouard, et al1619938392%NephrologyPR3 vs MPOSingle centerNo differenceNo difference
Franssen, et al1819959275%“Mixed”PR3 vs MPOTeaching hospitalsNo differenceNo difference
Hogan, et al131996107100%NephrologyC vs P-ANCASpecialized units
(Glomerular Disease
Collaborative Network)
No differenceWorse for C-ANCA
Westman, et al191998123100%NephrologyPR3 vs MPOSingle centerWorse for PR3#No difference
Franssen, et al1019989275%“Mixed”PR3 vs MPOTeaching hospitalsNRNo difference
Vizjak, et al242003135100%“Mixed”PR3 vs MPOSingle centerWorse MPONo difference
Weidner, et al12200480100%NephrologyPR3 vs MPOSingle centerNo differenceWorse for PR3
Rihova, et al25200561100%NephrologyC-vs P-ANCASingle centerNo differenceNo difference
Flossmann, et al262011535100%MixedPR3 vs MPOMulticenter clinical trialsNRWorse for MPO
Lionaki, et al5201250297%NephrologyPR3 vs MPOSpecialized units
(Glomerular Disease
Collaborative Network)
Worse for MPOWorse for MPO
de Joode, et al202013212100%NephrologyMPO vs PR3Single centerWorse for MPOWorse for MPO*
Present study201318376%“Mixed”PR3 vs MPOPopulation-basedWorse for MPONo difference
  • # Analysis with capture ANCA.

  • * Significant difference during the first 6 months after diagnosis. NR: not reported; ESRD: endstage renal disease; ANCA: antineutrophil cytoplasmic antibody; PR3: proteinase 3; MPO: myeloperoxidase.