Table 4.

Outcomes for patients with ANCA vasculitis on the basis of different classification systems evaluated in the same cohort characterized in Tables 2 and 3

Classification SystemTreatment Resistance (n=109 of 483)Relapse (n=147 of 374)ESRD (n=161 of 502)Death (n=139 of 502)
Classification on the basis of CHCC 2012 definitions
 GPA (n=117)20 of 117 (17%)58 of 97 (60%)24 of 117 (21%)20 of 117 (17%)
 MPA (n=264)56 of 255 (22%)74 of 199 (37%)80 of 264 (30%)79 of 264 (30%)
 RLV (n=121)33 of 111 (30%)15 of 78 (19%)57 of 121 (47%)40 of 121 (33%)
P value0.07<0.001<0.001<0.01
European Medicines Agency classification
 GPA (n=324)68 of 317 (22%)110 of 249 (44%)92 of 324 (28%)84 of 324 (26%)
 MPA (n=178)41 of 166 (25%)37 of 125 (30%)69 of 178 (39%)55 of 178 (31%)
P value0.42<0.01<0.020.25
Classification on the basis of ANCA specificity
 PR3-ANCA (n=219)37 of 213 (17%)90 of 176 (51%)56 of 219 (26%)50 of 219 (23%)
 MPO-ANCA (n=283)72 of 270 (27%)57 of 198 (29%)105 of 283 (37%)89 of 283 (31%)
P value<0.02<0.001<0.010.03
  • (Modified from Lionaki, et al. [5]). Treatment resistance = persistence or new appearance of extrarenal manifestations and/or progressive decline in renal function with active urine sediment in spite of immunosuppressive therapy. Relapse = reactivation of vasculitis in any organ after initial response to treatment. ESRD = chronic need for dialysis or transplantation. Death = death from any cause. CHCC 2012, 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; RLV, renal-limited vasculitis; PR3-ANCA, ANCA specific for proteinase 3; MPO-ANCA, ANCA specific for myeloperoxidase.