Table 1.

Validation studies of the ANCA-associated GN classification with renal survival

Clinical and Histopathologic ParametersThis StudyChang et al. (2)Berden et al. (1)Hilhorst et al. (3)Iwakiri et al. (4)Togashi et al. (5)Muso et al. (6)Quintana et al. (7)Tanna et al. (8)Ford et al. (9)Ellis et al. (10)Unlu et al. (11)Moroni et al. (12)
No. of patients21512110016410254871361041207614193
RegionNanjing, ChinaBeijing, ChinaEuropeThe NetherlandsJapanJapanJapanSpain and United KingdomUnited KingdomAustraliaUnited StatesTurkeyItaly
Clinical classification, n (%)
 MPA215 (100)68 (56.2)61 (61)ND97 (95.1)25 (46)87 (100)80 (58.8)NDND31 (41)20 (14)34 (36.5)
 GPA049 (40.5)39 (39)ND3 (2.9)28 (52)044 (32.4)NDND43 (57)55 (39)39 (41.2)
 EGPA000ND2 (2.0)1 (2)00NDND000
 RLV04 (3.3)0ND0008 (5.9)NDND2 (3)39 (28)10 (10.7)
ANCA type, n (%)
 MPO-ANCA215 (100)108 (89.3)47 (47)81 (49.4)86 (84.3)54 (100)76 (87)80 (58.8)49 (47)75 (62.5)a32 (42)a56 (40)a43 (46.2)
 PR3-ANCA013 (10.7)45 (45)83 (50.6)5 (4.9)0053 (39)49 (47)28 (23.3)a30 (39)a55 (39)a36 (38.7)
 Double positive000000000005 (4)a
 ANCA negative002 (2)011 (10.8)009 (6.6)6 (6)014 (18)a25 (18)a14 (15.0)
 ANCA missing003 (3)00011 (13)000000
AAGN classification, n (%)
 Focal27 (12.6)33 (27.3)16 (16)81 (49.4)46 (45.1)17 (31)40 (46.0)35 (26)23 (22.1)34 (28.3)20 (26)31 (22)12 (21)
 Mixed82 (38.1)24 (19.8)16 (16)39 (23.8)18 (17.6)19 (35)26 (29.9)53 (39)48 (46.2)33 (27.5)27 (36)29 (21)36 (39)
 Crescentic47 (21.9)53 (43.8)55 (55)43 (26.2)32 (31.4)8 (15)7 (8.0)31 (23)26 (25.0)33 (27.5)18 (24)69 (49)28 (30)
 Sclerotic59 (27.4)11 (9.1)13 (13)1 (0.6)6 (5.9)10 (19)14 (16.1)17 (13)7 (6.7)20 (16.7)11 (14)12 (9)9 (10)
Renal outcome, %5 yr 2 yr 5 yr 5 yr 41 mo, ESRD, n (%)5 yr5 yr 5 yr5 yr3.4 yr, ESRD or death, n (%)2 yr, ESRD, n (%)651±728 d, ESRD, n (%)5 yr
 Focal1009393912 (4.3)1001009610011 (32)2(10)4 (13)82
 Mixed58.97261698 (44)10096817713 (39)6(22)10 (34)81
 Crescentic67.46076649 (28)7586867414 (42)4(22)20 (29)37
 Sclerotic20.72950ND4 (67)7029612516 (80)4(36)8 (67)51
  • MPA, microscopic polyangiitis; ND, no data; GPA, granulomatosis with polyangiitis; EGPA, eosinophilic granulomatosis with polyangiitis; RLV, renal-limited vasculitis; MPO-ANCA, myeloperoxidase-ANCA; PR3-ANCA, proteinase 3-ANCA; AAGN, ANCA-associated GN.

  • a Cytoplasmic ANCA and perinuclear ANCA were reported instead of PR3-ANCA and MPO-ANCA, respectively.