Table 1.

Characteristics of the study population according to the year of diagnosis

CharacteristicTotal (n=123)1986–2000 (n=41)2001–2006 (n=38)2007–2015 (n=44)
Baseline (n=123)
 Age, yr51±1954±1946±2153±17
 Males66 (54)22 (54)24 (63)20 (46)
 Anti-GBM antibodies positivea92 (82)31 (87)28 (78)33 (81)
  Anti-GBM antibodies, ANCA negative53 (60)12 (44)20 (71)21 (64)
  Anti-GBM antibodies, ANCA positive35 (40)15 (56)8 (27)12 (36)
 ANCA typeb
  Anti-MPO23 (85)9 (90)7 (100)7 (70)
  Anti-PR34 (15)1 (10)0 (0)3 (30)
 Lung involvementc41 (35)14 (37)12 (33)15 (35)
 Serum creatinine, mg/dld7.0 (3.5–10.3)9.0 (5.8–13.9)7.3 (4.5–11.4)4.6 (1.9–7.0)
 Dialysis dependentd69 (56)28 (68)27 (71)14 (32)
Follow-up (n=123)
 Duration of follow-up, yrd4.6±4.05.3±5.15.9±3.52.8±2.2
 ESRD at end of follow-upe82 (67)31 (76)29 (76)22 (50)
 Transplantation32 (26)9 (22)15 (40)8 (18)
 Deathe34 (28)17 (42)11 (29)6 (14)
Treatment (n=110)
 Plasma exchange91 (83)24 (71)31 (89)38 (88)
  Median number of exchanges7 (6–12)6 (5–10)10 (7–10)7 (6–14)
 Cyclophosphamidee88 (80)21 (62)29 (88)38 (88)
  Oralf43 (60)13 (68)14 (67)16 (50)
  Intravenousf25 (35)6 (32)6 (28)13 (41)
  Both4 (5)0 (0)1 (5)3 (9)
 Corticosteroids106 (96)33 (94)32 (97)42 (98)
 Azathioprine21 (19)5 (15)6 (18)10 (23)
 Mycophenolate mofetil12 (11)4 (12)4 (12)4 (9)
 Rituximabd10 (9)0 (0)0 (0)10 (23)
  • Data are presented as numbers (%), means±SD, or medians (interquartile range). GBM, glomerular basement membrane; MPO, myeloperoxidase; PR3, proteinase 3.

  • a Results from anti-GBM antibody ELISA were available in 112 patients. Results from ANCA ELISA were available in 114 patients. Both tests were performed in 108 patients; four patients with positivity for anti-GBM antibodies had no available results from ANCA ELISA.

  • b Thirty-five patients were anti-GBM antibodies and ANCA positive. Of these, ANCA type (anti-MPO or anti-PR3) was known in 27 patients.

  • c Total, n=117 patients.

  • d Different between subgroups with P value <0.001.

  • e Different between subgroups with P value <0.05.

  • f Route of administration of cyclophosphamide was known for 72 patients. The mean oral cyclophosphamide dose at the start of therapy was 118±49 g (n=30), and the mean intravenous dose was 1113±460 g (n=8).