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Table 3 Main demographic and clinical characteristics of childhood-onset GPA, MPA and EGPA in two large published series (Cabral et al. and Sacri et al.), our unpublished Italian cohort and a literature review (Zwerina et al.)

From: ANCA-associated vasculitis in childhood: recent advances

  GPA MPA EGPA
  Cabral et al. [3] Sacri et al.
[13]
Italian cohort (unpublished) Cabral et al. [3] Sacri et al.
[13]
Italian cohort (unpublished) Zwerina et al. [15]
Total no. of patients 183 38 31 48 28 21 33
Female, no. (%) 113 (62) 21 (75) 22 (71) 35 (73) 34 (89) 11(52) 19 (58)
Caucasian, no. (%) 107 (59) 21 (75) 31 (100) 20 (42) 26 (68) 21 (100) 24 (73)
Median age (range) at diagnosis- years 14 (2–18) 12.8 (10.1-14.6) 14 (6–18) 12 (1–18) 11.2 (8.9-12.3) 11 (6–17) 13 (2–18)
Median time (range) to diagnosis- months 2.1 (0–73) 1.8 (0.8-5) 5 (0–60) 1.6 (0–39) 1.0 (0.6-8.1) 2.5 (1–24) NA
Constitutional symptoms, no. (%) 160 (88) 23 (82) 21 (68) 41 (85) 29(78) 14 (67) NA
Fever 97 (53) 19 (68) 16 (52) 25 (52) 18 (50) 11 (52) NA
Fatigue 160 (88) 23 (82) 21 (68) 37 (77) 29 (78) 14 (67) NA
Renal involvement, no. (%) 151 (83) 22 (78) 20 (65) 36 (75) 36 (95) 20 (95) 5/32 (16)
Pulmonary involvement, no. (%) 136 (74) 19 (68) 16 (52) 21 (44) 11 (29) 8 (38) 29 (88)
ENT, no. (%) 128 (70) 21 (75) 24 (78) 0 (0) 0 (0) 0 (0) 20 (77)
Eye involvement, no. (%) 78 (43) 6 (21) 14 (45) 15 (31) 3 (8) 0 (0) NA
Cutaneous involvement, no. (%) 86 (47) 15 (54) 8 (26) 25 (52) 13 (34) 7 (33) 21 (66)
Gastrointestinal involvement, no. (%) 66 (36) 5 (18) 2 (6) 28 (58) 4 (11) 4 (19) 12 (40)
Musculoskeletal involvement, no. (%) 24 (14) 1 (4) 14 (45) 9 (19) 1 (3) 6 (29) 7 (22)
CNS involvement, no. (%) 36 (20) 1 (4) 2 (6) 10 (21) 2(5) 6 (29) NA
Cardiovascular involvement, no. (%) 10 (5) NA 2 (6) 3 (6) NA 1 (5) 17 (55)