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 and Sacri et al.), our unpublished Italian cohort and a literature review (Zwerina et al.)

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)