Table 3 Published literature referring to short-term clinical course in children with IgAVN

Corticosteroids

 Kawasaki et al. [13]

Retrospective

n = 56

n = 56 biopsied

n = 56 CS pulse therapy + urokinase pulse therapy, dipyridamol and warfarin

8.6 ± 2.9*

*not specified whether median/mean

n = 31 proteinuria

n = 25 nephrotic syndrome

n = 10 renal insufficiency

6 months

decrease of proteinuria,

n = 0 renal insufficiency

 Deng et al. [21]

Retrospective

n = 186

number of renal biopsies unclear

n = 21 no immunosuppressant

n = 105 hydrocortisone

sodium succinate

n = 36 CS pulse therapy

n = 24 CS pulse therapy, tripterygium glycoside

n = 186 ACE inhibitors

9.56 – 10.25 depending on group*

*not specified whether median/mean

n = 183 proteinuria

4 weeks

n = 113 decrease of proteinuria

 Niaudet et al. [14]

Prospective

n = 38

n = 38 biopsied

n = 31 CS pulse therapy

n = 7 CS pulse therapy + CYC

7.7

(3.0–14.2)*

*mean age

n = 36 nephrotic syndrome

n = 3 renal insufficiency

5 months

decrease of proteinuria,

n = 12 resolution of proteinuria,

n = 0 renal insufficiency

Cyclophosphamide

 Wakaki et al. [22]

Retrospective

n = 42

n = 33 biopsied

n = 17 no immunosuppressants

n = 25 CS alone or CS + CYC + AZA and/or CS pulse therapy

7.4

(2.8–14.2)

n = 42 nephrotic-range proteinuria

n = 16 renal insufficiency

 < 3 months

n = 19 resolution of nephrotic-range proteinuria,

n = 17 complete clinical remission

 Iijima et al. [23]

Retrospective

n = 14

n = 14 biopsied

n = 14 CYC + CS, heparin, warfarin, dipyridamol

6.7

(5.0–17.5)

n = 7 heavy proteinuria

n = 1 nephrotic syndrome

n = 2 nephritic syndrome

n = 3 nephrotic-nephritic syndrome

4–5 months

n = 1 heavy proteinuria,

n = 0 renal insufficiency

 Flynn et al. [24]

Retrospective

n = 12

n = 12 biopsied

n = 4 oral CS + CYC

n = 8 CS pulse therapy + CYC

8.6

(5.9–15.3)

n = 12 nephrotic-range proteinuria

3 months

n = 12 decrease of protein-to-creatinine ratio

 Kawasaki et al. [25]

Retrospective

n = 37

n = 37 biopsied

n = 20 CS pulse therapy + urokinase pulse therapy

n = 17 CYC additionally

7.9 – 8

depending on group

n = 26 nephrotic-range proteinuria

n = 16 renal insufficiency

6 months

Decrease of proteinuria,

n = 0 renal insufficiency

n = 21 fully recovered

Cylosporine A

 Park et al. [26]

Retrospective

n = 29

n = 6 biopsied

n = 29 CS + CSA

n = 29 ACE inhibitors

8.6

(2.0–15.5)

n = 6 nephrotic syndrome

n = 23 nephrotic-range proteinuria

n = 1 renal insufficiency

1.8 months

Resolution of proteinuria at a mean of 1.8 months (1 week to 3.5 months)

 Jauhola et al. [27]

Randomized controlled

n = 24

n = 24 biopsied

n = 11 CSA

n = 13 CS pulse therapy

n = 24 ACE inhibitors

9.4

(4–16)

n = 24 nephrotic-range proteinuria or crescents

3 months

n = 18 remission of nephrotic-range

proteinuria

 Ronkainen et al. [28]

Prospective

n = 7

n = 7 biopsied

n = 7 CSA

n = 7 ACE inhibitors

10.6

(7.2–15.2)

n = 7 nephrotic-range proteinuria

1.4 months

Decrease of proteinuria at a mean of 1.4 months (1 week to 4 months)

MMF

 Du et al. [29]

Retrospective

n = 12

n = 12 biopsied

n = 12 oral CS, CS pulse therapy, MMF, heparin, dipyridamol

n = 12 ACE inhibitors

8.33

(6–12)

n = 12 nephrotic-range proteinuria

3 months

n = 12 decrease of proteinuria to a minimum of 50% of pretreatment level

 Ren et al. [30]

Retrospective

n = 53

n = 53 biopsied

n = 26 oral CS

n = 27 MMF

27 (14–62)

n = 53 heavy proteinuria

n = 4 renal insufficiency

6 months

n = 42 remission

Azathioprin

 Ninchoji et al. [31]

Retrospective

n = 50

n = 50 biopsied

n = 31 ACE inhibitors and/or AT1-antagonist

n = 19 oral CS + AZA/mizoribin, warfarin

8.73 ± 0.54*

*not specified whether median/mean

n = 31 moderately severe IgAVN (ISKDC I-III)

n = 19 severe IgAVN (ISKDC IV-V)

4.3 months

50% resolution of proteinuria in

moderately severe IgAVN

5.2 months

50% resolution of proteinuria in

severe IgAVN

 Altugan et al. [32]

Prospective

n = 18

n = 18 biopsied

n = 18 Oral CS ± CYC ± AZA

n = 18 ACE inhibitors

11.2 ± 4.0*

*mean age

n = 1 nephrotic syndrome

n = 4 nephritic syndrome

n = 13 nephritic syndrome + heavy proteinuria

n = 7 renal insufficiency

4–6 months

n = 18 resolution of proteinuria