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Flow diagram of study selection

Figuras y tablas -
Figure 1

Flow diagram of study selection

Risk of bias: Review authors' judgements about each methodological quality item presented as percentages across all included studies.

Figuras y tablas -
Figure 2

Risk of bias: Review authors' judgements about each methodological quality item presented as percentages across all included studies.

Risk of bias: Review authors' judgements about each risk of bias item for each included study

Figuras y tablas -
Figure 3

Risk of bias: Review authors' judgements about each risk of bias item for each included study

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 1: Persistent kidney disease at any time after treatment

Figuras y tablas -
Analysis 1.1

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 1: Persistent kidney disease at any time after treatment

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 2: Number of children with any continuing kidney disease at different time points

Figuras y tablas -
Analysis 1.2

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 2: Number of children with any continuing kidney disease at different time points

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 3: Any continuing kidney disease at different time points (study with high risk of bias excluded)

Figuras y tablas -
Analysis 1.3

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 3: Any continuing kidney disease at different time points (study with high risk of bias excluded)

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 4: Number of children with kidney disease in first month/with kidney disease at follow‐up

Figuras y tablas -
Analysis 1.4

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 4: Number of children with kidney disease in first month/with kidney disease at follow‐up

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 5: Number developing severe kidney disease

Figuras y tablas -
Analysis 1.5

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 5: Number developing severe kidney disease

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 6: Duration of kidney disease

Figuras y tablas -
Analysis 1.6

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 6: Duration of kidney disease

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 7: Gastrointestinal complications requiring hospital admission

Figuras y tablas -
Analysis 1.7

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 7: Gastrointestinal complications requiring hospital admission

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 8: Eight‐year outcomes

Figuras y tablas -
Analysis 1.8

Comparison 1: Prednisone versus placebo/supportive treatment for preventing persistent kidney disease, Outcome 8: Eight‐year outcomes

Comparison 2: Antiplatelet agents versus supportive treatment for preventing persistent kidney disease, Outcome 1: Kidney disease at any time

Figuras y tablas -
Analysis 2.1

Comparison 2: Antiplatelet agents versus supportive treatment for preventing persistent kidney disease, Outcome 1: Kidney disease at any time

Comparison 3: Heparin versus placebo for preventing persistent kidney disease, Outcome 1: Any kidney disease at 2 to 3 months after onset or relapse

Figuras y tablas -
Analysis 3.1

Comparison 3: Heparin versus placebo for preventing persistent kidney disease, Outcome 1: Any kidney disease at 2 to 3 months after onset or relapse

Comparison 3: Heparin versus placebo for preventing persistent kidney disease, Outcome 2: Type of kidney disease at 2 to 3 months or more after onset or relapse

Figuras y tablas -
Analysis 3.2

Comparison 3: Heparin versus placebo for preventing persistent kidney disease, Outcome 2: Type of kidney disease at 2 to 3 months or more after onset or relapse

Comparison 3: Heparin versus placebo for preventing persistent kidney disease, Outcome 3: Time to development of kidney disease

Figuras y tablas -
Analysis 3.3

Comparison 3: Heparin versus placebo for preventing persistent kidney disease, Outcome 3: Time to development of kidney disease

Comparison 4: Cyclophosphamide versus supportive treatment for treating severe kidney disease, Outcome 1: Persistent kidney disease

Figuras y tablas -
Analysis 4.1

Comparison 4: Cyclophosphamide versus supportive treatment for treating severe kidney disease, Outcome 1: Persistent kidney disease

Comparison 4: Cyclophosphamide versus supportive treatment for treating severe kidney disease, Outcome 2: Persistent severe kidney disease

Figuras y tablas -
Analysis 4.2

Comparison 4: Cyclophosphamide versus supportive treatment for treating severe kidney disease, Outcome 2: Persistent severe kidney disease

Comparison 4: Cyclophosphamide versus supportive treatment for treating severe kidney disease, Outcome 3: ESKD

Figuras y tablas -
Analysis 4.3

Comparison 4: Cyclophosphamide versus supportive treatment for treating severe kidney disease, Outcome 3: ESKD

Comparison 5: Cyclophosphamide + steroids versus steroids for treating severe kidney disease, Outcome 1: Primary outcome: BVAS at 6 months

Figuras y tablas -
Analysis 5.1

Comparison 5: Cyclophosphamide + steroids versus steroids for treating severe kidney disease, Outcome 1: Primary outcome: BVAS at 6 months

Comparison 5: Cyclophosphamide + steroids versus steroids for treating severe kidney disease, Outcome 2: Secondary endpoints at 12 months

Figuras y tablas -
Analysis 5.2

Comparison 5: Cyclophosphamide + steroids versus steroids for treating severe kidney disease, Outcome 2: Secondary endpoints at 12 months

Comparison 5: Cyclophosphamide + steroids versus steroids for treating severe kidney disease, Outcome 3: Adverse effects

Figuras y tablas -
Analysis 5.3

Comparison 5: Cyclophosphamide + steroids versus steroids for treating severe kidney disease, Outcome 3: Adverse effects

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 1: Number with resolution of proteinuria and haematuria at 2 years

Figuras y tablas -
Analysis 6.1

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 1: Number with resolution of proteinuria and haematuria at 2 years

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 2: Number with recurrence of proteinuria and haematuria at 2 years

Figuras y tablas -
Analysis 6.2

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 2: Number with recurrence of proteinuria and haematuria at 2 years

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 3: Laboratory parameters

Figuras y tablas -
Analysis 6.3

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 3: Laboratory parameters

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 4: Adverse effects

Figuras y tablas -
Analysis 6.4

Comparison 6: Tacrolimus versus IV cyclophosphamide for treating severe kidney disease, Outcome 4: Adverse effects

Comparison 7: Cyclosporin versus methylprednisolone for treating severe kidney disease, Outcome 1: Number with remission at 3 months

Figuras y tablas -
Analysis 7.1

Comparison 7: Cyclosporin versus methylprednisolone for treating severe kidney disease, Outcome 1: Number with remission at 3 months

Comparison 7: Cyclosporin versus methylprednisolone for treating severe kidney disease, Outcome 2: Number with remission at last follow‐up (mean 6.3 years)

Figuras y tablas -
Analysis 7.2

Comparison 7: Cyclosporin versus methylprednisolone for treating severe kidney disease, Outcome 2: Number with remission at last follow‐up (mean 6.3 years)

Comparison 8: Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease, Outcome 1: Number with complete remission

Figuras y tablas -
Analysis 8.1

Comparison 8: Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease, Outcome 1: Number with complete remission

Comparison 8: Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease, Outcome 2: Number with complete or partial remission

Figuras y tablas -
Analysis 8.2

Comparison 8: Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease, Outcome 2: Number with complete or partial remission

Comparison 8: Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease, Outcome 3: Adverse effects

Figuras y tablas -
Analysis 8.3

Comparison 8: Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease, Outcome 3: Adverse effects

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 1: Remission of proteinuria at 1 year

Figuras y tablas -
Analysis 9.1

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 1: Remission of proteinuria at 1 year

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 2: Regression of histological lesions at 1 year

Figuras y tablas -
Analysis 9.2

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 2: Regression of histological lesions at 1 year

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 3: Relapse of IgAV

Figuras y tablas -
Analysis 9.3

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 3: Relapse of IgAV

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 4: Glomerular filtration rate

Figuras y tablas -
Analysis 9.4

Comparison 9: Mycophenolate mofetil versus azathioprine for treating severe kidney disease, Outcome 4: Glomerular filtration rate

Comparison 10: Mycophenolate mofetil versus leflunomide for treating severe kidney disease, Outcome 1: 24‐hour urine proteinuria

Figuras y tablas -
Analysis 10.1

Comparison 10: Mycophenolate mofetil versus leflunomide for treating severe kidney disease, Outcome 1: 24‐hour urine proteinuria

Comparison 11: Double filtration plasmapheresis versus no plasmapheresis for treating severe kidney disease, Outcome 1: Remission

Figuras y tablas -
Analysis 11.1

Comparison 11: Double filtration plasmapheresis versus no plasmapheresis for treating severe kidney disease, Outcome 1: Remission

Comparison 11: Double filtration plasmapheresis versus no plasmapheresis for treating severe kidney disease, Outcome 2: Adverse effects

Figuras y tablas -
Analysis 11.2

Comparison 11: Double filtration plasmapheresis versus no plasmapheresis for treating severe kidney disease, Outcome 2: Adverse effects

Comparison 12: Fosinopril + supportive treatment versus supportive treatment for treating proteinuria in IgAV, Outcome 1: Proteinuria

Figuras y tablas -
Analysis 12.1

Comparison 12: Fosinopril + supportive treatment versus supportive treatment for treating proteinuria in IgAV, Outcome 1: Proteinuria

Summary of findings 1. Prednisone versus placebo or supportive treatment for preventing persistent kidney disease in patients with IgAV (Henoch‐ Schönlein Purpura)

Prednisone versus placebo or supportive treatment for preventing persistent kidney disease in patients with IgAV (Henoch‐Schönlein Purpura)

Patient or population: patients with IgAV
Settings: all settings
Intervention: prednisone
Comparison: placebo or supportive treatment

Outcomes

Illustrative comparative risks* (95% CI)

Relative effect
(95% CI)

No. of participants
(studies)

Certainty of the evidence
(GRADE)

Assumed risk

Corresponding risk

Placebo or supportive treatment

Prednisone

Persistent kidney disease at any time after treatment

143 per 1000

106 per 1000
(60 to 189)

RR 0.74 
(0.42 to 1.32)

746 (5)

⊕⊕⊕⊝
Moderate1

Children with any continuing kidney disease at 3 months

199 per 1000

165 per 1000
(92 to 303)

RR 0.83 
(0.46 to 1.52)

655 (4)

⊕⊕⊕⊝
Moderate2

Children with any continuing kidney disease at 6 months

100 per 1000

51 per 1000
(24 to 111)

RR 0.51 
(0.24 to 1.11)

379 (3)

⊕⊕⊕⊝
Moderate2

Children with any continuing kidney disease at 12 months

84 per 1000

89 per 1000
(32 to 244)

RR 1.06 
(0.38 to 2.91)

455 (3)

⊕⊕⊝⊝
Low2,3

Any continuing kidney disease at 3 months

(study with high risk of bias excluded)

243 per 1000

238 per 1000
(170 to 330)

RR 0.98 
(0.70 to 1.36)

487 (3)

⊕⊕⊕⊕
High

Any continuing kidney disease at 12 months

(study with high risk of bias excluded)

105 per 1000

146 per 1000
(79 to 272)

RR 1.39 
(0.75 to 2.59)

287 (2)

⊕⊕⊕⊝
Moderate3,4

Number developing severe kidney disease

14 per 1000

22 per 1000
(6 to 85)

RR 1.58 
(0.42 to 6)

418 (2)

⊕⊕⊝⊝
Low3,5

*The basis for the assumed risk (e.g. the median control group risk across studies) is provided in the footnotes. The corresponding risk (and its 95% CI) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
CI: confidence interval; RR: risk ratio

GRADE Working Group grades of evidence
High certainty: Further research is very unlikely to change our confidence in the estimate of effect
Moderate certainty: Further research is likely to have an important impact on our confidence in the estimate of effect and may change the estimate
Low certainty: Further research is very likely to have an important impact on our confidence in the estimate of effect and is likely to change the estimate
Very low certainty: We are very uncertain about the estimate
 

1 Two studies had unclear or biased allocation concealment & were not blinded
2 One study had inadequate allocation concealment & no blinding & one study had large loss to follow‐up
3 30% loss to follow‐up in largest included study
4 Small numbers of patients and events
5 Small numbers of events

Figuras y tablas -
Summary of findings 1. Prednisone versus placebo or supportive treatment for preventing persistent kidney disease in patients with IgAV (Henoch‐ Schönlein Purpura)
Comparison 1. Prednisone versus placebo/supportive treatment for preventing persistent kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

1.1 Persistent kidney disease at any time after treatment Show forest plot

5

746

Risk Ratio (M‐H, Random, 95% CI)

0.74 [0.42, 1.32]

1.2 Number of children with any continuing kidney disease at different time points Show forest plot

4

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

1.2.1 One month

4

655

Risk Ratio (M‐H, Random, 95% CI)

0.80 [0.34, 1.84]

1.2.2 Three months

4

655

Risk Ratio (M‐H, Random, 95% CI)

0.83 [0.46, 1.52]

1.2.3 Six months

3

379

Risk Ratio (M‐H, Random, 95% CI)

0.51 [0.24, 1.11]

1.2.4 Twelve months

3

455

Risk Ratio (M‐H, Random, 95% CI)

1.06 [0.38, 2.91]

1.3 Any continuing kidney disease at different time points (study with high risk of bias excluded) Show forest plot

3

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

1.3.1 One month

3

487

Risk Ratio (M‐H, Random, 95% CI)

1.02 [0.54, 1.93]

1.3.2 Three months

3

487

Risk Ratio (M‐H, Random, 95% CI)

0.98 [0.70, 1.36]

1.3.3 Six months

2

211

Risk Ratio (M‐H, Random, 95% CI)

0.59 [0.23, 1.50]

1.3.4 Twelve months

2

287

Risk Ratio (M‐H, Random, 95% CI)

1.39 [0.75, 2.59]

1.4 Number of children with kidney disease in first month/with kidney disease at follow‐up Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.4.1 One month

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.4.2 Three months

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.4.3 Six months

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.5 Number developing severe kidney disease Show forest plot

2

418

Risk Ratio (M‐H, Random, 95% CI)

1.58 [0.42, 6.00]

1.6 Duration of kidney disease Show forest plot

1

Mean Difference (IV, Random, 95% CI)

Totals not selected

1.6.1 Haematuria

1

Mean Difference (IV, Random, 95% CI)

Totals not selected

1.6.2 Proteinuria

1

Mean Difference (IV, Random, 95% CI)

Totals not selected

1.7 Gastrointestinal complications requiring hospital admission Show forest plot

3

517

Risk Ratio (M‐H, Random, 95% CI)

0.56 [0.25, 1.23]

1.8 Eight‐year outcomes Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.8.1 Haematuria

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.8.2 Proteinuria

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.8.3 Haematuria and proteinuria

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.8.4 Hypertension

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

1.8.5 Decreased GFR (Schwartz formula)

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

Figuras y tablas -
Comparison 1. Prednisone versus placebo/supportive treatment for preventing persistent kidney disease
Comparison 2. Antiplatelet agents versus supportive treatment for preventing persistent kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

2.1 Kidney disease at any time Show forest plot

3

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

2.1.1 Dipyridamole ± cyproheptadine in children without kidney disease at entry

2

101

Risk Ratio (M‐H, Random, 95% CI)

1.16 [0.46, 2.95]

2.1.2 Dipyridamole ± cyproheptadine in children with kidney disease at entry

1

19

Risk Ratio (M‐H, Random, 95% CI)

0.92 [0.23, 3.72]

2.1.3 Aspirin versus supportive treatment

1

18

Risk Ratio (M‐H, Random, 95% CI)

0.14 [0.01, 2.42]

Figuras y tablas -
Comparison 2. Antiplatelet agents versus supportive treatment for preventing persistent kidney disease
Comparison 3. Heparin versus placebo for preventing persistent kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

3.1 Any kidney disease at 2 to 3 months after onset or relapse Show forest plot

2

317

Risk Ratio (M‐H, Random, 95% CI)

0.48 [0.15, 1.54]

3.2 Type of kidney disease at 2 to 3 months or more after onset or relapse Show forest plot

2

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

3.2.1 Haematuria

2

317

Risk Ratio (M‐H, Random, 95% CI)

0.39 [0.07, 2.21]

3.2.2 Proteinuria

2

317

Risk Ratio (M‐H, Random, 95% CI)

0.47 [0.31, 0.73]

3.2.3 Nephrotic syndrome

1

228

Risk Ratio (M‐H, Random, 95% CI)

0.31 [0.03, 2.89]

3.3 Time to development of kidney disease Show forest plot

1

Mean Difference (IV, Random, 95% CI)

Subtotals only

Figuras y tablas -
Comparison 3. Heparin versus placebo for preventing persistent kidney disease
Comparison 4. Cyclophosphamide versus supportive treatment for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

4.1 Persistent kidney disease Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

4.2 Persistent severe kidney disease Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

4.3 ESKD Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

Figuras y tablas -
Comparison 4. Cyclophosphamide versus supportive treatment for treating severe kidney disease
Comparison 5. Cyclophosphamide + steroids versus steroids for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

5.1 Primary outcome: BVAS at 6 months Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.1.1 BVAS = 0 at 6 months

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.1.2 Improvement in BVAS score

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2 Secondary endpoints at 12 months Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2.1 BP > 125/75 mm Hg

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2.2 eGFR < 60 mL/min

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2.3 Proteinuria > 1 g/day

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2.4 RAS blockers

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2.5 Kidney function improvement > 50%

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2.6 ESKD

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.2.7 Death

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

5.3 Adverse effects Show forest plot

1

Risk Difference (M‐H, Random, 95% CI)

Totals not selected

5.3.1 infection

1

Risk Difference (M‐H, Random, 95% CI)

Totals not selected

5.3.2 Newly diagnosed or deterioration in existing diabetes

1

Risk Difference (M‐H, Random, 95% CI)

Totals not selected

5.3.3 Depression/anxiety

1

Risk Difference (M‐H, Random, 95% CI)

Totals not selected

5.3.4 Alopecia

1

Risk Difference (M‐H, Random, 95% CI)

Totals not selected

5.3.5 Insomnia

1

Risk Difference (M‐H, Random, 95% CI)

Totals not selected

Figuras y tablas -
Comparison 5. Cyclophosphamide + steroids versus steroids for treating severe kidney disease
Comparison 6. Tacrolimus versus IV cyclophosphamide for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

6.1 Number with resolution of proteinuria and haematuria at 2 years Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

6.1.1 Number without proteinuria

1

170

Risk Ratio (M‐H, Random, 95% CI)

1.13 [0.99, 1.30]

6.1.2 Number without haematuria

1

170

Risk Ratio (M‐H, Random, 95% CI)

1.18 [1.02, 1.37]

6.2 Number with recurrence of proteinuria and haematuria at 2 years Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

6.2.1 Number with proteinuria

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.72 [0.45, 1.15]

6.2.2 Number with haematuria

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.95 [0.61, 1.48]

6.3 Laboratory parameters Show forest plot

1

Mean Difference (IV, Random, 95% CI)

Subtotals only

6.3.1 Serum creatinine at 3 months

1

170

Mean Difference (IV, Random, 95% CI)

‐1.00 [‐2.80, 0.80]

6.3.2 Serum creatinine at 6 months

1

170

Mean Difference (IV, Random, 95% CI)

‐1.00 [‐2.06, 0.06]

6.3.3 24‐hour urinary protein at 3 months

1

170

Mean Difference (IV, Random, 95% CI)

‐0.31 [‐0.40, ‐0.22]

6.3.4 24‐hour urinary protein at 6 months

1

170

Mean Difference (IV, Random, 95% CI)

‐0.20 [‐0.24, ‐0.16]

6.3.5 Urine RBC/HPF at 3 months

1

170

Mean Difference (IV, Random, 95% CI)

‐2.62 [‐3.79, ‐1.45]

6.3.6 Urine RBC/HPF at 6 months

1

170

Mean Difference (IV, Random, 95% CI)

‐2.45 [‐2.90, ‐2.00]

6.4 Adverse effects Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

6.4.1 Leucopenia

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.64 [0.19, 2.17]

6.4.2 Respiratory infections

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.55 [0.38, 0.82]

6.4.3 Abnormal liver function tests

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.92 [0.58, 1.45]

6.4.4 Urinary tract infection

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.56 [0.27, 1.15]

6.4.5 Poor appetite

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.82 [0.47, 1.42]

6.4.6 Other serious adverse effect

1

170

Risk Ratio (M‐H, Random, 95% CI)

0.45 [0.20, 0.98]

Figuras y tablas -
Comparison 6. Tacrolimus versus IV cyclophosphamide for treating severe kidney disease
Comparison 7. Cyclosporin versus methylprednisolone for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

7.1 Number with remission at 3 months Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

7.2 Number with remission at last follow‐up (mean 6.3 years) Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

Figuras y tablas -
Comparison 7. Cyclosporin versus methylprednisolone for treating severe kidney disease
Comparison 8. Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

8.1 Number with complete remission Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

8.1.1 Three months

1

68

Risk Ratio (M‐H, Random, 95% CI)

1.20 [0.72, 1.99]

8.1.2 Six months

1

68

Risk Ratio (M‐H, Random, 95% CI)

1.02 [0.78, 1.34]

8.1.3 Twelve months

1

68

Risk Ratio (M‐H, Random, 95% CI)

1.06 [0.83, 1.35]

8.2 Number with complete or partial remission Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

8.2.1 Three months

1

68

Risk Ratio (M‐H, Random, 95% CI)

0.87 [0.75, 1.02]

8.2.2 Six months

1

68

Risk Ratio (M‐H, Random, 95% CI)

0.87 [0.75, 1.02]

8.2.3 Twelve months

1

68

Risk Ratio (M‐H, Random, 95% CI)

0.87 [0.75, 1.02]

8.3 Adverse effects Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

8.3.1 Cerebral abscess

1

68

Risk Ratio (M‐H, Random, 95% CI)

0.35 [0.01, 8.37]

8.3.2 Abnormal LFTS requiring treatment change

1

68

Risk Ratio (M‐H, Random, 95% CI)

5.29 [0.26, 106.33]

8.3.3 Infection

1

68

Risk Ratio (M‐H, Random, 95% CI)

0.71 [0.37, 1.35]

8.3.4 Gastrointestinal upsets

1

68

Risk Ratio (M‐H, Random, 95% CI)

3.18 [0.35, 29.08]

Figuras y tablas -
Comparison 8. Mycophenolate mofetil versus IV cyclophosphamide for treating severe kidney disease
Comparison 9. Mycophenolate mofetil versus azathioprine for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

9.1 Remission of proteinuria at 1 year Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

9.2 Regression of histological lesions at 1 year Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

9.3 Relapse of IgAV Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

9.4 Glomerular filtration rate Show forest plot

1

Mean Difference (IV, Random, 95% CI)

Totals not selected

Figuras y tablas -
Comparison 9. Mycophenolate mofetil versus azathioprine for treating severe kidney disease
Comparison 10. Mycophenolate mofetil versus leflunomide for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

10.1 24‐hour urine proteinuria Show forest plot

1

Mean Difference (IV, Random, 95% CI)

Subtotals only

10.1.1 Three months

1

18

Mean Difference (IV, Random, 95% CI)

360.00 [‐48.50, 768.50]

10.1.2 Nine months

1

18

Mean Difference (IV, Random, 95% CI)

49.00 [2.78, 95.22]

Figuras y tablas -
Comparison 10. Mycophenolate mofetil versus leflunomide for treating severe kidney disease
Comparison 11. Double filtration plasmapheresis versus no plasmapheresis for treating severe kidney disease

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

11.1 Remission Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

11.1.1 Complete remission after 3 courses of treatment

1

60

Risk Ratio (M‐H, Random, 95% CI)

1.43 [0.63, 3.25]

11.1.2 Complete and partial remission after 3 courses of treatment

1

60

Risk Ratio (M‐H, Random, 95% CI)

1.26 [0.91, 1.75]

11.1.3 Complete remission at 6 months

1

60

Risk Ratio (M‐H, Random, 95% CI)

1.13 [0.89, 1.44]

11.2 Adverse effects Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Subtotals only

11.2.1 Hypertension

1

60

Risk Ratio (M‐H, Random, 95% CI)

0.78 [0.33, 1.82]

11.2.2 Leucopenia

1

60

Risk Ratio (M‐H, Random, 95% CI)

3.00 [0.13, 70.83]

11.2.3 Need for haemodialysis

1

60

Risk Ratio (M‐H, Random, 95% CI)

0.55 [0.23, 1.28]

11.2.4 Respiratory infections

1

60

Risk Ratio (M‐H, Random, 95% CI)

1.33 [0.53, 3.38]

11.2.5 GIT disturbances

1

60

Risk Ratio (M‐H, Random, 95% CI)

1.22 [0.59, 2.51]

Figuras y tablas -
Comparison 11. Double filtration plasmapheresis versus no plasmapheresis for treating severe kidney disease
Comparison 12. Fosinopril + supportive treatment versus supportive treatment for treating proteinuria in IgAV

Outcome or subgroup title

No. of studies

No. of participants

Statistical method

Effect size

12.1 Proteinuria Show forest plot

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

12.1.1 Complete remission of proteinuria < 150 mg/day

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

12.1.2 Partial remission

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

12.1.3 Minimal response/no response

1

Risk Ratio (M‐H, Random, 95% CI)

Totals not selected

Figuras y tablas -
Comparison 12. Fosinopril + supportive treatment versus supportive treatment for treating proteinuria in IgAV