Cochrane · systematic review · CD003594

Interventions for idiopathic steroid‐resistant nephrotic syndrome in children

Liu, ID, Willis, NS, Craig, JC, et al. · Cochrane Database of Systematic Reviews · 2019 · Cochrane Library

Kidney and Transplant3 versionsAMSTAR 2: not assessed
SoF tableOnly the outcomes this review put in its Summary of Findings table are listed (the Cochrane Handbook caps them at about seven per comparison). Its other analyses are not shown here.
Questions
62
current version
GRADE rated
62
No studies
0
Included studies
NR
No studies 0 · 0%Ungraded 0 · 0%Very low 14 · 22.6%Low 42 · 67.7%Moderate 6 · 9.7%High 0 · 0%

Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.

Questions and results

Show all 3 versions

Cyclosporin versus placebo or no treatment for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission: all renal pathologies
Cyclosporin vs placebo or no treatment
RR 3.5 [1.09, 11.2]4 studies · 74 participantsLow
Downgraded: risk of bias
Complete remission: FSGS
Cyclosporin vs placebo or no treatment
RR 3.14 [0.97, 10.18]3 studies · 58 participantsLow
Downgraded: risk of bias
Complete or partial remission: all renal pathologies
Cyclosporin vs placebo or no treatment
RR 3.15 [1.04, 9.57]4 studies · 74 participantsLow
Downgraded: risk of bias
Complete or partial remission: FSGS
Cyclosporin vs placebo or no treatment
RR 2.66 [0.85, 8.31]2 studies · 49 participantsLow
Downgraded: risk of bias
Adverse events: worsening of hypertension
Cyclosporin vs placebo or no treatment
RR 1 [0.17, 5.98]1 study · 24 participantsVery low
Downgraded: risk of bias
Adverse events: infection
Cyclosporin vs placebo or no treatment
RR 0.7 [0.2, 2.51]1 study · 17 participantsVery low
Downgraded: risk of bias
Adverse events: ESKD
Cyclosporin vs placebo or no treatment
RR 0.23 [0.03, 1.79]1 study · 25 participantsVery low
Downgraded: risk of bias

Calcineurin inhibitor versus IV cyclophosphamide for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 3 to 6 months: complete or partial remission
Calcineurin inhibitor vs IV cyclophosphamide
RR 1.98 [1.25, 3.13]2 studies · 156 participantsLow
Downgraded: risk of bias, imprecision
Treatment response at 3 to 6 months: complete remission
Calcineurin inhibitor vs IV cyclophosphamide
RR 3.43 [1.84, 6.41]2 studies · 156 participantsLow
Downgraded: risk of bias, imprecision
Treatment response at 3 to 6 months: partial remission
Calcineurin inhibitor vs IV cyclophosphamide
RR 1.68 [0.43, 6.56]2 studies · 156 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events: treatment failure at 6 months (non response, serious infection, persistently elevated creatinine)
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.32 [0.18, 0.58]1 study · 124 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: medications ceased due to adverse events
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.2 [0.04, 0.86]1 study · 131 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: serious infections
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.49 [0.16, 1.56]1 study · 131 participantsModerate
Downgraded: risk of bias, imprecision, inconsistency
Adverse events: death
Calcineurin inhibitor vs IV cyclophosphamide
RR 0.33 [0.01, 7.92]1 study · 131 participantsLow
Downgraded: risk of bias, imprecision, inconsistency

Tacrolimus versus cyclosporin for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
6 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 6 months: complete remission
Tacrolimus vs cyclosporin
RR 1.14 [0.64, 2.03]1 study · 41 participantsLow
Treatment response at 6 months: complete and partial remission
Tacrolimus vs cyclosporin
RR 0.57 [0.16, 2.08]1 study · 41 participantsLow
Treatment response at 12 months: complete remission
Tacrolimus vs cyclosporin
RR 0.8 [0.45, 1.42]2 studies · 58 participantsLow
Downgraded: risk of bias, imprecision
Treatment response at 12 months: complete and partial remission
Tacrolimus vs cyclosporin
RR 1.05 [0.87, 1.25]2 studies · 58 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: persistent nephrotoxicity
Tacrolimus vs cyclosporin
RR 0.48 [0.05, 4.85]1 study · 41 participantsLow
Adverse events: worsening of hypertension
Tacrolimus vs cyclosporin
no estimate extracted2 studies · 58 participantsLow
Downgraded: risk of bias, imprecision

Cyclosporin versus mycophenolate mofetil with pulse dexamethasone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 52 weeks: complete remission (primary outcome 1, 2)
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 2.14 [0.87, 5.24]1 study · 138 participantsModerate
Treatment response at 52 weeks: partial remission (primary outcome 3)
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 1.09 [0.61, 1.93]1 study · 138 participantsModerate
Sustainable remission between 52 and 78 weeks: complete or partial remission (primary outcome 1, 2, 3)
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 1.38 [0.9, 2.1]1 study · 138 participantsModerate
CKD or death: death by 52 weeks
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 0.18 [0.01, 3.75]1 study · 138 participantsLow
CKD or death: 50% decline in GFR by 78 weeks
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 2.29 [0.46, 11.41]1 study · 138 participantsLow
Adverse events (weeks 0 to 26): serious infection requiring hospitalisation
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 0.65 [0.22, 1.96]1 study · 138 participantsLow
Adverse events (weeks 0 to 26): hypertension
Cyclosporin vs mycophenolate mofetil with pulse dexamethasone
RR 1.68 [0.66, 4.29]1 study · 138 participantsLow

Tacrolimus versus mycophenolate mofetil to maintain remission for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Number with complete or partial response at one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 2.01 [1.32, 3.07]1 study · 60 participantsLow
Downgraded: risk of bias
Number with complete response at one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 1.79 [1.11, 2.9]1 study · 60 participantsLow
Downgraded: risk of bias
Number with partial response at one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 4.68 [0.58, 37.68]1 study · 60 participantsLow
Downgraded: risk of bias
Number with treatment failure by one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 0.18 [0.06, 0.54]1 study · 60 participantsLow
Downgraded: risk of bias
Number with frequent relapses by one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 0.28 [0.09, 0.92]1 study · 60 participantsLow
Downgraded: risk of bias
Number with steroid resistance by one year
Tacrolimus vs mycophenolate mofetil to maintain remission
RR 0.07 [0, 1.23]1 study · 60 participantsLow
Downgraded: risk of bias
Change in GFR
Tacrolimus vs mycophenolate mofetil to maintain remission
no estimate extracted1 study · 60 participantsLow
Downgraded: risk of bias

Oral cyclophosphamide versus prednisone or placebo for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission: all renal pathologies
Oral cyclophosphamide vs prednisone or placebo
RR 1.06 [0.61, 1.87]2 studies · 84 participantsLow
Downgraded: risk of bias
Complete remission: FSGS
Oral cyclophosphamide vs prednisone or placebo
RR 1.01 [0.43, 2.37]2 studies · 63 participantsLow
Downgraded: risk of bias
Complete or partial remission
Oral cyclophosphamide vs prednisone or placebo
RR 0.88 [0.53, 1.45]1 study · 53 participantsLow
Downgraded: risk of bias
Complete or partial remission: FSGS
Oral cyclophosphamide vs prednisone or placebo
RR 0.88 [0.53, 1.45]1 study · 53 participantsLow
Downgraded: risk of bias
Treatment failure
Oral cyclophosphamide vs prednisone or placebo
RR 1.59 [0.87, 2.88]1 study · 60 participantsLow
Downgraded: risk of bias
Adverse events: death (all causes)
Oral cyclophosphamide vs prednisone or placebo
RR 1.07 [0.19, 5.95]1 study · 60 participantsLow
Downgraded: risk of bias
Adverse events: hypertension with seizures
Oral cyclophosphamide vs prednisone or placebo
RR 0.71 [0.05, 10.89]1 study · 60 participantsVery low
Downgraded: risk of bias

IV versus oral cyclophosphamide for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Complete remission
IV vs oral cyclophosphamide
RR 1.58 [0.65, 3.85]2 studies · 61 participantsLow
Downgraded: risk of bias, imprecision
Partial remission
IV vs oral cyclophosphamide
RR 1 [0.15, 6.55]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision
Continuing remission at one year
IV vs oral cyclophosphamide
RR 0.75 [0.19, 3.01]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: renal insufficiency
IV vs oral cyclophosphamide
RR 0.33 [0.04, 2.99]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision
Adverse events: bacterial infection
IV vs oral cyclophosphamide
RR 1.02 [0.1, 10.62]2 studies · 61 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events: vomiting
IV vs oral cyclophosphamide
RR 2.38 [0.35, 16.17]2 studies · 61 participantsVery low
Downgraded: risk of bias, imprecision
Adverse events: alopecia
IV vs oral cyclophosphamide
RR 1.5 [0.27, 8.22]1 study · 50 participantsLow
Downgraded: risk of bias, imprecision

IV cyclophosphamide versus oral cyclophosphamide plus IV dexamethasone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Treatment response at 6 months: complete remission
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 1.13 [0.65, 1.96]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 6 months: partial remission
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.88 [0.14, 5.79]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 6 months: complete or partial remission
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 1.09 [0.68, 1.74]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 18 months: sustained remission/steroid‐sensitive relapses
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 1.13 [0.65, 1.96]1 study · 49 participantsLow
Downgraded: imprecision
Treatment response at 18 months: CKD
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.88 [0.06, 13.35]1 study · 49 participantsLow
Downgraded: imprecision
Adverse events: hypertension
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.04 [0, 0.68]1 study · 49 participantsLow
Downgraded: imprecision
Adverse events: bacterial infections
IV cyclophosphamide vs oral cyclophosphamide plus IV dexamethasone
RR 0.66 [0.27, 1.63]1 study · 49 participantsLow
Downgraded: imprecision

Rituximab/cyclosporin/prednisolone versus cyclosporin/prednisolone for idiopathic steroid‐resistant nephrotic syndrome in children

Population: idiopathic steroid‐resistant nephrotic syndrome in children
7 questions
OutcomeEffectEvidenceStatus / GRADE
Number with complete remission: complete remission in initial steroid resistance
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
no estimate extracted1 study · 16 participantsVery low
Downgraded: risk of bias
Number with complete remission: complete remission in delayed steroid resistance
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 1.14 [0.33, 3.94]1 study · 15 participantsVery low
Downgraded: risk of bias
Number with complete remission: complete remission in all patients
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 0.94 [0.22, 3.94]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: bronchospasm/treatment discontinued
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 2.82 [0.12, 64.39]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: hypotension
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 2.82 [0.12, 64.39]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: skin rash
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 6.59 [0.37, 117.77]1 study · 31 participantsVery low
Downgraded: risk of bias
Adverse events: mild dyspnoea
Rituximab/cyclosporin/prednisolone vs cyclosporin/prednisolone
RR 4.71 [0.24, 90.69]1 study · 31 participantsVery low
Downgraded: risk of bias