Introduction
Current approaches
Supportive management
Diet modification
Weight optimisation
Smoking cessation
Fish oil supplementation
Renin–angiotensin system blockade
Tonsillectomy
Immunosuppression
Novel therapies
Atacicept (NCT02808429) and blisibimod (NCT02062684)
Trial | Intervention | Inclusion criteria | Exclusion criteria | Trial design | Primary end point | Follow-up duration |
---|---|---|---|---|---|---|
Atacicept NCT02808429 | Atacicept at varying doses vs placebo | Proteinuria 1–6 g/day Stabilised on RASi for 8 weeks | Prior cyclophosphamide treatment Use of other immunosuppressants within 4 months | Randomised, double-blind, placebo-controlled Phase II trial | Incidence of adverse events | 180 weeks |
BRIGHT-SC NCT02062684 | Blisibimod vs placebo | Proteinuria 1–6 g/day Stabilised on RASi for 8 weeks | Immunosuppressant use over last 6 months or corticosteroid use over last 3 months. Malignancy over last 5 years | Randomised, double-blind, placebo-controlled Phase II/III trial | Reduction of proteinuria at 24 weeks | 104 weeks |
SIGN NCT02112838 | Fostamatinib at varying doses vs placebo | Stabilised on RASi for 90 days. BP < 130/80 Proteinuria > 1 g/day at diagnosis and > 0.5 g/day at second screening visit | Recent use of corticosteroids, cyclophosphamide, mycophenolate mofetil, azathioprine or rituximab | Randomised, multicentre, double-blind, placebo-controlled, Phase II trial | Reduction of proteinuria at 24 weeks | 24 weeks |
VELCADE NCT01103778 | Bortezomib | Proteinuria > 1 g/day Stabilised on RASi for 4 weeks | Peripheral neuropathy, history of cardiac problems, malignancy within last 3 years | Open-label, Phase IV trial | Reduction of proteinuria at 1 year | 1 year |
ACTHAR NCT02282930 | Acthar gel | Proteinuria > 1 g/day Stabilised on RASi for 3 months BP > 130/80 HSP patients included | Crohn’s disease or celiac sprue Glucocorticoid treatment in last 3 months Immunosuppressive therapy in last 6 months Previous ACTH treatment History of malignancy History of cardiac or pulmonary disease | Open-label, Phase III trial | Reduction in proteinuria at 1 year, stabilisation of eGFR at 1 year | 1 year |
OMS721 NCT02682407 | OMS721 vs placebo | Patients on immunosuppressive patients included, if on stable dose for 2 months Optimised RASi, BP < 150/90, Urine ACR > 600 mg/g | Renal transplant History of malignancy Use of belimumab, rituximab, or eculizumab within last 6 months HSP within 2 years | Randomised, double-blind, placebo-controlled, Phase II trial | Incidence of adverse events | 18 weeks |
LNP023 NCT03373461 | LNP023 vs placebo | Stabilised on RASi for 90 days eGFR ≥ 30, proteinuria ≥ 0.75 g/day | Recent use of immunosuppression, history of drug/alcohol abuse, malignancy | Randomised, double-blind, placebo-controlled Phase IIa/IIb trial | Reduction of proteinuria at 90 days | 180 Days |
Fostamatinib (NCT01738035)
Rituximab (NCT00498368)
Trial | Intervention | Inclusion criteria | Exclusion criteria | Trial design | Primary end point | Follow-up | Sample size | Outcome |
---|---|---|---|---|---|---|---|---|
Avacopan NCT02384317 | Avacopan 30 mg twice daily | After 4 weeks of optimised RASi eGFR > 60 eGFR > 45 if decline < 10 mL/min/1.73 m2 over prior 24 weeks Urine PCR > 1 g/g | Proteinuria > 8 g/day, Malignancy within 5 years, cardiac disease, immunosuppression in last 24 weeks. HSP within last 2 years | Open-label Phase II trial | Incidence of adverse events | 12 weeks | 7 | Reduction of proteinuria in 6 of 7 patients |
Rituximab NCT00498368 | Rituximab + supportive care vs supportive care alone | After 2 months of optimised RASi Proteinuria ≥ 1 g/day BP < 130/80 HSP included | eGFR < 30 > 6 months of steroids > 50% glomerular senescence or cortical scarring, history of Crohn’s disease or celiac sprue | Randomised, open-label, multicentre, Phase IV trial | Proteinuria and eGFR at 12 months | 12 months | 34, American | No effect on end points |
NEFIGAN NCT01738035 | TRF-budesonide 8 mg or 16 mg + supportive care vs supportive care alone | After 6 months of optimised RASi Urine PCR ≥ 0.5 g/g OR urine protein ≥ 0.75 g/24 h eGFR ≥ 45, BP ≤ 160/100 | Immunosuppression over previous 24 months, or at any time for IgAN Renal transplant, diabetes, malignancy over last 3 years | Randomised, multicentre, double-blind, placebo-controlled, Phase II trial | Urine PCR at 9 months and 12 months | 12 months | 149, European | Reduction in urine PCR achieved by budesonide |