Glomerular diseases

Red Flags

Rapidly progressive glomerulonephritis (creatinine doubling within 3 months, dysmorphic haematuria, red cell casts) — emergency nephrology; same-day biopsy and immunosuppression^[1]
Pulmonary-renal syndrome (haemoptysis with AKI) — anti-GBM, ANCA vasculitis; emergency immunosuppression and plasma exchange^[1]
Nephrotic syndrome with thromboembolism (PE, DVT, renal vein thrombosis) — anticoagulate and treat underlying disease; consider prophylactic anticoagulation if albumin <25 g/L on no anticoagulant^[1]
Severe hypertension with target organ damage in glomerular disease — admit; IV antihypertensive; treat underlying disease^[1]

First-line treatment

Interventions

Foundation supportive therapy^[1]
All glomerular disease — sodium restriction, RAS blockade titrated to maximum tolerated, blood-pressure target <120/80 (standardised office), statin per cardiovascular risk, treat oedema with loop diuretic, vaccinate before immunosuppression, manage VTE risk

First-line drug therapy

Drug	Class	Adult	Paediatric	Notes
Prednisolone (induction)^[1]	Systemic corticosteroid	Minimal change: 1 mg/kg/day (max 80 mg) PO × 4–8 weeks then taper. Lupus nephritis class III/IV: methylprednisolone IV pulse 250–500 mg × 3 days then prednisolone 0.5–1 mg/kg/day (max 60 mg) tapering rapidly	MCD: 60 mg/m²/day (max 60 mg) × 4–6 weeks then alternate-day taper	Ulcer prophylaxis, calcium/vitamin D, glucose monitoring, infection prophylaxis (PJP) per regimen
Mycophenolate mofetil^[1]	Inosine monophosphate dehydrogenase inhibitor	Lupus nephritis induction 2–3 g/day PO in divided doses × 6 months; maintenance 1–2 g/day	600 mg/m² BD (max 2 g/day)	First-line induction and maintenance for lupus nephritis class III/IV; teratogenic — strict contraception and switch ≥6 weeks before pregnancy
Cyclophosphamide^[1]	Alkylating agent	Euro-Lupus 500 mg IV every 2 weeks × 6 doses for lupus nephritis. ANCA vasculitis: 15 mg/kg IV every 2 weeks × 3 then every 3 weeks (max 1.2 g/dose, age- and renal-adjusted)	Per local paediatric nephrology protocol	Counsel about gonadal toxicity and bladder cancer; mesna with high-dose IV; lifetime cumulative dose monitoring; PJP prophylaxis
Rituximab^[1]	Anti-CD20 monoclonal antibody	Membranous nephropathy: 1 g IV × 2 doses 2 weeks apart, repeat at 6 months by anti-PLA2R titre. ANCA vasculitis: 375 mg/m² IV weekly × 4 induction; 1 g IV every 6 months maintenance	—	First-line for high-risk membranous nephropathy and ANCA vasculitis; screen and prophylax HBV; live-vaccine hold
Tacrolimus or ciclosporin^[1]	Calcineurin inhibitor	Tacrolimus 0.05–0.1 mg/kg/day PO BD aiming trough 4–8 ng/mL; ciclosporin 3–5 mg/kg/day	Per paediatric nephrology	Steroid-resistant FSGS, frequently relapsing minimal change, refractory membranous nephropathy; nephrotoxicity, hypertension, glucose intolerance, hirsutism (ciclosporin) or alopecia (tacrolimus)
Belimumab or voclosporin (lupus nephritis adjunct)^[1]	Anti-BAFF monoclonal antibody / calcineurin inhibitor	Belimumab 10 mg/kg IV at 0, 2, 4 weeks then monthly; voclosporin 23.7 mg PO BD	—	Add-on to MMF + steroid for lupus nephritis class III/IV/V to deepen response; contraindicated eGFR <45 (voclosporin)
Sparsentan^[1]	Dual endothelin and angiotensin receptor antagonist	200 mg PO daily, increase to 400 mg if BP and electrolytes tolerate	—	IgA nephropathy with persistent proteinuria despite RAS inhibitor; replaces ACEi/ARB (do not combine); monitor LFTs
Targeted-release budesonide (Nefecon)^[1]	Locally-acting glucocorticoid	16 mg PO once daily × 9 months	—	IgA nephropathy with persistent proteinuria UPCR ≥0.5 g/g despite optimised supportive care; reduces systemic steroid exposure

Prednisolone (induction)^[1]

Systemic corticosteroid

Adult: Minimal change: 1 mg/kg/day (max 80 mg) PO × 4–8 weeks then taper. Lupus nephritis class III/IV: methylprednisolone IV pulse 250–500 mg × 3 days then prednisolone 0.5–1 mg/kg/day (max 60 mg) tapering rapidly
Paediatric: MCD: 60 mg/m²/day (max 60 mg) × 4–6 weeks then alternate-day taper

Ulcer prophylaxis, calcium/vitamin D, glucose monitoring, infection prophylaxis (PJP) per regimen

Mycophenolate mofetil^[1]

Inosine monophosphate dehydrogenase inhibitor

Adult: Lupus nephritis induction 2–3 g/day PO in divided doses × 6 months; maintenance 1–2 g/day
Paediatric: 600 mg/m² BD (max 2 g/day)

First-line induction and maintenance for lupus nephritis class III/IV; teratogenic — strict contraception and switch ≥6 weeks before pregnancy

Cyclophosphamide^[1]

Alkylating agent

Adult: Euro-Lupus 500 mg IV every 2 weeks × 6 doses for lupus nephritis. ANCA vasculitis: 15 mg/kg IV every 2 weeks × 3 then every 3 weeks (max 1.2 g/dose, age- and renal-adjusted)
Paediatric: Per local paediatric nephrology protocol

Counsel about gonadal toxicity and bladder cancer; mesna with high-dose IV; lifetime cumulative dose monitoring; PJP prophylaxis

Rituximab^[1]

Anti-CD20 monoclonal antibody

Adult: Membranous nephropathy: 1 g IV × 2 doses 2 weeks apart, repeat at 6 months by anti-PLA2R titre. ANCA vasculitis: 375 mg/m² IV weekly × 4 induction; 1 g IV every 6 months maintenance
Paediatric: —

First-line for high-risk membranous nephropathy and ANCA vasculitis; screen and prophylax HBV; live-vaccine hold

Tacrolimus or ciclosporin^[1]

Calcineurin inhibitor

Adult: Tacrolimus 0.05–0.1 mg/kg/day PO BD aiming trough 4–8 ng/mL; ciclosporin 3–5 mg/kg/day
Paediatric: Per paediatric nephrology

Steroid-resistant FSGS, frequently relapsing minimal change, refractory membranous nephropathy; nephrotoxicity, hypertension, glucose intolerance, hirsutism (ciclosporin) or alopecia (tacrolimus)

Belimumab or voclosporin (lupus nephritis adjunct)^[1]

Anti-BAFF monoclonal antibody / calcineurin inhibitor

Adult: Belimumab 10 mg/kg IV at 0, 2, 4 weeks then monthly; voclosporin 23.7 mg PO BD
Paediatric: —

Add-on to MMF + steroid for lupus nephritis class III/IV/V to deepen response; contraindicated eGFR <45 (voclosporin)

Sparsentan^[1]

Dual endothelin and angiotensin receptor antagonist

Adult: 200 mg PO daily, increase to 400 mg if BP and electrolytes tolerate
Paediatric: —

IgA nephropathy with persistent proteinuria despite RAS inhibitor; replaces ACEi/ARB (do not combine); monitor LFTs

Targeted-release budesonide (Nefecon)^[1]

Locally-acting glucocorticoid

Adult: 16 mg PO once daily × 9 months
Paediatric: —

IgA nephropathy with persistent proteinuria UPCR ≥0.5 g/g despite optimised supportive care; reduces systemic steroid exposure

Safety-net

On immunosuppression: any new fever, productive cough, herpes zoster rash, oral thrush, or unexplained fatigue — same-day medical review (opportunistic infection)^[1]
Take rituximab and other infusion-based therapy on schedule — gaps allow B-cell return and disease relapse^[1]
Pregnancy — many disease-modifying drugs are teratogenic (mycophenolate, cyclophosphamide, sparsentan); plan ≥3–6 months ahead with your nephrologist^[1]

Referral criteria

Any newly suspected glomerular disease (proteinuria UACR ≥30 mg/mmol with or without haematuria, AKI, or hypertension)Nephrology^[1]
Rapidly progressive glomerulonephritis or pulmonary-renal syndromeNephrology and HDU^[1]
Nephrotic syndrome (UPCR ≥3.0 g/g, albumin <30 g/L, oedema)Nephrology^[1]
Refractory or relapsed disease on first-line immunosuppressionTertiary glomerular disease centre^[1]

Clinical summary

Diagnosis and immunosuppressive management of primary and secondary glomerular diseases (IgA nephropathy, FSGS, MN, MCD, lupus nephritis, ANCA vasculitis).

References

1.KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases (with KDIGO 2024 Lupus Nephritis Update) (2021)