📋 Key Information Summary
- IgA nephropathy (Berger's disease) is the most common primary glomerulonephritis worldwide and the leading cause of GN-related end-stage kidney disease (ESKD) in young adults aged 20–40 years in Australia.
- Pathogenesis centres on galactose-deficient IgA1 (Gd-IgA1): aberrantly glycosylated IgA1 forms immune complexes that deposit in the mesangium, triggering complement activation and progressive nephron loss.
- Classic presentation: synpharyngitic haematuria — visible (macroscopic) haematuria within 1–2 days of an upper respiratory tract infection, with or without proteinuria.
- Diagnosis requires renal biopsy: mesangial IgA (± C3) deposits on immunofluorescence with mesangial hypercellularity on light microscopy (Oxford/MEST-C classification).
- Risk stratify using the International IgA Nephropathy Prediction Tool (IIgAN-PT) to guide intensity of therapy.
- First-line therapy for all patients: maximum-tolerated RAAS blockade — ACE inhibitor (ACEi) or ARB titrated to reduce proteinuria <0.5–1 g/day and systolic BP <125 mmHg.
- SGLT2 inhibitors (dapagliflozin or empagliflozin) are now recommended as add-on therapy for proteinuria ≥0.5 g/day with eGFR ≥20 mL/min/1.73 m², independent of diabetes status.
- Targeted-release budesonide (Nefecon® / TARPEYO®) is PBS-listed (Authority Required) for IgAN with persistent proteinuria ≥1 g/day despite optimised RAAS blockade, reducing Gd-IgA1 via gut mucosal immunomodulation.
- Immunosuppression (cyclophosphamide, mycophenolate, corticosteroids) is reserved for rapidly progressive or high-risk disease; do NOT use as first-line in all patients.
- Tonkin et al. STOP-IgA and TESTING trials demonstrated systemic corticosteroids reduce proteinuria but carry significant infection risk — use with caution.
- ATSI populations have higher rates of ESKD and often present later; culturally safe engagement and early nephrology referral are essential.
- Monitor proteinuria (uACR), eGFR, BP, and pregnancy status every 3–6 months; refer to nephrology if eGFR declining >5 mL/min/year or proteinuria >1 g/day despite therapy.
- Novel targeted therapies including iptacopan (complement factor B inhibitor) and narsoplimab (MASP-2 inhibitor) are in late-phase trials and may alter the treatment paradigm.
Introduction & Australian Epidemiology
IgA nephropathy (IgAN), also known as Berger's disease, is the most common primary glomerulonephritis (GN) worldwide and a leading cause of kidney failure in young adults. It is characterised by the deposition of galactose-deficient IgA1 (Gd-IgA1) immune complexes in the glomerular mesangium, triggering mesangial proliferation, complement activation, and progressive nephron loss.
In Australia, IgAN accounts for approximately 10–20% of all primary GN diagnoses on renal biopsy, with the highest incidence in individuals aged 20–40 years. There is a 2:1 male predominance. Aboriginal and Torres Strait Islander peoples experience disproportionately higher rates of glomerular disease overall, though IgAN remains less common than in non-Indigenous populations — lupus nephritis and glomerulosclerosis are more prevalent in ATSI communities.
Disease course is highly variable: up to 40% of patients will progress to ESKD within 20 years of diagnosis if untreated. Early identification, risk stratification, and timely initiation of renin–angiotensin system (RAS) blockade are the cornerstones of management. Recent advances — including SGLT2 inhibitors and targeted-release budesonide — have expanded the therapeutic armamentarium and shifted the paradigm away from empirical immunosuppression.
This guideline synthesises current evidence from the TESTING trial, STOP-IgA, NefIgArd, and KDIGO 2024 update, contextualised for Australian practice including PBS availability, MBS billing, and ATSI health considerations.
Pathogenesis — Galactose-Deficient IgA1
The pathogenesis of IgAN is best understood as a multi-hit process:
- Hit 1 — Overproduction of Gd-IgA1: Peyer's patches and mucosa-associated lymphoid tissue (MALT) in the gut produce abnormally glycosylated IgA1 with deficient galactose residues on the hinge-region O-linked glycans. This is driven by mucosal immune dysregulation, often triggered by respiratory or gastrointestinal infections.
- Hit 2 — Anti-glycan antibody production: The host mounts IgG or IgA autoantibodies against the exposed N-acetylgalactosamine (GalNAc) epitopes on Gd-IgA1, forming circulating immune complexes (CICs).
- Hit 3 — Mesangial deposition: CICs deposit in the glomerular mesangium via interactions with fibronectin and integrin receptors, activating mesangial cell proliferation and extracellular matrix expansion.
- Hit 4 — Complement activation and injury: Mesangial deposits activate the lectin pathway (via MBL/MASP-2), alternative pathway (via factor B), and classical pathway, generating C5a and the membrane attack complex (MAC), driving tubulointerstitial fibrosis and progressive nephron loss.
Clinical significance: The understanding of gut–kidney axis pathogenesis directly informs the rationale for targeted-release budesonide (Nefecon®), which acts on gut-associated lymphoid tissue to reduce Gd-IgA1 production at its source.
Key Pathological Findings (Oxford MEST-C Classification)
| Score | Parameter | Definition | Prognostic Significance |
|---|---|---|---|
| M0/M1 | Mesangial hypercellularity | <4 vs ≥4 mesangial cells per mesangial area | M1 predicts faster progression |
| E0/E1 | Endocapillary hypercellularity | Absent vs present | E1 associated with active inflammation; may respond to immunosuppression |
| S0/S1 | Segmental sclerosis | Absent vs present | S1 predicts worse renal outcome |
| T0/T1/T2 | Tubular atrophy / interstitial fibrosis | <25% / 25–50% / >50% | T1 and T2 are the strongest histological predictors of ESKD |
| C0/C1/C2 | Crescents (cellular/fibrocellular) | None / <50% / ≥50% of glomeruli | C2 indicates aggressive disease; consider immunosuppression |
Clinical Features & Diagnostic Criteria
Classic Presentation: Synpharyngitic Haematuria
The hallmark presentation of IgAN is synpharyngitic (or "synpharyngitic") haematuria — visible haematuria appearing within 24–48 hours of an upper respiratory tract infection (URTI), pharyngitis, or less commonly, gastroenteritis. This rapid onset distinguishes it from post-streptococcal GN, where haematuria appears 1–3 weeks after infection.
Episodes typically last 2–5 days and may be accompanied by:
- Dark brown or "cola-coloured" urine
- Mild loin or flank pain (bilateral)
- Low-grade fever during the precipitating infection
- Transient oedema (rare unless nephrotic-range proteinuria)
Other Presentations
| Presentation | Frequency | Features |
|---|---|---|
| Synpharyngitic haematuria | 40–50% (children up to 80%) | Macroscopic haematuria 1–2 days post-URTI |
| Asymptomatic haematuria / proteinuria | 30–40% | Incidental finding on urinalysis or health screen |
| Nephrotic syndrome | 5% | Oedema, heavy proteinuria >3.5 g/day; consider concurrent minimal change disease |
| Rapidly progressive GN (RPGN) | <5% | Rapid rise in creatinine over weeks, crescentic histology (MEST-C C2); urgent nephrology |
| Malignant hypertension | <5% | BP >180/120, papilloedema, AKI; emergency management required |
Red flags requiring urgent nephrology referral: Rapidly rising creatinine (>50% rise over 2–4 weeks), nephrotic syndrome, crescentic GN on biopsy (MEST-C C2), or malignant hypertension. These may represent rapidly progressive IgAN and require aggressive immunosuppression.
When to Suspect IgAN
- Recurrent macroscopic haematuria associated with URTI in a young adult (20–40 years)
- Persistent microscopic haematuria with dysmorphic red cells or red cell casts on urine microscopy
- Isolated proteinuria 0.2–3.5 g/day with normal renal function in a young patient
- Haematuria with concurrent low C3 (suggesting complement activation) but normal C4
Investigations & Renal Biopsy
Baseline Investigations
Essential
Urinalysis + urine microscopy
Dysmorphic RBCs, RBC casts, proteinuria (uACR). MBS item 71055 (urine microscopy).
Essential
Serum creatinine, eGFR (CKD-EPI 2021)
Establish baseline renal function. MBS item 66500.
Essential
24-hour urine protein or spot uPCR/uACR
Quantify proteinuria. uACR preferred (MBS item 66501).
Available
Serum IgA level
Elevated in ~50% of IgAN patients; not diagnostic but supportive. MBS item 66822.
Available
Serum complement (C3, C4)
Low C3 with normal C4 is common in IgAN due to lectin/alternative pathway activation.
Available
Hepatitis B & C serology, HIV
Exclude secondary causes of mesangial IgA deposition.
Referral
Gd-IgA1 / Gd-IgA1-IgG immune complexes
Research/specialised assay; not routinely available in Australia. May assist in ambiguous cases.
Renal Biopsy — Gold Standard
Definitive diagnosis of IgAN requires a percutaneous renal biopsy with immunofluorescence (IF) and electron microscopy (EM).
Biopsy indication: Persistent proteinuria ≥0.5 g/day despite 3–6 months of optimised RAAS blockade, unexplained AKI, recurrent macroscopic haematuria with declining eGFR, or nephrotic syndrome. Refer to nephrology for biopsy decision.
Histopathological Criteria for IgAN Diagnosis
| Modality | Finding | Comment |
|---|---|---|
| Light microscopy | Mesangial hypercellularity, matrix expansion | May show focal/diffuse proliferative pattern; classify by MEST-C |
| Immunofluorescence (IF) | IgA dominant mesangial deposits (≥2+ on 0–3 scale) | Diagnostic criterion; C3 co-deposits in >90%; IgG or IgM may co-deposit |
| Electron microscopy (EM) | Electron-dense mesangial deposits | Confirms IF findings; subendothelial or subepithelial deposits suggest other diagnoses (e.g., lupus nephritis, IgA-dominant infection-related GN) |
Differential diagnosis: IgA-dominant infection-related GN (staphylococcal-associated, especially in diabetic patients), lupus nephritis (full-house IF pattern: IgG + IgA + IgM + C3 + C1q), and IgA vasculitis (Henoch–Schönlein purpura — similar histology with systemic vasculitis features). Always correlate histology with clinical context.
Risk Stratification & Prognosis
The International IgA Nephropathy Prediction Tool (IIgAN-PT), developed by the IgA Nephropathy Network (NEPTUNE/CureGN), is the preferred validated risk calculator. It integrates clinical and histological variables at the time of biopsy to estimate 5-year and 7-year risk of a 50% decline in eGFR or ESKD.
Clinical Risk Factors for Progression
- Persistent proteinuria >1 g/day despite RAAS blockade
- Hypertension (BP >140/90 or requiring antihypertensives)
- Reduced eGFR (<60 mL/min/1.73 m²) at presentation
- Histological MEST-C scores: M1, E1, S1, T1/T2, C1/C2
- Older age at diagnosis
- Male sex
- Obesity (BMI >30 kg/m²) and metabolic syndrome
Low Risk
Indolent Disease
Proteinuria <0.5 g/day, normal eGFR, MEST-C M0E0S0T0C0. 10-year ESKD risk <5%.
Setting: GP-led with nephrology oversight; annual review
Moderate Risk
Active Disease
Proteinuria 0.5–1 g/day, eGFR 45–89, MEST-C M1 or S1. 10-year ESKD risk 15–30%.
Setting: Nephrology co-management; consider SGLT2i + budesonide
High Risk
Aggressive Disease
Proteinuria >1 g/day, eGFR <45, MEST-C T1/T2 or C1/C2. 10-year ESKD risk >50%.
Setting: Specialist nephrology; consider immunosuppression; transplant evaluation
Management
Management of IgAN is stratified by proteinuria level, eGFR, and histological risk. The current therapeutic approach follows a layered strategy: supportive care first, then targeted therapies for higher-risk disease.
Layer 1 — Supportive / Foundation Therapy (All Patients)
RAAS Blockade — ACE Inhibitors or ARBs
Maximum-tolerated RAAS blockade is the cornerstone of IgAN management. ACEi or ARB should be titrated to achieve:
- Proteinuria <0.5–1 g/day (target <0.5 g/day ideal)
- Systolic BP <125 mmHg (or <130 mmHg if tolerated poorly)
- Avoid combining ACEi + ARB (ONTARGET trial — no benefit, increased harm)
Ramipril
Ramace®, Tritace® · ACE inhibitor
Adult dose
Start 2.5 mg PO daily; titrate to 5–10 mg PO daily
Paediatric dose
0.1 mg/kg/day (max 5 mg/day); not recommended <6 months
Renal adjustment
eGFR <30: start 1.25 mg daily; titrate cautiously
PBS status
✔ PBS General Benefit
Irbesartan
Avapro®, Karvea® · ARB
Adult dose
Start 150 mg PO daily; titrate to 300 mg PO daily
Paediatric dose
Not established in children <13 years for IgAN
Renal adjustment
No dose change required; monitor K⁺ and creatinine
PBS status
✔ PBS General Benefit
Layer 2 — SGLT2 Inhibitors
Based on the DAPA-CKD and EMPA-KIDNEY trials, SGLT2 inhibitors reduce proteinuria and slow eGFR decline in CKD including IgAN. They are recommended as add-on therapy for all IgAN patients with:
- Proteinuria ≥0.5 g/day
- eGFR ≥20 mL/min/1.73 m²
- On maximised RAAS blockade
- Independent of diabetes status
Dapagliflozin
Forxiga® · SGLT2 inhibitor
Adult dose
10 mg PO once daily
Renal adjustment
Initiate if eGFR ≥20; if already on, continue down to eGFR ~15
Key counselling
Hold during acute illness ("sick day rules"); adequate hydration; genital hygiene to prevent mycotic infections
PBS status
✔ PBS General Benefit
Empagliflozin
Jardiance® · SGLT2 inhibitor
Adult dose
10 mg PO once daily
Renal adjustment
Initiate if eGFR ≥20; continue if previously tolerated at lower eGFR
PBS status
✔ PBS General Benefit
Layer 3 — Targeted-Release Budesonide (Nefecon®)
Nefecon® (TARPEYO®) is a novel targeted-release budesonide formulation (16 mg/day) that delivers corticosteroids to the distal ileum — the primary site of Gd-IgA1-producing Peyer's patches — with minimal systemic absorption (~10% oral bioavailability).
NefIgArd trial evidence: The phase 3 NefIgArd trial demonstrated that Nefecon® 16 mg/day for 9 months (followed by 3 months washout) significantly reduced proteinuria (26% relative reduction vs placebo) and stabilised eGFR compared with supportive care alone. The benefit was sustained at 2-year follow-up.
Nefecon (Targeted-Release Budesonide)
Nefecon® · Delayed-release ileal budesonide
Adult dose
16 mg (4 × 4 mg capsules) PO once daily in the morning, 1 hour before breakfast
Duration
9 months total treatment course
Indication
IgAN with persistent proteinuria ≥1 g/day despite ≥3 months optimised RAAS blockade
Renal adjustment
No dose adjustment required; use with caution if eGFR <30
Hepatic adjustment
Avoid in severe hepatic impairment (Child-Pugh C)
Key side effects
Peripheral oedema, HTN, acne, headache — lower systemic steroid effects than oral prednisolone
PBS status
⚠ PBS Authority Required
PBS Authority criteria for Nefecon®: Patient must have biopsy-proven IgA nephropathy AND proteinuria ≥1 g/day (uPCR ≥100 mg/mmol) despite ≥3 months of maximum-tolerated ACEi or ARB therapy. Authority is granted for a single 9-month treatment course.
Layer 4 — Immunosuppression (Specialist-Only)
Systemic immunosuppression is reserved for high-risk or rapidly progressive disease. It should be initiated only by a nephrologist after optimising supportive therapy (Layers 1–3).
Systemic Corticosteroids
The TESTING trial (2022 update) showed that a modified-dose methylprednisolone/prednisolone regimen reduced the risk of 40% eGFR decline or ESKD by 47%, but was associated with serious infections. The reduced-dose regimen (0.4 mg/kg/day prednisolone, max 32 mg/day, tapered over 6–8 months) has a more favourable safety profile.
Prednisolone
Solone®, Panafcortelone® · Systemic corticosteroid
Adult dose (TESTING modified)
0.4 mg/kg/day PO (max 32 mg/day) for 2 months, then taper by 4 mg/month over 6–8 months
Co-prescribe
Trimethoprim 300 mg PO daily (PJP prophylaxis) + PPI for GI protection + calcium/vitamin D
PBS status
✔ PBS General Benefit
STOP-IgA & TESTING lessons: Do NOT use systemic immunosuppression empirically in all IgAN patients. The risk of serious infections (including sepsis and PJP) outweighs benefit in low-risk or moderate-risk disease managed with RAAS blockade + SGLT2i + budesonide. Reserve immunosuppression for patients with >1 g/day proteinuria despite all supportive measures, or crescentic (C2) disease.
Other Immunosuppressive Options (Specialist Use)
| Agent | Indication | Dose | PBS Status |
|---|---|---|---|
| Mycophenolate mofetil (CellCept®) | Consider in Asian populations with M1E1 disease (variable evidence); not first-line in Caucasians | 1 g PO BD for 12–24 months | PBS General Benefit (for transplant, not IgAN PBS-listed indication) |
| Cyclophosphamide | Crescentic/RPGN IgAN (MEST-C C2); rapidly progressive disease | 2 mg/kg/day PO for 2–3 months (or IV pulse 500–750 mg/m² monthly × 6) | PBS General Benefit |
| Azathioprine | Maintenance after cyclophosphamide (if steroid-sparing needed) | 2 mg/kg/day PO; check TPMT prior | PBS General Benefit |
| Rituximab | Case series data only; NOT recommended routinely for IgAN | 1000 mg IV × 2 doses (day 1 and day 15) | class="guideline-td">PBS Authority Required (for specific indications only)
Novel & Emerging Therapies (2024–2025)
The therapeutic landscape for IgAN is evolving rapidly. The following agents are in advanced clinical trials and may change practice in the near future:
- Iptacopan (complement factor B inhibitor): APPLAUSE-IgAN phase 3 trial demonstrated significant proteinuria reduction. May target the alternative complement pathway implicated in IgAN progression.
- Narsoplimab (anti-MASP-2 monoclonal antibody): Blocks lectin pathway activation. Phase 2/3 data in IgAN; TGA not yet approved.
- Sibeprenlimab (anti-APRIL antibody): Reduces IgA and Gd-IgA1 production. Phase 2 trial (ENVISION) showed significant proteinuria reduction.
- Atacicept (TACI-Ig fusion protein): Inhibits BAFF and APRIL, reducing IgA-producing B cells. Phase 2 results promising.
- Zigakibart (anti-APRIL antibody): Another APRIL-targeting agent with phase 3 trial underway.
These agents are not yet PBS-listed or TGA-approved for IgAN as of 2025. Refer to clinicaltrials.gov for trial enrolment eligibility.
Monitoring & Follow-Up
Monitoring Schedule
Every 1–3 months
uACR or uPCR, serum creatinine, eGFR, BP, K⁺ — during active titration of RAAS blockade or on immunosuppression
Every 3–6 months
Stable disease: uACR, eGFR, BP. Assess for treatment side effects (SGLT2i — volume depletion, UTI; budesonide — oedema, HTN)
Annually
FBC, LFTs, lipids, HbA1c (especially if on corticosteroids), bone density if prolonged steroids, retinal screening if diabetic
Re-biopsy consideration
If unexplained rapid eGFR decline, new nephrotic syndrome, or to assess treatment response — specialist decision
When to Refer to Nephrology
- All newly diagnosed IgAN patients — initial nephrology assessment recommended
- Proteinuria >0.5 g/day despite 3–6 months of optimised RAAS blockade
- eGFR declining >5 mL/min/year
- eGFR <30 mL/min/1.73 m² (pre-dialysis planning)
- Nephrotic syndrome or rapidly progressive GN
- Pregnancy planning (ACEi/ARB cessation required)
- Consideration for immunosuppression or clinical trial enrolment
Special Populations
Pregnancy
ACEi / ARB
Contraindicated in pregnancy — teratogenic (renal agenesis, oligohydramnios). Cease pre-conception or immediately upon pregnancy confirmation. Switch to labetalol, nifedipine, or methyldopa for BP control.
SGLT2 inhibitors
Contraindicated in pregnancy — potential foetal renal toxicity. Cease pre-conception.
Nefecon® (budesonide)
Avoid in pregnancy — corticosteroid effects. Limited data; discontinue if pregnancy occurs.
Mycophenolate mofetil
Absolute contraindication — FDA Category X. Teratogenic. Must cease ≥6 weeks pre-conception and use reliable contraception during therapy.
Preferred BP agents in pregnancy
Labetalol (100–400 mg PO BD–TDS, PBS General), nifedipine SR (30–90 mg daily, PBS General), methyldopa (250–500 mg PO BD–TDS, PBS General).
Paediatrics
Presentation
Up to 80% of paediatric IgAN presents with synpharyngitic haematuria. Prognosis is generally more favourable than adult-onset disease.
RAAS blockade
Enalapril 0.1 mg/kg/day titrated to 0.5 mg/kg/day (max 40 mg/day) or losartan 0.7 mg/kg/day (max 50 mg/day). Both PBS General Benefit.
SGLT2 inhibitors
Not TGA-approved in children <18 years for CKD. Off-label use requires specialist decision.
Nefecon®
Not studied in paediatric IgAN. Off-label use not recommended outside clinical trial.
Advanced CKD (eGFR <30)
RAAS blockade
Continue if tolerated; monitor K⁺ closely (risk of hyperkalaemia). May need sodium zirconium cyclosilicate (Lokelma®) for K⁺ management.
SGLT2 inhibitors
Initiate only if eGFR ≥20 at start. Continue down to eGFR ~15 if already on treatment (KDIGO 2024).
Immunosuppression
Reduced benefit when eGFR <30 and established fibrosis (T2). Specialist discussion re: risks vs benefits. Consider transplant waitlist.
Immunocompromised
Corticosteroids
Increased infection risk. Mandatory PJP prophylaxis (co-trimoxazole 300 mg daily). Screen for latent TB (IGRA) before starting.
Cyclophosphamide
Significant immunosuppression. Avoid in active infection. Monitor FBC fortnightly for neutropaenia.
Hepatic Impairment
Nefecon® (budesonide)
Metabolised by CYP3A4. Avoid in severe hepatic impairment (Child-Pugh C). Caution in moderate impairment.
Mycophenolate
No dose adjustment but monitor LFTs; hepatotoxicity reported.
Elderly (>65 years)
General considerations
Higher risk of AKI with RAAS blockade; start low and titrate slowly. SGLT2i-associated volume depletion is more common — assess postural BP. Immunosuppression risks (infection, osteoporosis, diabetes) are amplified — use modified-dose regimens if indicated.
Aboriginal and Torres Strait Islander Health Considerations
Aboriginal and Torres Strait Islander Health
Aboriginal and Torres Strait Islander peoples experience a disproportionate burden of kidney disease in Australia. While IgA nephropathy is less common in ATSI populations than non-Indigenous Australians, the overall rate of ESKD is 8–10 times higher in Indigenous communities (AIHW 2023). Glomerulonephritis in ATSI peoples is more often due to lupus nephritis, post-infectious GN, and glomerulosclerosis associated with obesity and diabetes.
When IgAN does occur in ATSI patients, late presentation, reduced access to specialist nephrology services, and comorbid conditions (diabetes, cardiovascular disease, obesity) may worsen outcomes. The following considerations are essential:
AIHW & RHDAustralia guidance: All ATSI patients with persistent haematuria or proteinuria should be referred for nephrology assessment within 3 months. Annual health checks (MBS Item 715) must include uACR and eGFR. Kidney Health Australia's "Living Well with Kidney Disease" resources are available in Aboriginal languages via kidney.org.au.
Quick Reference — Treatment Summary
All IgAN patients
ACEi or ARB (max tolerated dose)
Lifelong
Target proteinuria <0.5 g/day, SBP <125 mmHg
Proteinuria ≥0.5 g/day, eGFR ≥20
+ Dapagliflozin 10 mg daily or empagliflozin 10 mg daily
Lifelong (or until dialysis/transplant)
Independent of diabetes status; hold during acute illness
Proteinuria ≥1 g/day despite RAAS blockade (≥3 months)
+ Nefecon® 16 mg daily
9 months
PBS Authority Required; biopsy-proven IgAN; specialist initiation
High-risk / crescentic (C2) / RPGN
Prednisolone 0.4 mg/kg/day (max 32 mg) taper 6–8 months ± cyclophosphamide
6–8 months (corticosteroids); 2–3 months (cyclophosphamide)
Specialist only; PJP prophylaxis mandatory; monitor for infection
📚 References
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- 11. RHDAustralia (a program of NACCHO). Recommendations for the diagnosis and management of chronic kidney disease in Aboriginal and Torres Strait Islander peoples. Darwin: RHDAustralia; 2023.
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