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renal.iga-nephropathy.v1PRODUCTION
renal.iga-nephropathy.v1

IgA Nephropathy

nephrologychronicacuteadult
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Confirm IgA nephropathy via biopsy with dominant mesangial IgA deposition + persistent hematuria/proteinuria (KDIGO 2021 GN)

Inputs
3
Actions
0
Advance rule
Set
Advance when

Biopsy-confirmed or high clinical suspicion (KDIGO 2021 GN)

Patient inputs (11)

Age at presentation + ethnicity informs IIgAN risk + therapy selection (KDIGO 2021 GN; Barbour 2018)

eGFR is core risk + treatment escalation driver (KDIGO 2021 GN)

BP target <120/70 (or <130/80 per latest); ACEi/ARB max-dose first-line (KDIGO 2021 GN)

Hematuria + proteinuria + dysmorphic RBCs define presentation (KDIGO 2021 GN)

Proteinuria quantification drives therapy escalation; >0.75-1 g/d threshold for budesonide (NefIgArd Lafayette Lancet 2023)

Synpharyngitic timing is classic — 1-3 days post URI vs 2-3 weeks post-infectious GN (KDIGO 2021 GN)

IgA vasculitis (HSP) overlap — palpable purpura, arthralgia, abdominal pain (KDIGO 2021 GN)

Familial IgAN rare; informs counselling (KDIGO 2021 GN)

Elevated in ~50% of IgAN; supportive but non-diagnostic (KDIGO 2021 GN)

Normal C3/C4 expected in IgAN; low complement → reconsider lupus/MPGN/post-infectious (KDIGO 2021 GN)

IIgAN risk-prediction tool stratifies by race/ethnicity (Barbour JAMA Intern Med 2019 PMID 30980653)

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Severity triggers (12)

12 need judgement
  • informationallife_threateningrpgn_iga_crescentic
    RPGN-IgA crescentic — rapid eGFR decline + RBC casts + crescents on biopsy → route renal.rpgn.core.v1 (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverenephrotic_range_proteinuria
    Nephrotic-range proteinuria >3.5 g/d in IgAN — guarded prognosis (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereckd_progression_to_esrd
    CKD progression to ESRD — ~25-30% of IgAN at 20 years; transplant evaluation (KDIGO 2024 CKD)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveremest_c_high_activity
    High Oxford MEST-C activity on biopsy — M1/E1/S1/T1/T2/C1/C2 (Mesangial / Endocapillary / Segmental sclerosis / Tubular atrophy / Crescents) (Coppo Kidney Int 2014; KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereiigan_high_risk_calculator
    High risk per International IgA Nephropathy Risk Prediction Tool (Barbour Kidney Int 2018) — eGFR + UPCR + BP + MEST-C + race → 5-yr 50% eGFR decline/ESRD risk (Barbour 2018 PMID 30980653)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereiga_vasculitis_hsp_overlap
    IgA Vasculitis (Henoch-Schönlein purpura) — palpable purpura + arthralgia + GI involvement + IgA nephritis (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererefractory_to_budesonide
    Continued progression on budesonide — sparsentan or refractory regimens (PROTECT Lancet 2023)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_planning_on_sparsentan
    Pregnancy intent or pregnancy on sparsentan — teratogen; REMS contraception mandatory (PROTECT Lancet 2023; FDA REMS)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatepersistent_microscopic_hematuria_proteinuria
    Persistent microscopic hematuria + proteinuria — classic IgAN presentation (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderaterefractory_to_optimized_supportive
    UPCR >0.75-1 g/g after 3-6 months optimized supportive therapy — escalate to budesonide (NefIgArd Lancet 2023)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildsynpharyngitic_hematuria
    Gross hematuria 1-3 days after URI — pathognomonic IgAN presentation (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildfamilial_iga_nephropathy
    Familial IgA nephropathy — rare; counselling + family screening (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

RISK_STRATIFICATIONrequiredDrives severity classification
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Recommended regimen

IgA nephropathy stepwise therapy — optimized supportive → budesonide → sparsentan/refractory (KDIGO 2021 GN; NefIgArd Lancet 2023; PROTECT Lancet 2023)
axis: igan_disease_modifying_ladderstep step_1_optimized_supportive - Step 1 — Optimized supportive therapy (RAS max-dose + SGLT2i + BP/Na/lifestyle/CV-risk)
Selected step "Step 1 — Optimized supportive therapy (RAS max-dose + SGLT2i + BP/Na/lifestyle/CV-risk)" — All IgAN patients with proteinuria ≥0.5 g/d at any eGFR (KDIGO 2021 GN)
  • lisinopril
    first line
    acei
    10 mg PO daily, titrate to max tolerated (typically 40 mg) • PO • daily
    triggers: proteinuria_or_htn
    KDIGO 2021 GN — max-dose ACEi/ARB is foundation; titrate to BP <120/70 + minimize proteinuria
    rxcui 29046
  • losartan
    first line
    arb
    50-100 mg PO daily, titrate to max tolerated • PO • daily
    triggers: acei_intolerant
    KDIGO 2021 GN — ARB equivalent first-line if ACEi cough/angioedema
    rxcui 52175
  • empagliflozin
    add on
    sglt2i
    10 mg PO daily • PO • daily
    triggers: proteinuria, egfr_gte_20
    EMPA-KIDNEY + DAPA-CKD — SGLT2i slows progression of proteinuric CKD including IgAN subgroup; KDIGO 2024 CKD
    rxcui 1545653
  • dapagliflozin
    add on
    sglt2i
    10 mg PO daily • PO • daily
    triggers: proteinuria, egfr_gte_25, empa_unavailable
    DAPA-CKD Heerspink NEJM 2020 — alternative SGLT2i; KDIGO 2024 CKD
    rxcui 1488564

outpatient playbook — drug actions (5)

  1. 1. ACEi/ARB max-dose
    Lisinopril titrated to 40 mg or losartan 100 mg • PO • daily
    trigger: All IgAN
    KDIGO 2021 GN foundation
  2. 2. SGLT2i (empa/dapa)
    10 mg PO daily • PO • daily
    trigger: Proteinuria + eGFR >20-25
    EMPA-KIDNEY / DAPA-CKD (KDIGO 2024 CKD)
  3. 3. budesonide targeted-release
    16 mg PO daily × 9 months then taper • PO • daily
    trigger: UPCR >0.75-1 g/g on Step 1 ≥3 months
    NefIgArd Lafayette Lancet 2023 PMID 37591292
  4. 4. sparsentan (replaces ACEi/ARB)
    200-400 mg PO daily • PO • daily
    trigger: Refractory to Step 1+2 with progressive eGFR decline
    PROTECT Heerspink Lancet 2023 PMID 37015244; REMS — LFT + pregnancy monitoring
  5. 5. statin per ASCVD risk
    Moderate-intensity • PO • daily
    trigger: CV-risk modification
    KDIGO 2024 CKD + ACC/AHA Lipid 2026

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Gross hematuria 1-3 days after URI (synpharyngitic — pathognomonic) (KDIGO 2021 GN); Persistent microscopic hematuria + proteinuria (KDIGO 2021 GN); eGFR decline >50% over days-weeks (crescentic IgA → route renal.rpgn.core.v1) (KDIGO 2021 GN).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**IgA Nephropathy** (renal.iga-nephropathy.v1).
Phenotype framing: IgAN classic / IgA vasculitis (HSP) / crescentic-IgA (→ renal.rpgn) / post-infectious GN / lupus / MPGN / Alport (KDIGO 2021 GN)
Scope: Confirm IgA nephropathy via biopsy with dominant mesangial IgA deposition + persistent hematuria/proteinuria (KDIGO 2021 GN)

No severity triggers fired against current inputs.

Plan

Regimen axis: **IgA nephropathy stepwise therapy — optimized supportive → budesonide → sparsentan/refractory (KDIGO 2021 GN; NefIgArd Lancet 2023; PROTECT Lancet 2023)** — step "Step 1 — Optimized supportive therapy (RAS max-dose + SGLT2i + BP/Na/lifestyle/CV-risk)".
1. lisinopril 10 mg PO daily, titrate to max tolerated (typically 40 mg) PO daily (acei, first line) — KDIGO 2021 GN — max-dose ACEi/ARB is foundation; titrate to BP <120/70 + minimize proteinuria
2. losartan 50-100 mg PO daily, titrate to max tolerated PO daily (arb, first line) — KDIGO 2021 GN — ARB equivalent first-line if ACEi cough/angioedema
3. empagliflozin 10 mg PO daily PO daily (sglt2i, add on) — EMPA-KIDNEY + DAPA-CKD — SGLT2i slows progression of proteinuric CKD including IgAN subgroup; KDIGO 2024 CKD
4. dapagliflozin 10 mg PO daily PO daily (sglt2i, add on) — DAPA-CKD Heerspink NEJM 2020 — alternative SGLT2i; KDIGO 2024 CKD

Setting playbook (outpatient) — Primary management setting — RAS/SGLT2i optimization, IIgAN risk surveillance, budesonide/sparsentan escalation, CV/bone/fertility long-term (KDIGO 2021 GN; Barbour 2018; NefIgArd 2023)
5. ACEi/ARB max-dose Lisinopril titrated to 40 mg or losartan 100 mg PO daily — All IgAN (KDIGO 2021 GN foundation)
6. SGLT2i (empa/dapa) 10 mg PO daily PO daily — Proteinuria + eGFR >20-25 (EMPA-KIDNEY / DAPA-CKD (KDIGO 2024 CKD))
7. budesonide targeted-release 16 mg PO daily × 9 months then taper PO daily — UPCR >0.75-1 g/g on Step 1 ≥3 months (NefIgArd Lafayette Lancet 2023 PMID 37591292)
8. sparsentan (replaces ACEi/ARB) 200-400 mg PO daily PO daily — Refractory to Step 1+2 with progressive eGFR decline (PROTECT Heerspink Lancet 2023 PMID 37015244; REMS — LFT + pregnancy monitoring)
9. statin per ASCVD risk Moderate-intensity PO daily — CV-risk modification (KDIGO 2024 CKD + ACC/AHA Lipid 2026)

Non-pharmacologic actions:
- Low-Na (<2 g/day) + low-protein (0.8 g/kg/day) diet (KDIGO 2021 GN)
- Smoking cessation (KDIGO 2024 CKD)
- Annual vaccination per ACIP 2026 — flu, COVID, pneumococcal (PCV20) (KDIGO 2021 GN)
- Bone health Ca + vit D ± bisphosphonate if on systemic steroid (KDIGO 2021 GN)
- Pregnancy counselling + REMS contraception if on sparsentan (PROTECT 2023)
- Patient action card reinforced — gross hematuria with new symptoms / hemoptysis / oliguria → call MD/ED (KDIGO 2021 GN)

AVOID / contraindication checks:
- Acei arb hold if aki or hyperk (KDIGO 2021 GN)
- Sglt2i hold if euglycemic dka risk (KDIGO 2024 CKD)
- Budesonide pjp prophylaxis not routine but monitor (NefIgArd Lancet 2023)
- Sparsentan rems liver monitoring pregnancy prevention (PROTECT Lancet 2023; FDA REMS)
- Systemic steroid infection screen pre treatment (TESTING JAMA 2017)
- Rituximab vaccinate prior hbv screen (KDIGO 2021 GN)

Monitoring

Regimen monitoring:
- UPCR + UACR q3 months on therapy (KDIGO 2021 GN)
- eGFR + Cr q3 months (KDIGO 2021 GN)
- BP at each visit, target <120/70 or <130/80 (KDIGO 2021 GN)
- LFTs for sparsentan REMS (PROTECT Lancet 2023)
- Glucose + BP + bone health if systemic steroid (TESTING JAMA 2017)

Setting (outpatient) monitoring:
- UPCR + eGFR q3 months (KDIGO 2021 GN)
- BP at each visit (KDIGO 2021 GN)
- LFTs for sparsentan REMS (PROTECT 2023)
- IIgAN risk score recalculated annually (Barbour 2018)
- DEXA + bone health (steroid exposure) (KDIGO 2021 GN)

Follow-up plan: q3-6 month nephrology visits; CV-risk modification (statin per ACC/AHA Lipid 2026); transplant evaluation if approaching ESRD; vaccination + lifestyle (KDIGO 2021 GN; KDIGO 2024 CKD)
- Close-out criterion: Long-term plan documented (KDIGO 2021 GN)

Monitoring phase: UPCR + eGFR + BP at each visit; MEST-C re-biopsy considered for treatment failure; budesonide adverse-effect monitoring (KDIGO 2021 GN)

Disposition

Current setting: outpatient — Primary management setting — RAS/SGLT2i optimization, IIgAN risk surveillance, budesonide/sparsentan escalation, CV/bone/fertility long-term (KDIGO 2021 GN; Barbour 2018; NefIgArd 2023)

Disposition criteria:
- Continue outpatient nephrology q3-6 months if stable (KDIGO 2021 GN)
- Transition to transplant clinic if approaching ESRD (KDIGO 2024 CKD)

Escalation triggers (move to higher acuity):
- Rapidly rising UPCR + falling eGFR despite ladder → re-biopsy + specialty referral (KDIGO 2021 GN)
- New gross hematuria + falling eGFR → consider crescentic-IgA → route renal.rpgn.core.v1 (KDIGO 2021 GN)
- CKD progression toward ESRD → transplant evaluation → route neph.ckd.core.v1 (KDIGO 2024 CKD)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] RPGN-IgA crescentic — rapid eGFR decline + RBC casts + crescents on biopsy → route renal.rpgn.core.v1 (KDIGO 2021 GN)
- [SEVERE] Nephrotic-range proteinuria >3.5 g/d in IgAN — guarded prognosis (KDIGO 2021 GN)
- [SEVERE] CKD progression to ESRD — ~25-30% of IgAN at 20 years; transplant evaluation (KDIGO 2024 CKD)

Citations

- KDIGO 2021 Glomerular Diseases (Kidney Int Oct 2021) + NefIgArd Lafayette Lancet 2023 (budesonide Tarpeyo FDA 2023) + PROTECT Heerspink Lancet 2023 (sparsentan Filspari FDA 2023) + STOP-IgAN Rauen NEJM 2015 (negative for immunosuppression vs supportive) + TESTING Lv JAMA 2017 (corticosteroids — infection signal) + Oxford MEST-C update Trimarchi Kidney Int 2017 + International IgA Nephropathy Risk Prediction Tool (Barbour JAMA Intern Med 2019) + KDIGO 2024 CKD + EMPA-KIDNEY/DAPA-CKD SGLT2i [PMID:34556256](https://pubmed.ncbi.nlm.nih.gov/34556256/)
- Cited evidence (PMID 30980653) [PMID:30980653](https://pubmed.ncbi.nlm.nih.gov/30980653/)
- Cited evidence (PMID 37591292) [PMID:37591292](https://pubmed.ncbi.nlm.nih.gov/37591292/)
- Cited evidence (PMID 37015244) [PMID:37015244](https://pubmed.ncbi.nlm.nih.gov/37015244/)
- Cited evidence (PMID 26630142) [PMID:26630142](https://pubmed.ncbi.nlm.nih.gov/26630142/)

Last reconciled with current guidelines: 2026-05-22.
References
  • KDIGO 2021 Glomerular Diseases (Kidney Int Oct 2021) + NefIgArd Lafayette Lancet 2023 (budesonide Tarpeyo FDA 2023) + PROTECT Heerspink Lancet 2023 (sparsentan Filspari FDA 2023) + STOP-IgAN Rauen NEJM 2015 (negative for immunosuppression vs supportive) + TESTING Lv JAMA 2017 (corticosteroids — infection signal) + Oxford MEST-C update Trimarchi Kidney Int 2017 + International IgA Nephropathy Risk Prediction Tool (Barbour JAMA Intern Med 2019) + KDIGO 2024 CKD + EMPA-KIDNEY/DAPA-CKD SGLT2i — PMID:34556256
  • Cited evidence (PMID 30980653) — PMID:30980653
  • Cited evidence (PMID 37591292) — PMID:37591292
  • Cited evidence (PMID 37015244) — PMID:37015244
  • Cited evidence (PMID 26630142) — PMID:26630142