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renal.iga-nephropathy.v1

IgA Nephropathy

nephrologychronicacuteadultoutpatientinpatient
Start encounter →Print handoutPRODUCTION

Phase C expansion 2026-05-14: IgA nephropathy (Berger disease) dossier authored. Captures KDIGO 2021 GN disease-modifying ladder — Step 1 optimized supportive (RAS max-dose + SGLT2i) → Step 2 budesonide (NefIgArd Tarpeyo FDA 2023) → Step 3 sparsentan (PROTECT Filspari FDA 2023) → Step 4 systemic steroid (TESTING — caution infection signal) → Step 5 rituximab in select refractory. Reuses canonical AKI manifest pointer (no IgAN-specific manifest authored). 5 setting playbooks: home (low-risk monitoring + adherence) / ed (RPGN-IgA recognition + hypertensive flare) / icu (rare — pulmonary-renal IgA vasculitis routed to renal.rpgn.core.v1) / inpatient (RPGN-IgA crescentic biopsy + induction) / outpatient (primary chronic disease-modifying setting). Sibling routing: crescentic-IgA → renal.rpgn.core.v1; lupus nephritis differentiation → rheum.sle-flare.core.v1; CKD progression → neph.ckd.core.v1. Schema-blocked calculator queue: International IgA Nephropathy Risk Prediction Tool (Barbour 2018 PMID 30980653 — not in registry), Oxford MEST-C classification (not in registry). Surfaced as text in required_assessments + severity_triggers (mest_c_high_activity, iigan_high_risk_calculator). Owner: shard-0 clinical-tools-registry maintainer. Status INTEGRATED (not PRODUCTION) — manifest is reused from neph.aki.core.v1 (no IgAN-specific manifest); calculator registry IDs for IIgAN/MEST-C not yet present. Sparsentan REMS — LFT monitoring + pregnancy prevention required (PROTECT 2023 FDA-approved 2023); teratogen. Citation + RxCUI remediation 2026-05-22: every PMID re-verified live via PubMed — the prior evidence block was entirely fabricated/mis-attributed (34556257=autism-mouse, 31048499=ARF6-melanoma, 36368506=diuron-fish, 34800366=mito-proteome, 26465647=trichodermatide, 28267206=Didymosphenia, 27512215=spondylectomy). Replaced with verified anchors: KDIGO 2021 GN 34556256, Barbour IIgAN-tool 30980653 (JAMA Intern Med 2019), NefIgArd 37591292, PROTECT 37015244, STOP-IgAN 26630142, TESTING 28763548, Oxford MEST-C/Trimarchi 28341274. RxCUIs corrected to RxNav-verified codes: budesonide 1599538→19831, sparsentan 2638750→2630535.

Entry points (5)

  • symptom
    Gross hematuria 1-3 days after URI (synpharyngitic — pathognomonic) (KDIGO 2021 GN)
    synpharyngitic_hematuria
  • lab_abnormality
    Persistent microscopic hematuria + proteinuria (KDIGO 2021 GN)
    microscopic_hematuria_proteinuria
  • lab_abnormality
    eGFR decline >50% over days-weeks (crescentic IgA → route renal.rpgn.core.v1) (KDIGO 2021 GN)
    rapid_egfr_decline
  • imaging
    Renal biopsy with dominant IgA deposition on IF (KDIGO 2021 GN)
    biopsy_iga_dominant
  • problem_list
    IgA nephropathy on problem list (continuation visit)
    iga_nephropathy

Required inputs (11)

  • agerequired
    demographic • used at CONTEXT
    Age at presentation + ethnicity informs IIgAN risk + therapy selection (KDIGO 2021 GN; Barbour 2018)
  • race_ethnicity
    demographic • used at RISK_STRATIFICATION
    IIgAN risk-prediction tool stratifies by race/ethnicity (Barbour JAMA Intern Med 2019 PMID 30980653)
  • creatininerequired
    lab • used at CONTEXT
    eGFR is core risk + treatment escalation driver (KDIGO 2021 GN)
  • urinalysisrequired
    lab • used at INITIAL_WORKUP
    Hematuria + proteinuria + dysmorphic RBCs define presentation (KDIGO 2021 GN)
  • upcrrequired
    lab • used at INITIAL_WORKUP
    Proteinuria quantification drives therapy escalation; >0.75-1 g/d threshold for budesonide (NefIgArd Lafayette Lancet 2023)
  • sbprequired
    vital • used at CONTEXT
    BP target <120/70 (or <130/80 per latest); ACEi/ARB max-dose first-line (KDIGO 2021 GN)
  • serum_iga
    lab • used at INITIAL_WORKUP
    Elevated in ~50% of IgAN; supportive but non-diagnostic (KDIGO 2021 GN)
  • complement
    lab • used at INITIAL_WORKUP
    Normal C3/C4 expected in IgAN; low complement → reconsider lupus/MPGN/post-infectious (KDIGO 2021 GN)
  • preceding_uri
    history • used at CONTEXT
    Synpharyngitic timing is classic — 1-3 days post URI vs 2-3 weeks post-infectious GN (KDIGO 2021 GN)
  • skin_joint_gi_symptoms
    history • used at CONTEXT
    IgA vasculitis (HSP) overlap — palpable purpura, arthralgia, abdominal pain (KDIGO 2021 GN)
  • family_iga_nephropathy
    history • used at CONTEXT
    Familial IgAN rare; informs counselling (KDIGO 2021 GN)

12-phase flow (12)

  1. 1FRAME
    Confirm IgA nephropathy via biopsy with dominant mesangial IgA deposition + persistent hematuria/proteinuria (KDIGO 2021 GN)
    inputs: creatinine, urinalysis, upcr
    advance: Biopsy-confirmed or high clinical suspicion (KDIGO 2021 GN)
  2. 2ENTRY
    Recognize synpharyngitic gross hematuria, persistent micro-hematuria + proteinuria, or rapid eGFR decline (KDIGO 2021 GN)
    inputs: urinalysis
    advance: Presentation phenotype identified (KDIGO 2021 GN)
  3. 3CONTEXT
    Capture age, race/ethnicity, BP, preceding URI/skin-joint-GI symptoms, family history (KDIGO 2021 GN; Barbour 2018)
    inputs: age, sbp, creatinine, preceding_uri, skin_joint_gi_symptoms, family_iga_nephropathy
    advance: Context captured (KDIGO 2021 GN)
  4. 4RED_FLAGS
    Rapid eGFR decline / RPGN-IgA crescentic / nephrotic-range proteinuria / IgA vasculitis with pulmonary-renal (KDIGO 2021 GN)
    advance: No emergent phenotype OR routed to renal.rpgn.core.v1 (KDIGO 2021 GN)
  5. 5INITIAL_WORKUP
    UA with microscopy, UPCR/UACR, eGFR, serum IgA, complement, hepatitis serologies, ANA + ANCA to exclude mimics (KDIGO 2021 GN)
    inputs: urinalysis, upcr, creatinine, serum_iga, complement
    actions: panel.renal
    advance: Lab profile complete + mimics excluded (KDIGO 2021 GN)
  6. 6BRANCHING_WORKUP
    Renal biopsy with Oxford MEST-C scoring if active disease + proteinuria persisting on RAS blockade (KDIGO 2021 GN; Coppo Kidney Int 2014)
    advance: Biopsy + MEST-C documented OR clinical decision to defer (KDIGO 2021 GN)
  7. 7DIFFERENTIAL
    IgAN classic / IgA vasculitis (HSP) / crescentic-IgA (→ renal.rpgn) / post-infectious GN / lupus / MPGN / Alport (KDIGO 2021 GN)
    advance: Differential resolved (KDIGO 2021 GN)
  8. 8RISK_STRATIFICATION
    International IgA Nephropathy Risk Prediction Tool (eGFR + UPCR + BP + MEST-C + race/ethnicity → 5-yr 50% eGFR-decline/ESRD risk) (Barbour JAMA Intern Med 2019 PMID 30980653)
    inputs: creatinine, upcr, sbp, age, race_ethnicity
    actions: calc.ckd_epi_2021
    advance: Risk tier documented (KDIGO 2021 GN)
  9. 9TREATMENT
    Step 1 — optimized supportive (max-dose ACEi/ARB + SGLT2i + BP/Na/lifestyle); Step 2 — budesonide 16 mg/d if proteinuria >0.75-1 g/d persists (NefIgArd Lafayette Lancet 2023); Step 3 — sparsentan / steroids / rituximab refractory (PROTECT 2023; TESTING Lv 2017) (KDIGO 2021 GN)
    inputs: upcr, creatinine, sbp
    advance: Therapy tier executed + tolerability documented (KDIGO 2021 GN)
  10. 10DISPOSITION
    Outpatient nephrology primary; admit for RPGN-IgA crescentic / nephrotic flare / acute kidney injury / pulmonary-renal (KDIGO 2021 GN)
    advance: Disposition set (KDIGO 2021 GN)
  11. 11MONITORING
    UPCR + eGFR + BP at each visit; MEST-C re-biopsy considered for treatment failure; budesonide adverse-effect monitoring (KDIGO 2021 GN)
    inputs: creatinine, upcr, sbp
    actions: panel.renal
    advance: Monitoring schedule documented (KDIGO 2021 GN)
  12. 12FOLLOWUP
    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)
    advance: Long-term plan documented (KDIGO 2021 GN)