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renal.lupus-nephritis.v1

Lupus Nephritis (ISN/RPS Classes I-VI)

nephrologychronicacuteadultoutpatientinpatient
Start encounter →Print handoutPRODUCTION

Phase C wave-8 expansion 2026-05-15: Lupus Nephritis (ISN/RPS Classes I-VI) dossier authored from renal-domain perspective. KDIGO 2024 GN + EULAR/ACR 2019 SLE updates are the binding spine. Companion engines rheum.sle.core.v1 (chronic SLE) and rheum.sle-flare.core.v1 (extra-renal flare) co-manage non-renal manifestations. Class-stratified induction + maintenance ladder — universal HCQ → Class III/IV induction MMF (ALMS Appel JASN 2009 PMID 19369404) or low-dose IV CY (Euro-Lupus regimen) + pulse MP → adjunct belimumab (BLISS-LN Furie NEJM 2020 PMID 32937045; FDA 2020) → adjunct voclosporin (AURORA-1 Rovin Lancet 2021 PMID 33971155; FDA 2021) → Class V membranous MMF + steroids + CNI add-on → Class VI advanced sclerotic RRT/transplant planning → maintenance MMF or AZA + low-dose steroid → renoprotective ACEi/ARB + SGLT2i emerging adjunct. 5 setting playbooks: home (adherence + sick-day + flare recognition) / outpatient (primary chronic + induction → maintenance) / ed (flare + biopsy planning + hypertensive urgency) / icu (pulmonary-renal + TMA-on-LN + severe sepsis) / inpatient (induction protocol + biopsy + infection prophylaxis). 12 severity triggers covering ISN/RPS Class I-VI + mixed III+V/IV+V + nephrotic-range + refractory + pregnancy + TMA-on-LN + pulmonary-renal. Sibling routing: rheum.sle.core.v1 (chronic SLE multi-organ baseline), rheum.sle-flare.core.v1 (extra-renal flare), renal.rpgn.core.v1 (Class IV with crescents may overlap), renal.iga-nephropathy.v1 (immune-complex GN differentiation). Schema-blocked calculator queue: ISN/RPS classification + activity/chronicity index + SLEDAI + SLICC damage index — none registered in clinical-tools-registry. Surfaced as text. Owner: shard-0 clinical-tools-registry maintainer. Status INTEGRATED (not PRODUCTION) — manifest stub authored; engine runtime class not yet present; calculator registry IDs (ISN/RPS, SLEDAI, etc.) not registered. Modern adjuncts encoded: belimumab (BLISS-LN PMID 32937045; FDA 2020) + voclosporin (AURORA-1 PMID 33971155; FDA 2021) + tacrolimus for Class V membranous + emerging SGLT2i renoprotection. Pregnancy planning: MMF/CY teratogenic; switch to AZA + HCQ + low-dose steroid 3-6 months pre-conception; close MFM + rheumatology + nephrology comanagement (EULAR/ACR 2019 Aringer Arthritis Rheumatol 2019 PMID 31385462). 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, 35219617=SAPIEN-valve, 32726528=nirsevimab-RSV, 33812489=Psoriasis-Lancet, 21622708=arsenic-bacterium, 28114205=nasal-valve-suturing). Replaced with verified anchors: KDIGO 2021 GN 34556256, EULAR/ACR 2019 SLE 31385462, BLISS-LN 32937045, AURORA-1 33971155, ALMS 19369404. Unverifiable Euro-Lupus/Houssiau PMID removed (regimen text retained). RxCUIs corrected to RxNav-verified codes: mycophenolate_mofetil 6932→68149, belimumab 1442132→1092437, voclosporin 2546843→2475166, tacrolimus 10318→42316.

Entry points (5)

  • lab_abnormality
    New proteinuria (UPCR >0.5 g/g) in SLE patient (KDIGO 2024 GN; EULAR/ACR 2019)
    new_proteinuria_in_sle
  • lab_abnormality
    Active urinary sediment (RBC casts, dysmorphic RBCs) in SLE (KDIGO 2024 GN)
    active_urine_sediment_sle
  • lab_abnormality
    AKI with low C3/C4 + positive ANA/anti-dsDNA → class III/IV lupus nephritis suspected (KDIGO 2024 GN)
    aki_with_low_complement
  • imaging
    Renal biopsy with ISN/RPS classification I-VI (KDIGO 2024 GN; ISN/RPS 2003)
    biopsy_lupus_nephritis
  • problem_list
    SLE on problem list (continuation visit with renal involvement)
    sle_known

Required inputs (10)

  • agerequired
    demographic • used at CONTEXT
    Age informs induction agent selection (CY fertility risk in young women; voclosporin/belimumab approved adults) (KDIGO 2024 GN; EULAR/ACR 2019)
  • pregnancy_statusrequired
    demographic • used at CONTEXT
    Pregnancy + LN special considerations — MMF/CY teratogenic; azathioprine + HCQ + steroids preferred (EULAR/ACR 2019)
  • creatininerequired
    lab • used at CONTEXT
    eGFR + AKI vs CKD trajectory drives urgency + agent dosing (KDIGO 2024 GN)
  • urinalysisrequired
    lab • used at INITIAL_WORKUP
    Active sediment + proteinuria define renal involvement (KDIGO 2024 GN)
  • upcrrequired
    lab • used at INITIAL_WORKUP
    Proteinuria quantification drives class III/IV/V escalation; nephrotic-range >3500 mg/d (KDIGO 2024 GN)
  • complementrequired
    lab • used at INITIAL_WORKUP
    Low C3/C4 marks active LN flare; tracks induction response (KDIGO 2024 GN; EULAR/ACR 2019)
  • anti_dsdnarequired
    lab • used at INITIAL_WORKUP
    Active disease marker; rises in LN flare; tracks induction response (EULAR/ACR 2019)
  • anarequired
    lab • used at INITIAL_WORKUP
    Diagnostic anchor for SLE per EULAR/ACR 2019 classification (EULAR/ACR 2019 PMID 31385462)
  • sbprequired
    vital • used at CONTEXT
    BP target <130/80 (or <120/70 if high CV risk); ACEi/ARB renoprotection (KDIGO 2024 GN)
  • sle_diagnosisrequired
    history • used at FRAME
    EULAR/ACR 2019 SLE criteria threshold — ANA ≥1:80 plus weighted features ≥10 (EULAR/ACR 2019)

12-phase flow (12)

  1. 1FRAME
    Confirm SLE per EULAR/ACR 2019 + renal involvement; biopsy ISN/RPS class drives therapy (KDIGO 2024 GN; ISN/RPS 2003)
    inputs: sle_diagnosis, creatinine, upcr
    advance: SLE confirmed + renal involvement documented (KDIGO 2024 GN)
  2. 2ENTRY
    New proteinuria / active sediment / AKI with low C3 / known SLE flare (KDIGO 2024 GN; EULAR/ACR 2019)
    advance: Renal involvement triggers engine (KDIGO 2024 GN)
  3. 3CONTEXT
    Capture age, sex, ethnicity (LN more severe in Black + Hispanic + Asian), pregnancy status, extra-renal flare features, prior induction agents, HCQ adherence (KDIGO 2024 GN; EULAR/ACR 2019)
    inputs: age, pregnancy_status, sle_diagnosis, sbp
    advance: Context complete (KDIGO 2024 GN)
  4. 4RED_FLAGS
    Acute kidney injury with active sediment / nephrotic flare / alveolar hemorrhage (pulmonary-renal syndrome) / cellular casts / TMA-on-LN overlap (KDIGO 2024 GN; EULAR/ACR 2019)
    advance: Acuity tier set + emergent phenotype routed (KDIGO 2024 GN)
  5. 5INITIAL_WORKUP
    UA with micro, UPCR/UACR, eGFR, ANA, anti-dsDNA, anti-Sm, anti-Ro/La, anti-RNP, complement, CBC, BMP, LFTs, lipids, fasting glucose, infection screen pre-immunosuppression (HBV, HCV, HIV, TB, strongyloides) (KDIGO 2024 GN; EULAR/ACR 2019)
    inputs: urinalysis, upcr, creatinine, complement, anti_dsdna, ana
    actions: panel.renal, panel.cbc, panel.lft, panel.inflammation
    advance: Initial labs complete + biopsy planned if UPCR >0.5 g/g + active sediment (KDIGO 2024 GN)
  6. 6BRANCHING_WORKUP
    Renal biopsy with ISN/RPS classification + activity index + chronicity index (KDIGO 2024 GN; ISN/RPS 2003)
    advance: Class I-VI assigned + activity/chronicity scored (KDIGO 2024 GN)
  7. 7DIFFERENTIAL
    LN Class I-VI / mixed Class III+V or IV+V / TMA-on-LN / drug-induced lupus / overlap with other GN (anti-GBM, ANCA) (KDIGO 2024 GN)
    advance: Differential resolved (KDIGO 2024 GN)
  8. 8RISK_STRATIFICATION
    ISN/RPS class + activity/chronicity index + serology pattern + ethnicity + prior treatment response (KDIGO 2024 GN; EULAR/ACR 2019)
    inputs: creatinine, upcr, complement
    actions: calc.ckd_epi_2021
    advance: Risk tier + induction plan set (KDIGO 2024 GN)
  9. 9TREATMENT
    HCQ for ALL LN patients (reduces flare + renal survival); induction (Class III/IV/V) → MMF 2-3 g/d OR IV CY low-dose Euro-Lupus 500 mg q2w × 6 + steroid pulse (KDIGO 2024 GN; ALMS Appel JASN 2009); + belimumab BLISS-LN (Furie NEJM 2020); + voclosporin AURORA-1 if proteinuria persistent (Rovin Lancet 2021); maintenance → MMF 1-2 g/d OR azathioprine + low-dose steroid; ACEi/ARB renoprotective; SGLT2i emerging adjunct
    inputs: upcr, creatinine
    advance: Induction regimen started + agents reconciled (KDIGO 2024 GN)
  10. 10DISPOSITION
    Outpatient primary; admit for severe AKI / nephrotic flare / induction protocol / infection on immunosuppression; ICU for pulmonary-renal / refractory shock (KDIGO 2024 GN)
    advance: Disposition set (KDIGO 2024 GN)
  11. 11MONITORING
    UPCR + eGFR + complement + anti-dsDNA q1-3 months during induction; CBC for CY/MMF toxicity; infection surveillance; flare detection (KDIGO 2024 GN; EULAR/ACR 2019)
    inputs: creatinine, upcr, complement, anti_dsdna
    actions: panel.renal, panel.cbc
    advance: Monitoring schedule documented (KDIGO 2024 GN)
  12. 12FOLLOWUP
    Maintenance immunosuppression 3-5 years; CV-risk modification (statin, BP, glycemic); contraception/fertility planning; vaccination per ACIP 2026 (avoid live vaccines on immunosuppression); pregnancy planning post-remission (KDIGO 2024 GN; EULAR/ACR 2019)
    advance: Long-term plan documented (KDIGO 2024 GN)