Rapidly Progressive Glomerulonephritis
Phase B contract-depth deepening 2026-05-14: IF mechanism phenotypes (Type I-V) encoded as severity_triggers per shard pattern (siblings unavailable for anti-GBM-specific / ANCA-vasculitis-specific / cryoglobulinemic engines — Phase C targets). 5 settings (home/ed/icu/inpatient/outpatient) — home covers patient action plan + maintenance home actions; outpatient covers maintenance immunosuppression + relapse surveillance + bone/CV/fertility planning. Sources workup: workup.rpgn (registered in clinical-tools-registry.ts). Engine registry id (runtime class) not yet present in src/lib/engines/nephrology/. Linked from neph.aki.core.v1 GN phenotype branch. Evidence references RAVE / RITUXVAS / CYCLOPS / PEXIVAS / MEPEX / MAINRITSAN / Levy 2001 / AURORA-1 / BLISS-LN / NefIgArd / KDIGO 2021 GN. Schema-blocked calculator queue (depth bundle): BVAS (Birmingham Vasculitis Activity Score), VDI (Vasculitis Damage Index), KDIGO AKI staging calculator, IgA nephropathy risk-prediction (Barbour 2019 PMID 30980653), UPCR — none registered in clinical-tools-registry; surfaced as required_assessments text until registry expansion (owner: shard-0 clinical-tools-registry maintainer). Terminology expanded 2026-05-12 to cover ANCA subtype ICD-10 (M31.x / M30.1 / M31.0) + serology LOINC (anti-MPO, anti-PR3, anti-GBM, C3/C4, cryoglobulin, anti-dsDNA) + SNOMED for GPA / MPA / EGPA / Goodpasture / lupus nephritis / IgA / crescentic GN / MPGN. Citation + RxCUI remediation 2026-05-22: every evidence PMID re-verified live via PubMed. Verified-on-topic anchors retained (RAVE 20647199, RITUXVAS 20647198, CYCLOPS 19451574, PEXIVAS 32053298) and cross-referenced GN trials corrected (AURORA-1 33812489→33971155, BLISS-LN 32726528→32937045, NefIgArd 36368506→37591292, KDIGO-GN 34556250→34556256, Barbour-IIgAN 31048499→30980653). Fabricated/mis-attributed codes removed from evidence array (MAINRITSAN 25372088=handoff-errors, MEPEX 17944708=fungal-lacaziosis, Levy 11576985=BMJ-SIGN-NICE, ADVOCATE 33596357=uterine-fibroid) — those trial names remain as PMID-less text. RxCUI corrected: mycophenolate_mofetil 6932→68149.
Entry points (3)
- lab_abnormalityRBC casts on urine microscopy (KDIGO 2021 GN)rbc_casts
- lab_abnormalityeGFR decline >=50% over days-weeks (KDIGO 2021 GN)rapid_egfr_decline
- symptomPulmonary-renal syndrome hemoptysis + AKI (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)pulmonary_renal
Required inputs (6)
- agerequireddemographic • used at CONTEXTCyclophosphamide vs rituximab selection; transplant candidacy (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
- creatininerequiredlab • used at CONTEXTRate of decline drives urgency for biopsy + induction (KDIGO 2021 GN)
- urinalysisrequiredlab • used at INITIAL_WORKUPRBC casts + dysmorphic RBCs + proteinuria define active sediment (KDIGO 2021 GN)
- ancalab • used at BRANCHING_WORKUPPR3/MPO ANCA pauci-immune GPA/MPA/EGPA (ACR 2021 ANCA vasculitis; KDIGO 2021 GN)
- anti_gbmlab • used at BRANCHING_WORKUPAnti-GBM antibody linear IgG; pulmonary-renal emergency (KDIGO 2021 GN)
- complementlab • used at BRANCHING_WORKUPLow C3/C4 immune-complex GN lupus, post-infectious, MPGN (KDIGO 2021 GN)
12-phase flow (12)
- 1FRAMEConfirm RPGN: rapid GFR loss + nephritic sediment; nephrology emergency (KDIGO 2021 GN)inputs: creatinine, urinalysisadvance: RPGN clinically suspected (KDIGO 2021 GN)
- 2ENTRYTriggered from neph.aki.core.v1 GN phenotype or independent presentation (KDIGO 2021 GN)advance: Patient routed (KDIGO 2021 GN)
- 3CONTEXTCapture autoimmune history, medications, recent infection, pulmonary symptoms (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)inputs: creatinineadvance: Comorbidity review complete (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
- 4RED_FLAGSPulmonary hemorrhage; severe AKI requiring RRT; respiratory failure (KDIGO 2021 GN)advance: Stabilized (KDIGO 2021 GN)
- 5INITIAL_WORKUPUrine micro, UPCR, complement, ANA, ANCA, anti-GBM, hepatitis serologies, cryoglobulins, CXR (KDIGO 2021 GN)inputs: urinalysisactions: workup.rpgnadvance: Serology panel sent + biopsy planned (KDIGO 2021 GN)
- 6BRANCHING_WORKUPRenal biopsy STAT for IF classification linear / pauci-immune / immune-complex (KDIGO 2021 GN)inputs: anca, anti_gbm, complementadvance: Biopsy classification + serology resolved (KDIGO 2021 GN)
- 7DIFFERENTIALAnti-GBM / pauci-immune ANCA GPA MPA EGPA / immune-complex lupus IgA post-infectious MPGN (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)advance: Subtype assigned (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
- 8RISK_STRATIFICATIONSeverity by eGFR, dialysis-dependence, pulmonary involvement, BVAS for ANCA (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)advance: Severity documented (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
- 9TREATMENTInduction: pulse methylprednisolone + cyclophosphamide OR rituximab RAVE Stone NEJM 2010; plasmapheresis for anti-GBM and severe pulm hemorrhage PEXIVAS Walsh NEJM 2020; maintenance with rituximab MAINRITSAN or AZA (KDIGO 2021 GN)advance: Induction regimen started (KDIGO 2021 GN; RAVE Stone NEJM 2010)
- 10DISPOSITIONICU if pulm hemorrhage; nephrology + rheumatology co-management (KDIGO 2021 GN)advance: Disposition set (KDIGO 2021 GN)
- 11MONITORINGDaily Cr, UOP, CBC for cyclophosphamide toxicity, infection surveillance (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)inputs: creatinineadvance: Monitoring schedule documented (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
- 12FOLLOWUPMaintenance immunosuppression MAINRITSAN rituximab, relapse surveillance ANCA titers + urine, vaccination, transplant if ESRD (KDIGO 2021 GN)advance: Follow-up scheduled (KDIGO 2021 GN)