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renal.rpgn.core.v1PRODUCTION
renal.rpgn.core.v1

Rapidly Progressive Glomerulonephritis

nephrologyacuteadult
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Care setting:

Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Confirm RPGN: rapid GFR loss + nephritic sediment; nephrology emergency (KDIGO 2021 GN)

Inputs
2
Actions
0
Advance rule
Set
Advance when

RPGN clinically suspected (KDIGO 2021 GN)

Patient inputs (6)

Cyclophosphamide vs rituximab selection; transplant candidacy (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)

Rate of decline drives urgency for biopsy + induction (KDIGO 2021 GN)

RBC casts + dysmorphic RBCs + proteinuria define active sediment (KDIGO 2021 GN)

PR3/MPO ANCA pauci-immune GPA/MPA/EGPA (ACR 2021 ANCA vasculitis; KDIGO 2021 GN)

Anti-GBM antibody linear IgG; pulmonary-renal emergency (KDIGO 2021 GN)

Low C3/C4 immune-complex GN lupus, post-infectious, MPGN (KDIGO 2021 GN)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (14)

14 need judgement
  • informationallife_threateninganti_gbm_type_i
    Type I anti-GBM disease — linear IgG on IF biopsy; anti-GBM antibody positive (Goodpasture if pulmonary involvement; KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpauci_immune_type_iii_anca
    Type III pauci-immune ANCA-associated vasculitis — negative or scant IF; PR3-ANCA (GPA) or MPO-ANCA (MPA/EGPA) (RAVE Stone NEJM 2010; ACR 2021 ANCA vasculitis)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningdouble_positive_type_iv
    Type IV double-positive — anti-GBM antibody + ANCA both detected (overlap phenotype; treat both pathways) (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpulmonary_renal_syndrome
    Hemoptysis + pulmonary infiltrates + AKI + active sediment — emergent overlap with GPA, MPA, anti-GBM, lupus, cryoglobulinemic (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningalveolar_hemorrhage
    Diffuse alveolar hemorrhage — hemoptysis + dropping Hb + bilateral infiltrates + BAL increasingly bloody (KDIGO 2021 GN; PEXIVAS Walsh NEJM 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningopportunistic_infection_on_immunosuppression — KDIGO 2021
    New fever / leukopenia / hypoxia in patient on induction CY or rituximab (suspect PJP, CMV, invasive fungal, Strongyloides hyperinfection, HBV reactivation — KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereic_mediated_type_ii
    Type II immune-complex mediated — granular IF pattern (sub-types: IgA-vasculitis/HSP, lupus nephritis class III/IV/V, post-infectious GN, cryoglobulinemic, MPGN; treatment per underlying disease) (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereidiopathic_type_v
    Type V idiopathic RPGN — crescentic GN without identifiable IF pattern, negative serology, no underlying disease (rare; KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverecrescentic_gt_50pct
    Crescentic on biopsy — >50% glomeruli with cellular/fibrocellular crescents (worst histologic prognosis; aggressive immunosuppression mandatory) (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveredialysis_dependent_at_presentation
    Dialysis-dependent on presentation — lower likelihood of renal recovery; consider PLEX for ANCA per PEXIVAS subgroup analysis (PEXIVAS Walsh NEJM 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererefractory_after_5_pex — KDIGO 2021
    No improvement after 5 PEX sessions in anti-GBM/severe AAV (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererefractory_induction_3_months — KDIGO 2021
    Persistent active disease at 3 months despite full induction (cyclophosphamide or rituximab + steroids — KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatecyclophosphamide_pregnancy_contraindication
    Cyclophosphamide contraindicated — pregnancy, future fertility desired without banking, severe leukopenia, prior CY exposure (lifetime cumulative >36 g) (ACR 2021 ANCA vasculitis; KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatebiopsy_delay_high_suspicion — KDIGO 2021
    High clinical suspicion + positive serology + biopsy delay >24h (KDIGO 2021 GN)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

This dossier does not reference any calculators.

Recommended regimen

RPGN induction by histopathology subtype (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
axis: rpgn_inductionstep pulse_steroid - Pulse methylprednisolone — universal induction backbone (KDIGO 2021 GN)
Selected step "Pulse methylprednisolone — universal induction backbone (KDIGO 2021 GN)" — Biopsy-confirmed crescentic GN OR high clinical suspicion + serology positive (KDIGO 2021 GN)
  • methylprednisolone
    first line
    corticosteroid_pulse
    1000 mg IV • IV • daily × 3 days
    triggers: crescentic_gn
    KDIGO 2021 GN — pulse precedes oral; rapid anti-inflammatory effect
    rxcui 6902
  • prednisone
    first line
    corticosteroid_oral
    1 mg/kg PO daily (max 60 mg) • PO • daily, taper over 6 months per PEXIVAS reduced-dose schedule
    triggers: after_pulse
    PEXIVAS Walsh NEJM 2020 reduced-dose regimen — equivalent efficacy, less infection
    rxcui 8640

outpatient playbook — drug actions (5)

  1. 1. rituximab maintenance
    500 mg IV q6 months × 2-4 years • IV • q6 months
    trigger: Remission achieved post-induction (MAINRITSAN Guillevin NEJM 2014)
    MAINRITSAN — superior to azathioprine for AAV maintenance
  2. 2. azathioprine OR mycophenolate (alternative)
    AZA 1.5-2 mg/kg PO daily; MMF 1000 mg PO BID • PO • daily or BID
    trigger: Rituximab unavailable or contraindicated (KDIGO 2021 GN)
    CYCAZAREM AZA arm; MMF if AZA intolerant or TPMT deficient (KDIGO 2021 GN)
  3. 3. oral prednisone taper continuation
    Per PEXIVAS reduced-dose schedule • PO • daily
    trigger: Ongoing taper at clinic visit
    PEXIVAS Walsh NEJM 2020 reduced-dose protocol
  4. 4. TMP-SMX prophylaxis
    160/800 PO M-W-F or 80/400 daily • PO • 3x/week or daily
    trigger: First 6-12 months of maintenance immunosuppression
    PJP prevention (KDIGO 2021 GN)
  5. 5. antihypertensive (ACEi/ARB)
    Per BP target <130/80 • PO • daily
    trigger: Proteinuria or HTN
    Renoprotection + CV-risk (KDIGO 2021 GN)

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: RBC casts on urine microscopy (KDIGO 2021 GN); eGFR decline >=50% over days-weeks (KDIGO 2021 GN); Pulmonary-renal syndrome hemoptysis + AKI (KDIGO 2021 GN; ACR 2021 ANCA vasculitis).

Objective

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

Assessment

**Rapidly Progressive Glomerulonephritis** (renal.rpgn.core.v1).
Phenotype framing: Anti-GBM / pauci-immune ANCA GPA MPA EGPA / immune-complex lupus IgA post-infectious MPGN (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
Scope: Confirm RPGN: rapid GFR loss + nephritic sediment; nephrology emergency (KDIGO 2021 GN)

No severity triggers fired against current inputs.

Plan

Regimen axis: **RPGN induction by histopathology subtype (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)** — step "Pulse methylprednisolone — universal induction backbone (KDIGO 2021 GN)".
1. methylprednisolone 1000 mg IV IV daily × 3 days (corticosteroid_pulse, first line) — KDIGO 2021 GN — pulse precedes oral; rapid anti-inflammatory effect
2. prednisone 1 mg/kg PO daily (max 60 mg) PO daily, taper over 6 months per PEXIVAS reduced-dose schedule (corticosteroid_oral, first line) — PEXIVAS Walsh NEJM 2020 reduced-dose regimen — equivalent efficacy, less infection

Setting playbook (outpatient) — Maintenance immunosuppression, relapse surveillance, infection prophylaxis, taper schedule + bone/CV health + fertility planning (KDIGO 2021 GN; MAINRITSAN Guillevin NEJM 2014)
3. rituximab maintenance 500 mg IV q6 months × 2-4 years IV q6 months — Remission achieved post-induction (MAINRITSAN Guillevin NEJM 2014) (MAINRITSAN — superior to azathioprine for AAV maintenance)
4. azathioprine OR mycophenolate (alternative) AZA 1.5-2 mg/kg PO daily; MMF 1000 mg PO BID PO daily or BID — Rituximab unavailable or contraindicated (KDIGO 2021 GN) (CYCAZAREM AZA arm; MMF if AZA intolerant or TPMT deficient (KDIGO 2021 GN))
5. oral prednisone taper continuation Per PEXIVAS reduced-dose schedule PO daily — Ongoing taper at clinic visit (PEXIVAS Walsh NEJM 2020 reduced-dose protocol)
6. TMP-SMX prophylaxis 160/800 PO M-W-F or 80/400 daily PO 3x/week or daily — First 6-12 months of maintenance immunosuppression (PJP prevention (KDIGO 2021 GN))
7. antihypertensive (ACEi/ARB) Per BP target <130/80 PO daily — Proteinuria or HTN (Renoprotection + CV-risk (KDIGO 2021 GN))

Non-pharmacologic actions:
- Maintenance vaccination per ACIP 2026 — annual influenza, COVID, pneumococcal (PCV20), HBV; AVOID live vaccines while on rituximab/CY (KDIGO 2021 GN)
- Bone health — Ca + vit D + bisphosphonate per FRAX (KDIGO 2021 GN)
- CV-risk modification — statin per ACC/AHA Lipid 2026; smoking cessation (KDIGO 2021 GN)
- Fertility counselling — sperm/oocyte banking pre-CY if planned (KDIGO 2021 GN)
- Cervical/skin cancer surveillance (long-term immunosuppression; KDIGO 2021 GN)
- Patient action card reinforced — hemoptysis/oliguria/edema → ED (KDIGO 2021 GN)

AVOID / contraindication checks:
- Cyclophosphamide fertility counsel and uroprotect (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)
- Rituximab vaccinate prior (KDIGO 2021 GN)
- Steroid pjp prophylaxis (KDIGO 2021 GN)
- Pulse mp monitor glucose bp (KDIGO 2021 GN)

Monitoring

Regimen monitoring:
- CBC q1w during induction (KDIGO 2021 GN)
- Cr q24-48h during induction (KDIGO 2021 GN)
- ANCA q3m for relapse surveillance (ACR 2021 ANCA vasculitis)
- CD19 count for rituximab redosing (KDIGO 2021 GN; MAINRITSAN Guillevin NEJM 2014)
- HBV HCV TB baseline screen (KDIGO 2021 GN)

Setting (outpatient) monitoring:
- Cr + UA every clinic visit (KDIGO 2021 GN)
- ANCA titer q3 months (ACR 2021 ANCA vasculitis)
- Hep B DNA if HBcAb+ or HBsAg+ on rituximab (KDIGO 2021 GN)
- DEXA at 6-12 months (KDIGO 2021 GN)
- Annual cancer screen (long-term CY exposure — bladder, hematologic; KDIGO 2021 GN)

Follow-up plan: Maintenance immunosuppression MAINRITSAN rituximab, relapse surveillance ANCA titers + urine, vaccination, transplant if ESRD (KDIGO 2021 GN)
- Close-out criterion: Follow-up scheduled (KDIGO 2021 GN)

Monitoring phase: Daily Cr, UOP, CBC for cyclophosphamide toxicity, infection surveillance (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)

Disposition

Current setting: outpatient — Maintenance immunosuppression, relapse surveillance, infection prophylaxis, taper schedule + bone/CV health + fertility planning (KDIGO 2021 GN; MAINRITSAN Guillevin NEJM 2014)

Disposition criteria:
- Continue maintenance at 3-monthly visits if stable (KDIGO 2021 GN)
- Transition to nephrology + transplant clinic if approaching ESRD (KDIGO 2021 GN)

Escalation triggers (move to higher acuity):
- Rising ANCA titer + new sediment + new symptoms → relapse → re-induction (ACR 2021 ANCA vasculitis)
- New hemoptysis → ED (KDIGO 2021 GN)
- New cytopenia → CBC + BM consideration; CY/AZA toxicity (KDIGO 2021 GN)
- CKD progression toward ESRD → transplant evaluation (KDIGO 2021 GN)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Type I anti-GBM disease — linear IgG on IF biopsy; anti-GBM antibody positive (Goodpasture if pulmonary involvement; KDIGO 2021 GN)
- [LIFE_THREATENING] Type III pauci-immune ANCA-associated vasculitis — negative or scant IF; PR3-ANCA (GPA) or MPO-ANCA (MPA/EGPA) (RAVE Stone NEJM 2010; ACR 2021 ANCA vasculitis)
- [LIFE_THREATENING] Type IV double-positive — anti-GBM antibody + ANCA both detected (overlap phenotype; treat both pathways) (KDIGO 2021 GN)

Citations

- KDIGO 2021 Glomerular Diseases (Kidney Int Oct 2021) + ACR/EULAR ANCA-Associated Vasculitis guidance + RAVE NEJM 2010 + RITUXVAS NEJM 2010 + CYCLOPS Ann Intern Med 2009 + PEXIVAS NEJM 2020 + MAINRITSAN NEJM 2014 (rituximab maintenance) + MEPEX JASN 2007 + Levy 2001 Goodpasture cohort + ADVOCATE NEJM 2021 (avacopan) + AURORA-1 Lancet 2021 (voclosporin in LN) + BLISS-LN NEJM 2020 (belimumab) + NefIgArd Lancet 2023 (budesonide in IgAN) [PMID:20647199](https://pubmed.ncbi.nlm.nih.gov/20647199/)
- Cited evidence (PMID 20647198) [PMID:20647198](https://pubmed.ncbi.nlm.nih.gov/20647198/)
- Cited evidence (PMID 19451574) [PMID:19451574](https://pubmed.ncbi.nlm.nih.gov/19451574/)
- Cited evidence (PMID 32053298) [PMID:32053298](https://pubmed.ncbi.nlm.nih.gov/32053298/)
- Cited evidence (PMID 34556256) [PMID:34556256](https://pubmed.ncbi.nlm.nih.gov/34556256/)

Last reconciled with current guidelines: 2026-05-22.
References
  • KDIGO 2021 Glomerular Diseases (Kidney Int Oct 2021) + ACR/EULAR ANCA-Associated Vasculitis guidance + RAVE NEJM 2010 + RITUXVAS NEJM 2010 + CYCLOPS Ann Intern Med 2009 + PEXIVAS NEJM 2020 + MAINRITSAN NEJM 2014 (rituximab maintenance) + MEPEX JASN 2007 + Levy 2001 Goodpasture cohort + ADVOCATE NEJM 2021 (avacopan) + AURORA-1 Lancet 2021 (voclosporin in LN) + BLISS-LN NEJM 2020 (belimumab) + NefIgArd Lancet 2023 (budesonide in IgAN) — PMID:20647199
  • Cited evidence (PMID 20647198) — PMID:20647198
  • Cited evidence (PMID 19451574) — PMID:19451574
  • Cited evidence (PMID 32053298) — PMID:32053298
  • Cited evidence (PMID 34556256) — PMID:34556256