Patient handout

Rapidly Progressive Glomerulonephritis

PRODUCTION

1. Your condition

This handout is for rapidly progressive glomerulonephritis. Your care team identified this based on: rbc casts on urine microscopy (kdigo 2021 gn).

Other reasons your team may use this plan: egfr decline >=50% over days-weeks (kdigo 2021 gn); pulmonary-renal syndrome hemoptysis + aki (kdigo 2021 gn; acr 2021 anca vasculitis).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

Medication	Starting dose	How	When	What it does
methylprednisolone	1000 mg IV	IV	daily × 3 days	KDIGO 2021 GN — pulse precedes oral; rapid anti-inflammatory effect
prednisone	1 mg/kg PO daily (max 60 mg)	PO	daily, taper over 6 months per PEXIVAS reduced-dose schedule	PEXIVAS Walsh NEJM 2020 reduced-dose regimen — equivalent efficacy, less infection

Plan: RPGN induction by histopathology subtype (KDIGO 2021 GN; ACR 2021 ANCA vasculitis)

3. When to call your provider

Contact your care team if any of the following happen:

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)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

Type I anti-GBM disease — linear IgG on IF biopsy; anti-GBM antibody positive (Goodpasture if pulmonary involvement; KDIGO 2021 GN)(life-threatening)
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)
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)(life-threatening)
Type V idiopathic RPGN — crescentic GN without identifiable IF pattern, negative serology, no underlying disease (rare; KDIGO 2021 GN)
Hemoptysis + pulmonary infiltrates + AKI + active sediment — emergent overlap with GPA, MPA, anti-GBM, lupus, cryoglobulinemic (KDIGO 2021 GN)(life-threatening)
Crescentic on biopsy — >50% glomeruli with cellular/fibrocellular crescents (worst histologic prognosis; aggressive immunosuppression mandatory) (KDIGO 2021 GN)
Dialysis-dependent on presentation — lower likelihood of renal recovery; consider PLEX for ANCA per PEXIVAS subgroup analysis (PEXIVAS Walsh NEJM 2020)
Diffuse alveolar hemorrhage — hemoptysis + dropping Hb + bilateral infiltrates + BAL increasingly bloody (KDIGO 2021 GN; PEXIVAS Walsh NEJM 2020)(life-threatening)
No improvement after 5 PEX sessions in anti-GBM/severe AAV (KDIGO 2021 GN)
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)(life-threatening)
Persistent active disease at 3 months despite full induction (cyclophosphamide or rituximab + steroids — KDIGO 2021 GN; ACR 2021 ANCA vasculitis)

5. Follow-up

Maintenance immunosuppression MAINRITSAN rituximab, relapse surveillance ANCA titers + urine, vaccination, transplant if ESRD (KDIGO 2021 GN)

6. Sources

Guideline: 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)