# ANCA Vasculitis and RPGN: Recognize an Organ-Threatening Syndrome

Escalate the pulmonary–renal syndrome immediately, establish the immune mechanism, and distinguish urgent induction from selected plasma exchange and long-term relapse prevention.

![Infographic: ANCA Vasculitis and RPGN: Recognize an Organ-Threatening Syndrome](https://urinenephrology.org/visual-reference/images/student-anca-rpgn.png?v=20261003c)

## Recognize organ-threatening disease

AKI with glomerular hematuria/proteinuria plus pulmonary hemorrhage, hypoxemia, purpura, neuropathy, or ENT disease needs urgent vasculitis assessment. RPGN is a clinical syndrome, not a synonym for ANCA disease. Infection, anti-GBM, and immune-complex disorders can mimic it.

## Send targeted tests promptly

Obtain MPO/PR3-ANCA, anti-GBM, complements, urine microscopy/protein, CBC, and infection testing as indicated. Arrange kidney biopsy when feasible. Compatible rapidly progressive disease with positive serology may require treatment before the biopsy report; diagnostic workup and stabilization proceed together.

## Induction requires a specialist plan

For new organ-threatening ANCA vasculitis, established induction uses glucocorticoids with rituximab or cyclophosphamide. Disease severity, relapsing/PR3 phenotype, kidney function, fertility, infection risk, and prior exposure influence selection. A positive ANCA result without the clinical syndrome is not an indication.

## Plasma exchange is selective

KDIGO 2024 suggests consideration with creatinine >3.4 mg/dL, dialysis requirement or rapidly rising creatinine, or hypoxemic diffuse alveolar hemorrhage. Add it for ANCA/anti-GBM overlap. These are expert decisions balancing kidney/bleeding severity against infection and procedural risks, not automatic treatment of every positive ANCA.

## Recovery and maintenance are separate decisions

Dialysis at presentation does not prove irreversibility. Follow urine output, creatinine, sediment, lung status, and biopsy chronicity. Once remission is achieved, arrange maintenance and relapse surveillance; infection prophylaxis, CBC, immunoglobulins, and drug-specific toxicity monitoring remain integral.

## Update the avacopan evidence

ADVOCATE was retracted in 2026. Its historical efficacy and comparative-safety results are unreliable supporting evidence. Keep the trial labeled retracted with the notice and reassess other evidence/current guidance separately; older guideline text predates this event.

## Supporting evidence

- [Official clinical guidance](https://kdigo.org/wp-content/uploads/2024/05/KDIGO-2024-ANCA-Vasculitis-Guideline-Update.pdf)
- [RETRACTED — historical ADVOCATE report: Avacopan for the Treatment of ANCA-Associated Vasculitis (2021)](https://pubmed.ncbi.nlm.nih.gov/33596356/)
- [Retraction notice for the ADVOCATE avacopan report (2026)](https://pubmed.ncbi.nlm.nih.gov/42377355/)

## Source lessons

- [anca vasculitis student handout](https://urinenephrology.org/student-resources/glomerular/anca-vasculitis-student-handout.html)

Read alongside the full lessons; the findings and decisions shown here require the stated clinical context.