Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a relatively uncommon group of diseases. People with ANCA-associated vasculitides have inflammation and necrosis of small and medium sized blood vessels. There are three subtypes: granulomatosis with polyangitis (GPA), microscopic polyangitis (MPA), and eosinophilic granulomatosis with polyangitis (EGPA). GPA and MPA are the most common subtypes and are the focus of today's article. With these subtypes, treatment aims to rapidly induce remission with immunosuppression while limiting treatment toxicity. 

In today's side-by-side comparison, we compare the latest clinical practice guidelines from the American College of Rheumatology (ACR), the European League Against Rheumatism (EULAR), Kidney Disease Improving Global Outcomes (KDIGO), and the British Society for Rheumatology (BSR) on AAV. The recommendations made are meant to guide clinical practice, taking into consideration the unique desires and needs of individual patients. Pediatric recommendations were not reviewed in this article.

Guidelines for Comparison
Item2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated VasculitisEULAR Recommendations for the Management of ANCA-associated Vasculitis: 2022 UpdateKDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated VasculitisThe 2025 British Society for Rheumatology Management Recommendations for ANCA-associated Vasculitis
Authoring SocietyAmerican College of RheumatologyEuropean League Against RheumatismKidney Disease Improving Global OutcomesBritish Society of Rheumatology
Publication DateJuly 2021January 2024February 2024June 2025
Graded RecommendationsYesNoYesYes
Uses GRADEYesNoYesYes
LinksSummary / Full TextSummary / Full TextSummary / Full TextSummary / Full Text
Key Takeaways

Induction of Remission

  • Cyclophosphamide (CYC) or rituximab (RTX)
    • EULAR, KDIGO, and BSR recommend either CYC or RTX for induction of remission with no preference for either agent. 
    • ACR prefers RTX over CYC for patients with active severe disease.
  • Combination CYC and RTX
    • BSR recommends considering combination therapy with both CYC and RTX for patients with organ- or life-threatening disease. 
    • KDIGO suggests considering combination CYC and RTX treatment for patients with markedly reduced or rapidly declining glomerular filtration rate (GFR).
    • ACR and EULAR did not make a recommendation regarding combination therapy.
  • Methotrexate (MTX)
    • BSR and EULAR recommend considering MTX for certain patients as an alternative to RTX and CYC.
    • ACR recommends MTX over CYC or RTX for patients with non-severe disease.
    • KDIGO did not make a recommendation on the use of MTX for induction therapy.
  • Mycophenolate mofetil (MMF)
    • BSR and EULAR recommend considering MMF for certain patients as an alternative to RTX and CYC.
    • ACR suggests MMF as an option for certain patients.
    • KDIGO did not make a recommendation on the use of MMF for induction therapy.

Avacopan

  • BSR and EULAR recommend considering use of avacopan as a steroid sparing agent, with or without short course glucocorticoids.
  • KDIGO recommends considering the use of avacopan as an alternative to glucocorticoids in induction therapy.
  • Acacopan was not FDA approved at the time of the ACR's guideline development and therefore was not addressed.

Glucocorticoids

  • All of the guidelines agree with reduced-dose glucocorticoid tapering for induction of remission.
  • Both ACR and BSR note that IV pulse glucocorticoids may be offered to some patients with organ- or life-threatening disease.

Plasmapharesis

  • EULAR, KDIGO, and BSR consider plasmapheresis as an option for patients with serum creatinine >300 µmol/L.
  • KDIGO practice point suggests considering plasmapheresis in patients with diffuse alveolar hemorrhage and hypoxaemia. This is in disagreement with ACR, EULAR, and BSR which do not recommend plasmapheresis for patients with alveolar hemorrhage.
  • ACR does not recommend routine plasmapheresis.

Maintenance of Remission

  • RTX
    • RTX is the preferred agent for remission maintenance according to BSR, EULAR, and ACR.
    • KDIGO suggests rituximab or azathioprine (AZA) for maintenance of remission.
  • AZA or MTX
    • BSR, EULAR and ACR recommend AZA and MTX as alternatives that may be used for maintenance of remission when rituximab is not used.
    • KDIGO suggests considering MTX for patients unable to take AZA for maintenance therapy.
  • MMF
    • BSR recommends the use of MMF for maintenance of remission be limited to patients who cannot use RTX, AZA, and MTX.
    • KDIGO considers the use of MMF for patients unable to take AZA for maintenance therapy.

Duration of Immunosuppression

  • EULAR and BSR recommend maintenance immunosuppressive therapy for 24-48 months.
  • KDIGO recommends maintenance immunosuppressive therapy between 18-48 months. 
  • ACR recommends the duration of maintenance therapy be guided by the patient's clinical condition, values, and preferences.
  • BSR and KDIGO note that maintenance of remission may not be needed for patients with renal limited disease who are on dialysis. This was not addressed by the other guidelines.

Maintenance of Remission During Glucocorticoid Steroid Treatment

  • Only ACR and BSR made recommendations regarding the duration of glucocorticoid therapy for maintenance of remission. 
  • BSR states that the duration of glucocorticoids for maintenance of remission is uncertain, but withdrawal may be possible within 6 to 12 months following induction of remission treatment.
  • ACR recommends the duration of treatment be guided by the patient’s clinical condition, preferences, and values.

Timing of Kidney Transplant

  • BSR and KDIGO recommend patients be in stable remission for at least 6 to 12 months before receiving a kidney transplant.
  • ACR recommends patients with stage 5 chronic kidney disease be evaluated for renal transplantation.
  • EULAR did not address kidney transplantation.
Comparison of Recommendations
TypeACREULARKDIGOBSR
Induction of RemissionFor patients with active, severe GPA/MPA, we conditionally recommend treatment with rituximab over cyclophosphamide for remission induction.

Remission induction for active, nonsevere disease:

For patients with active, nonsevere GPA, we conditionally recommend initiating treatment with methotrexate over cyclophosphamide or rituximab. 

For patients with active, nonsevere GPA, we conditionally recommend initiating treatment with methotrexate and GCs over GCs alone.

For patients with active, nonsevere GPA, we conditionally recommend initiating treatment with methotrexate and GCs over azathioprine and GCs or mycophenolate mofetil and GCs.

For patients with active, nonsevere GPA, we conditionally recommend initiating treatment with methotrexate and GCs over trimethoprim/sulfamethoxazole and GCs.		For induction of remission in patients with new-onset or relapsing GPA or MPA with organ-threatening or life-threatening disease, we recommend treatment with a combination of glucocorticoids (GCs) and either RTX or CYC. RTX is preferred in relapsing disease.

For induction of remission of non-organ-threatening or non-life-threatening GPA or MPA, treatment with a combination of GCs and RTX is recommended. Methotrexate (MTX) or MMF can be considered as alternatives to RTX.		We recommend that glucocorticoids in combination with rituximab or cyclophosphamide be used as initial treatment of new-onset AAV.

In patients presenting with markedly reduced or rapidly declining glomerular filtration rate (GFR) (serum creatinine [SCr] >4 mg/dl [>354 μmol/l]), there are limited data to support rituximab and glucocorticoids. Both cyclophosphamide and glucocorticoids, and the combination of rituximab and cyclophosphamide can be considered in this setting.		All people with lived experience of active GPA or MPA should be assessed for induction of remission treatment with immunosuppressants combined with glucocorticoids (GC) or avacopan. 

The recommended options for immunosuppression for remission induction of newly diagnosed GPA or MPA are intravenous pulsed cyclophosphamide (CYC) or rituximab (RTX). 

For active relapsing disease, treatment with RTX is preferred.

A combination of both CYC and RTX can be considered for organ-threatening or life-threatening disease.

Certain individuals with active GPA or MPA, with no evidence of life- or organ-threatening disease, may be considered for alternative induction therapy with methotrexate (MTX) or mycophenolate mofetil (MMF).
AvacopanNot addressed.Avacopan, in combination with RTX or CYC, may be considered for induction of remission in GPA or MPA as part of a strategy to substantially reduce exposure to GCs.Avacopan may be used as an alternative to glucocorticoids. Patients with an increased risk of glucocorticoids toxicity are likely to receive the most benefit from avacopan. Patients with lower GFR may benefit from greater GFR recovery.Patients with active GPA or MPA should be considered for avacopan use as a steroid sparing agent, with or without a short course of glucocorticoids (tapering over four weeks).
GlucocorticoidsIn patients with active, severe GPA/MPA, we conditionally recommend a reduced-dose GC regimen over a standard-dose GC regimen for remission induction.

For patients with active, severe GPA/MPA, either IV pulse GCs or high-dose oral GCs may be prescribed as part of initial therapy.		As part of regimens for induction of remission in GPA or MPA, we recommend treatment with oral GCs at a starting dose of 50–75 mg prednisolone equivalent/day, depending on body weight. We recommend stepwise reduction in GCs according to PEXIVAS trial, achieving a dose of 5 mg prednisolone equivalent per day by 4–5 months.Prednisolone starting dose depends on weight and should be between 50 - 75 mg, dose tapering in accordance with the PEXIVAS trial.In organ- or life-threatening disease, we advocate treatment with oral GC at a starting dose of 50–75 mg or 1.0 mg/kg/day (dependent on weight with a maximum of 75 mg daily). Oral GC (prednisolone) should be tapered in accordance with the PEXIVAS tapering schedule, achieving a dose of 5 mg prednisolone equivalent per day by 4–5 months.

In non-organ- or non-life-threatening disease, lower GC-tapering regimens can be considered, at a starting dose of 0.5 mg/kg/day oral GC (prednisolone), with tapering in accordance with the LoVAS regimen.

Despite commonplace use, there is a lack of supporting trial evidence for intravenous methylprednisolone (IV MP) pulses. Therefore, IV MP pulses are not routinely recommended but can be reserved as an option for the management of organ-threatening manifestations, including active renal disease and diffuse alveolar haemorrhage.
PlasmapheresisIn patients with GPA/MPA with active glomerulonephritis, we conditionally recommend against the routine addition of plasma exchange to remission induction therapy.

In patients with active, severe GPA/MPA with alveolar hemorrhage, we conditionally recommend against adding plasma exchange to remission induction therapies.		PLEX may be considered as part of therapy to induce remission in GPA or MPA for those with a serum creatinine >300 µmol/L due to active glomerulonephritis.

Routine use of plasma exchange (PLEX) to treat alveolar haemorrhage in GPA and MPA is not recommended.		Consider plasma exchange for patients with SCr >3.4 mg/dl (>300 μmol/l), patients requiring dialysis or with rapidly increasing SCr, or patients with diffuse alveolar hemorrhage who have hypoxemia.

In the setting of diffuse alveolar bleeding with hypoxemia, plasma exchange can be considered in addition to glucocorticoids with either cyclophosphamide or rituximab.		Active GPA or MPA and severe kidney involvement with creatinine >300 μmol/l should be considered for adjunctive plasmapheresis provided their risk of potential adverse events has been considered.

Adjunctive plasmapheresis is not routinely recommended for pulmonary haemorrhage without severe kidney involvement.
Maintenance of RemissionFor patients with severe GPA/MPA whose disease has entered remission after treatment with cyclophosphamide or rituximab, we conditionally recommend treatment with rituximab over methotrexate or azathioprine for remission maintenance.

For patients with GPA/MPA who are receiving rituximab for remission maintenance, we conditionally recommend scheduled re-dosing over using ANCA titers or CD19+ B cell counts to guide re-dosing.

For patients with severe GPA/MPA whose disease has entered remission after treatment with cyclophosphamide or rituximab, we conditionally recommend treatment with methotrexate or azathioprine over mycophenolate mofetil for remission maintenance.

For patients with severe GPA/MPA whose disease has entered remission after treatment with cyclophosphamide or rituximab, we conditionally recommend treatment with methotrexate or azathioprine over leflunomide for remission maintenance.

For patients with GPA whose disease has entered remission, we conditionally recommend treatment with methotrexate or azathioprine over trimethoprim/sulfame thoxazole for remission maintenance.

In patients with GPA whose disease has entered remission, we conditionally recommend against adding trimethoprim/sulfamethoxazole to other therapies (e.g., rituximab, azathioprine, methotrexate, etc.) for the purpose of remission maintenance.

For patients with GPA/MPA receiving remission maintenance therapy with rituximab who have hypogammaglobulinemia (e.g., IgG <3 gm/liter) and recurrent severe infections, we conditionally recommend immunoglobulin supplementation.		For maintenance of remission of GPA and MPA, after induction of remission with either RTX or CYC, we recommend treatment with RTX. AZA or MTX may be considered as alternatives.We recommend maintenance therapy with either rituximab, or azathioprine and low-dose glucocorticoids after induction of remission.

Recommended rituximab dosing: 
1. 500mg x 2 at complete remission, and 500 mg at month 6, 12, and 18 thereafter OR 
2. 1000 mg infusion after induction of remission, and at month 4, 8, 12, and 16 after the first infusion.

Consider mycophenolate mofetil (MMF) or methotrexate as alternatives to azathioprine for maintenance therapy in patients intolerant of azathioprine. Methotrexate should not be used for patients with a GFR <60 ml/min per 1.73 m2.		Following induction of remission with an RTX or CYC-based treatment regimen, we recommend maintenance of remission with RTX in preference to other agents.

Azathioprine (AZA) or MTX may be considered as alternative options.

MMF is an option only where there is intolerance, or a contraindication, to RTX, AZA or MTX.
Duration of Immunosuppression for Remission MaintenanceThe duration of non-GC remission maintenance therapy in GPA/MPA should be guided by the patient’s clinical condition, preferences, and values.We recommend that therapy to maintain remission for GPA and MPA be continued for 24–48 months following induction of remission of new-onset disease.

Longer duration of therapy should be considered in relapsing patients or those with an increased risk of relapse, but should be balanced against patient preferences and risks of continuing immunosuppression.		The optimal duration of remission therapy is between 18 months and 4 years after induction of remission.

Consider discontinuation of immunosuppressive therapy after 3 months in patients who remain on dialysis and who do not have any extrarenal manifestations of disease.		Maintenance of remission should be continued for a period of 24–48 months.

People living with severe renal involvement who remain dialysis dependent have a high risk of infection. Patients with renal limited disease who remain dialysis dependent may not require ongoing immunotherapy. Maintenance of remission therapy to prevent relapse should be balanced against the risks of immunosuppression.
Remission Maintenance During GC TreatmentThe duration of GC therapy for GPA/MPA should be guided by the patient’s clinical condition, preferences, and values.Not addressed.Not addressed.The optimum length of treatment with GC during the maintenance phase is uncertain. Depending on concurrent immunosuppression, complete GC withdrawal may be possible within 6–12 months following induction of remission.
Timing of Kidney TransplantFor patients with GPA/MPA in remission and stage 5 chronic kidney disease, we conditionally recommend evaluation for renal transplantation.Not addressed.Delay transplantation until patients are in complete clinical remission for ≥6 months. The persistence of ANCA should not delay transplantation.People living with GPA or MPA should be in stable clinical remission for at least 6–12 months prior to receiving a kidney transplant.

This concludes our side-by-side comparison on ANCA-associated vasculitis guidelines. Don’t forget to sign up for alerts to stay informed on the latest published guidelines and articles.

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