Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis

Publication Date: February 19, 2024
Last Updated: May 13, 2024

Diagnosis and Prognosis

Practice Point 9.1.1

In the case of a clinical presentation compatible with small-vessel vasculitis in combination with positive myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA serology, waiting for a kidney biopsy to be performed or reported should not delay starting immunosuppressive therapy, especially in patients who are rapidly deteriorating (Figure 1). (, )
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Practice Point 9.1.2

Patients with ANCA-associated vasculitis (AAV) should be treated at centers with experience in AAV management. (, )
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Practice Point 9.2.3.1

The persistence of ANCA positivity, an increase in ANCA levels, or a change in ANCA from negative to positive may be predictive of future disease relapse and should be considered when making treatment decisions. (, )
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Treatment

Recommendation 9.3.1.1

We recommend that glucocorticoids in combination with rituximab or cyclophosphamide be used as initial treatment of new-onset AAV. (1, B)
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Practice Point 9.3.1.1

A practical treatment algorithm for AAV with kidney involvement is given in Figure 6. (, )
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Practice Point 9.3.1.2

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. (, )
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Practice Point 9.3.1.3

Considerations for choosing between rituximab and cyclophosphamide for induction therapy are given in Figure 7. (, )
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Practice Point 9.3.1.4

Considerations for choosing the route of administration of cyclophosphamide are given in Figure 8. (, )
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Practice Point 9.3.1.5

Consider discontinuation of immunosuppressive therapy after 3 months in patients who remain on dialysis and who do not have any extrarenal manifestations of disease. (, )
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Practice Point 9.3.1.6

Recommendations for oral glucocorticoid tapering are given in Figure 9. (, )
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Practice Point 9.3.1.7

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. (, )
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Practice Point 9.3.1.8

Recommendations for immunosuppressive dosing are given in Figure 10. (, )
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Practice Point 9.3.1.9

Consider plasma exchange for patients with SCr >3.4 mg/dl (>300 mmol/l), patients requiring dialysis or with rapidly increasing SCr, or patients with diffuse alveolar hemorrhage who have hypoxemia. (, )
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Practice Point 9.3.1.10

Add plasma exchange for patients with an overlap syndrome of ANCA-associated vasculitis and anti-glomerular basement membrane (GBM). (, )
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Maintenance Therapy

Recommendation 9.3.2.1

We recommend maintenance therapy with either rituximab, or azathioprine and low-dose glucocorticoids after induction of remission. (1, C)
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Practice Point 9.3.2.1

Following rituximab induction, maintenance immunosuppressive therapy should be given to most patients. (, )
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Practice Point 9.3.2.2

The optimal duration of remission therapy is between 18 months and 4 years after induction of remission. (, )
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Practice Point 9.3.2.3

When considering withdrawal of maintenance therapy, the risk of relapse should be considered, and patients should be informed of the need for prompt attention if symptoms recur (Figure 12). (, )
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Practice Point 9.3.2.4

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. (, )
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Practice Point 9.3.2.5

Considerations for choosing rituximab or azathioprine for maintenance therapy are presented in Figure 13. (, )
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Practice Point 9.3.2.6

Recommendations for dosing and duration of maintenance therapy are given in Figure 14. (, )
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Practice Point 9.3.3.1

Patients with relapsing disease should be reinduced (Recommendation 9.3.1.1.), preferably with rituximab. (, )
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Practice Point 9.4.1.1

Refractory disease can be treated by an increase in glucocorticoids (intravenous or oral), by the addition of rituximab if cyclophosphamide induction had been used previously, or vice versa. Plasma exchange can be considered. (, )
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Practice Point 9.4.1.2

In the setting of diffuse alveolar bleeding with hypoxemia, plasma exchange can be considered in addition to glucocorticoids with either cyclophosphamide or rituximab. (, )
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Practice Point 9.4.2.1

Delay transplantation until patients are in complete clinical remission for ≥6 months. The persistence of ANCA should not delay transplantation. (, )
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Recommendation Grading

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.

Overview

Title

Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis

Authoring Organization

Kidney Disease Improving Global Outcomes

Publication Month/Year

February 19, 2024

Last Updated Month/Year

April 1, 2024

Supplemental Implementation Tools

Document Type

Guideline

Country of Publication

Global

Document Objectives

The Kidney Disease: Improving Global Outcomes (KDIGO) 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis represents a focused update of the ANCA-Associated Vasculitis chapter from the KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. The aim is to assist clinicians caring for people with ANCA-associated vasculitis. The update takes into consideration evidence from randomized controlled trials published since February 2022. As in 2021, the chapter follows the same template providing guidance related to diagnosis, prognosis, treatment, and special situations. Based on the evidence, this update is mostly focused on guidance related to treatment of ANCA-associated vasculitis. Development of this guideline followed an explicit process of evidence review and appraisal. Treatment approaches and guideline recommendations are based on systematic reviews of relevant studies, and appraisal of the certainty of the evidence and the strength of recommendations followed the “Grading of Recommendations Assessment, Development and Evaluation” (GRADE) approach. Limitations of the evidence are discussed and areas of future research are also presented.

Inclusion Criteria

Male, Female, Adult, Older adult

Health Care Settings

Ambulatory, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Assessment and screening, Treatment, Management

Diseases/Conditions (MeSH)

D056648 - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, D014657 - Vasculitis

Keywords

glomerulonephritis, nephrotic syndrome, vasculitis, glomerular diseases, antineutrophil cytoplasmic antibody-associated vasculitis, ANCA–Associated Vasculitis

Source Citation

Jürgen Floege, David R.W. Jayne, Jan-Stephan F. Sanders, Vladimír Tesar, Brad H. Rovin, KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)–Associated Vasculitis, Kidney International, Volume 105, Issue 3, Supplement, 2024, Pages S71-S116, ISSN 0085-2538, https://doi.org/10.1016/j.kint.2023.10.008.

Supplemental Methodology Resources

Methodology Supplement, Data Supplement

Methodology

Number of Source Documents
86
Literature Search Start Date
July 6, 2022
Literature Search End Date
April 24, 2023