New KDIGO Guideline Issued for Managing ANCA-Associated Vasculitis – Renal and Urology News

The Kidney Disease: Improving Global Outcomes organization has published a new guideline for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

The 46-page document, published in a supplement to Kidney International, includes recommendations and practice points based on the latest data gathered from literature searches conducted in July 2022 and updated in April 2023.

“It is designed to assist decision-making. It is not intended to define a standard of care and should not be interpreted as prescribing an exclusive course of management,” the guideline authors wrote. “Variations in practice will inevitably and appropriately occur when clinicians consider the needs of individual patients, available resources, and limitations unique to an institution or type of practice. Healthcare professionals using these recommendations should decide how to apply them to their own clinical practice.”

In cases involving clinical presentation compatible with small-vessel vasculitis combined 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.”

In cases of suspected kidney vasculitis, patients with a clinical presentation compatible with AAV and who are MPO- or PR3-ANCA positive with a low suspicion for secondary vasculitis should be referred to a center experienced in AAV management. Treatment should be initiated and a biopsy should be performed after starting treatment when feasible. When clinical presentation is compatible with any primary small-vessel vasculitis and the patient is MPO- and PR3-ANCA negative, a biopsy should be performed if there is no contraindication to the procedure.

In a practice point, the guideline states that “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.”

The guideline recommends that glucocorticoids combined with rituximab or cyclophosphamide be used as initial treatment for new-onset AAV.

The guideline includes a practice treatment algorithm for AAV with kidney involvement. Diagnosis of AAV should be followed by disease assessment and induction of remission. If there is no organ-threatening involvement, clinicians can use either rituximab plus glucocorticoid taper or avacopan or cyclophosphamide plus glucocorticoid taper or avacopan. For patients with vital organ/life-threatening serum creatinine levels higher than 3.4 mg/dL, the algorithm recommends rituximab plus cyclophosphamide or cyclophosphamide plus glucocorticoid taper or avacopan. Plasma exchange can be considered.

For maintenance therapy following “on drug” remission, the algorithm suggests either continuing rituximab and then stopping it when remission is achieved or switching to azathioprine and tapering glucocorticoids and then tapering azathioprine until remission occurs.

According to the guideline, rituximab is preferred for children and adolescents; premenopausal women and men concerned about fertility; frail older adults; relapsing disease; PR3-ANCA disease; and when glucocorticoid-sparing is particularly important. Cyclophosphamide is preferred when rituximab is difficult to access or in cases of severe glomerulonephritis (serum creatinine levels above 4 mg/dL).