Guideline for managing ANCA-associated vasculitis gets update

New recommendations are designed for AAV associated with kidney damage

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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The Kidney Disease: Improving Global Outcomes (KDIGO) work group has released an updated practice guideline based on developments in how best to manage ANCA-associated vasculitis (AAV) associated with inflammation or damage to the kidneys.

The guideline now offers advice on the use of lower doses of glucocorticoids or an alternative, the recently approved Tavneos (avacopan), with the goal of achieving remission, or no symptoms appearing for a time, while reducing side effects.

No major changes have been made to recommendations and practice points on how to diagnose AAV, predict its outcomes, or maintain the disease in remission.

“We anticipate that the comprehensive research, clinical perspectives, and collaborative expertise reflected in this guideline will contribute to better outcomes,” Jürgen Floege, MD, a professor at the University of Aachen in Germany who co-chaired the guideline group, said in a press release.

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A quick reference to the most important guideline changes is in “Executive summary of the KDIGO 2024 Clinical Practice Guideline for the Management of ANCA-Associated Vasculitis,” published in Kidney International.

AAV causes inflammation and damage to small blood vessels. Over time, that damage can become irreversible and lead to scarring and tissue death. When that happens in the kidneys, they may no longer function well on their own, resulting in kidney failure.

KDIGO, a nonprofit organization, regularly brings together a group of kidney specialists to reach consensus on evidence-based recommendations and practice points for managing kidney disease.

KDIGO is now updating its 2021 Clinical Practice Guideline for the Management of Glomerular Diseases on an ongoing, chapter-by-chapter basis, building upon new advances to keep it as current as possible.

The update to the AAV chapter was mainly prompted by the approval of Tavneos as an add-on treatment for adults with severe granulomatosis with polyangiitis or microscopic polyangiitis, two types of AAV, in the U.S. and the European Union.

New treatment guidance

That chapter covers the entire patient journey, from clinical suspicion and diagnosis to treatments to push the disease into remission and maintain that status. It also covers relapses, where symptoms return after a period of remission, and treatment-resistant disease. 

While the main recommendation on how to start treatment for new-onset AAV remains the same — using glucocorticoids with either cyclophosphamide or rituximab (often sold as Rituxan) — there’s now a stronger suggestion to taper glucocorticoids more quickly.

That recommendation comes in response to growing evidence on the use of lower doses of glucocorticoids to avoid side effects, such as weight gain and mood changes, knowing that lower doses of glucocorticoids given alongside rituximab work just as well as higher doses for long-term disease control.

In patients with markedly reduced or rapidly declining kidney function, a combination of cyclophosphamide and rituximab (or cyclophosphamide and tapering glucocorticoids) can be considered. 

While routine plasma exchange, a blood-cleaning procedure, is not recommended for patients with impaired kidney function, its use can be considered in more severe presentations. However, “it comes at the cost of an increased risk of serious infections,” the team wrote.

Tavneos is now included as a treatment option for AAV as part of a combined treatment regimen with either cyclophosphamide or rituximab. The team added this recommendation based on two randomized controlled trials (RCTs).

Those RCTs showed that Tavneos may help patients enter remission and even reverse severe kidney disease associated with AAV, without the harmful side effects of glucocorticoids. Patients more sensitive to those medicines are most likely to benefit from Tavneos.

While this result “is obviously desirable, given the short- and long-term complications of glucocorticoids, it is less clear which patients need [Tavneos],” the team wrote, noting that Tavneos “adds significant cost to treatment, and long-term safety data are currently lacking.”

The main recommendation on how to maintain remission in AAV remains the same: using either rituximab or a combination of azathioprine and low-dose glucocorticoids. But the updated guideline now suggests an optimal treatment duration of 18 months to four years for either option.

The guideline also underscores the need for tailored approaches to treatment, considering disease severity, relapse risk, and whether other medical conditions are present. It recommends rituximab as first-line treatment for certain patients.

“We are better equipped than ever to tailor interventions, optimize patient care, and strive towards improved outcomes,” said co-chair Brad Rovin, MD, from the Ohio State University College of Medicine in Columbus.

But recommendations “still call for RCTs that incorporate patient-reported outcomes, more prolonged long-term outcome studies, studies aimed at defining the role of rituximab in severe AAV, and studies conducted in ethnically diverse populations,” the team wrote.

“Another area in which there is a large unmet need is the identification of biomarkers to better guide treatment. Hopefully, these future trials, if successful, will lead to yet another update of the KDIGO AAV guideline in the near-term,” they wrote.