Updated ANCA Vasculitis Guideline Aims to Improve Care

TOPLINE:

A new guideline from Kidney Disease: Improving Global Outcomes (KDIGO) updates recommendations on the management of antineutrophilic cytoplasmic antibody (ANCA)–associated vasculitis (AAV) to address key recent developments, most importantly the recent regulatory approval of the C5a receptor inhibitor avacopan as an add-on therapy to the standard-of-care.

The recommendations offer guidance on the use of avacopan as an alternative to glucocorticoids, while also addressing new evidence on the use of lower doses of corticosteroids.

Further recommendations address therapies including plasma exchange for use in patients with advanced kidney disease.

METHODOLOGY:

• In 2021, KDIGO published Clinical Practice Guidelines for Glomerular Diseases, which summarized recommendations for 11 diseases based on all available evidence through June 2020.
• The new recommendations are based on the latest evidence from randomized controlled trials, published since July 2022.
• The updates reflect the “unprecedented pace of scientific discovery in the field and centers on guidance regarding the diagnosis, treatment and monitoring of kidney development in ANCA-associated vasculitis,” according to an executive summary published with the report.
• Recommendations are based on a systematic review of the evidence and are graded for strength of the recommendation and certainty of the evidence.
• Practice points, which are issued when there has not been a systematic review, are ungraded, consensus-based statements, representing the judgement of the KDIGO Work Group.

TAKEAWAY:

• The most important update, according to the executive summary, relates to induction therapy, with a recommendation for a more rapid reduction of the glucocorticoid dose when used in combination with cyclophosphamide or rituximab in the initial treatment of new-onset AAV.
• The guideline notes that data show patients most likely to benefit from avacopan are those with an increased risk for glucocorticoid toxicity.
• Further evidence shows greater recovery of low glomerular filtration rate (GFR) with avacopan compared with glucocorticoid therapy.
• The guideline recommends against the routine use of plasma exchange for patients presenting with a GFR < 50 mL/min/1.73 m², but it can be considered in patients with more severe presentations, or with alveolar hemorrhage and hypoxemia, who have high mortality rates.
• Plasma exchange is not required for therapy of diffuse alveolar hemorrhage in the absence of hypoxemia.
• Plasma exchange is recommended for patients with concomitant anti–glomerular basement membrane disease.
• No major changes have been made in recommendations regarding diagnosis and assessment of prognosis of AAV or regarding maintenance therapy.
• The guideline also has no new clinical recommendations for AAV patients who have relapsing or refractory disease or who have had kidney transplantation.

IN PRACTICE:

“The ANCA Vasculitis Guideline includes significant updates aimed at enhancing clinical decision-making, integrating new treatments with established clinical practices,” said Co-chair Jurgen Floege, MD, senior professor in the Division of Nephrology and Immunology at the University of Aachen, Aachen, Germany, in a press statement.

“We anticipate that the comprehensive research, clinical perspectives, and collaborative expertise reflected in this guideline will contribute to better outcomes for individuals grappling with this complex condition,” he said.

Co-chair Brad Rovin, MD, the Lee A. Hebert Professor of Nephrology at The Ohio State University Wexner Medical Center, Columbus, Ohio, added that “we are excited to release the KDIGO 2024 ANCA Vasculitis Guideline, which offers new guidance for managing the intricacies of ANCA vasculitis treatment, particularly with the emergence of innovative therapies.”

SOURCE:

The practice guideline was published in the March 2024 edition of Kidney International.

LIMITATIONS:

Randomized controlled trials were prioritized as the primary source of evidence. “In the development of these guidelines, no scoping exercise with patients, limited searches of the qualitative literature, or formal qualitative evidence synthesis examining patient experiences and priorities were undertaken,” the authors noted. While resources were considered in developing the recommendations, “formal economic evaluations were not undertaken for all topics.”

DISCLOSURES:

The authors’ disclosures were detailed in the published report.