THE OFFICIAL NEWS SOURCE OF ACR CONVERGENCE 2022 • NOVEMBER 10-14



Treatment for ANCA-associated vasculitis evolves

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Peter A. Merkel, MD, MPH

Treatment for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis has changed. What was once an acute, highly lethal disease has become a less lethal, more often chronic condition.

Most patients achieve remission with current treatment regimens, but remission is not permanent. Most patients can expect one or more relapses over the course of their disease.

“Because ANCA-associated vasculitis is now so frequently a relapsing disease, we have new challenges to address with our patients,” said Peter A. Merkel, MD, MPH, Chief of Rheumatology and Professor of Medicine and Epidemiology at the University of Pennsylvania. “The challenges include maintaining remission in a disease in which relapses are frequent. There is also a challenge in knowing in which patients you can stop therapy, because some patients are not destined to relapse, and which patients must continue long-term therapy because they are high risk for relapsing. All of this while managing the acute toxicities from treatment.”

Dr. Merkel will discuss the latest advances in treatment during a clinical symposium on Maintenance Therapy in ANCA-Associated Vasculitis: Evaluation and Treatment of Patients in Remission on Monday morning. As patients live longer with ANCA-associated vasculitis, management involves addressing the longer-term complications from both the disease and the agents used to treat it.

Patients over time may develop chronic renal insufficiency as well as upper airway disease, he said. Both conditions require management even if they are not progressing. Typical complications from treatment include all the familiar problems associated with long-term glucocorticoid use as well as increased risk of infection.

One of the management issues just now starting to be understood is the multitude of different pathways that patients in remission may follow. Each pathway produces its own set of complications. Some pathways lead to more durable remission, while others seem to set the stage for the next relapse.

“We are entering an era when we will have increasingly precise tools to evaluate each patient,” Dr. Merkel said. “We will be able to look at a patient’s clinical course, laboratory markers, and perhaps genetic markers and individually tailor the appropriate maintenance regimen, whether that is treatment with a specific agent or no treatment at all.”

Clinicians already have the ability to implement data-driven strategies that can reliably deliver good decisions during remission. For now, the availability of rituximab, in addition to the traditional mainstays of treatment, azathioprine and methotrexate, offer multiple approaches to help prolong remission and minimize periods of relapse. The symposium will review the current understanding of management during remission, the options for therapy to maintain remission, current precision medicine strategies, and the state of current research efforts.

“The vasculitis research community, through collaborative, multicenter, randomized trials, is generating excellent data to help clinicians and patients make more informed decisions,” he said. “Decisions regarding remission and maintenance of remission are the most pressing questions for our patients today. We now have good data to guide us, and better data are on the way.”

Dr. Merkel is co-principal investigator for the international, multicenter RITAZAREM trial designed to provide some of those better answers. The study is powered to compare the efficacy of rituximab to azathioprine for maintenance of remission as well as to study the long-term effects of repeated treatments with rituximab.

“ANCA-associated vasculitis is disproportionately of interest to clinicians,” he said. “We all see it, and it is hard to treat. What to do during remission to maintain remission is the question I get several times a week from clinicians around the country. This symposium will present the data to date in a practical format that is geared towards clinical practice.”