Lacking options, clinicians still 'throw the kitchen sink' at ANCA-associated vasculitis
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Despite an ever-growing array of therapeutic options, patients with antineutrophil cytoplasmic antibody-associated vasculitis often fail to achieve remission, according to a speaker at the ACR State-of-the-Art Clinical Symposium.
While ANCA-associated vasculitis can be described, in short, as damage to small blood vessels, there are a “whole slew” of different manifestations, according to Anisha Dua, MD, MPH, director of the Vasculitis Center at the University of Northwestern Feinberg School of Medicine.
The skin may be involved, as may the heart, kidneys, sinuses, the eyes, ears and nervous system. “The severity of organs involved really dictates the management strategies you are going to employ,” Dua said.
Corticosteroids and cyclophosphamide were the mainstays of treatment until recently. In the last 10 years, however, the clinical and research communities have gained understanding to improve treatments to prevent relapses and minimize toxicity.
Dua offered a case presentation of a 28-year-old woman diagnosed with severe microscopic polyangiitis. “What do you do?” she asked, noting that the patient “is rapidly deteriorating in front of your eyes.”
At that point, Dua suggested that clinicians should “throw the kitchen sink” at this patient.
“The first thing to think about is pulse steroids,” she said. “We often use those before we even get labs and serology back.”
Dua acknowledged, however, the risks associated with steroids, and noted that the ACR/Vasculitis Foundation guidelines suggest a reduced dose of glucocorticoids in granulomatosis with polyangiitis and microscopic polyangiitis.
The next options are cyclophosphamide or rituximab (Rituxan, Genentech). “The recommendation to use rituximab is based on findings from the RAVE and RITUXIVAST trials,” Dua said.
She added that rituximab can be administered at a 500 mg dose every 6 months as maintenance therapy for approximately 4 years.
Moving away from that specific case patient and looking at other options for patients with various forms of ANCA vasculitis, Dua discussed the possibility of plasmapheresis. She noted that ACR/VF guidelines conditionally recommend against adding plasma exchange to cyclophosphamide or rituximab.
Dua next addressed the use of the complement cascade in ANCA-associated vasculitis. The oral C5a receptor antagonist avacopan (ChemoCentryx) has been shown to reduce neutrophil activation, accumulation and adhesion. It also has beneficial impacts on vascular permeability.
“With increased understanding of the role of complement, we are moving towards a possible steroid sparing agent in ANCA-associated vasculitis,” Dua said.
Another treatment to consider is mepolizumab (Nucala, GlaxoSmithKline), an interleukinIL-5 inhibitor that has shown efficacy in eosinophilic granulomatosis with polyangiitis (EPGA). Dua added that this agent is also “steroid sparing” and has particular efficacy in upper airway and upper respiratory manifestations of EPGA. “We should be using less steroids than we ever have been. Despite all this progress, about 30% of our patients do not achieve remission in 6 months,” Dua concluded.
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Dua A. Updates in ANCA-Associated Vasculitis Management. Presented at: American College of Rheumatology State-of-the-Art Clinical Symposium. April 9-11, 2021 (virtual meeting).
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