The soon-to-be published vasculitis guidelines are the first ever to be produced and endorsed by the ACR and the Vasculitis Foundation. (Note: The guidelines should be available in Early View in June.) Although most of the recommendations are conditional, due to the relative rarity of these conditions and correspondingly limited evidence base, these guidelines will provide clinicians with comprehensive information to help them make informed treatment choices with pediatric and adult vasculitis patients.
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Explore This IssueMay 2021
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Needs & Challenges
Sharon Chung, MD, MAS, director of the vasculitis clinic at the University of California, San Francisco, served as the principal investigator of the overall vasculitis guideline effort. “In the past, we have essentially had prednisone and one other treatment for severe vasculitis—cyclophosphamide,” Dr. Chung points out. Other conventional immunosuppressive agents, such as methotrexate and azathioprine, have also been options for some vasculitides.
“More recently, we’ve had additional treatment options develop,” she adds. “Thus, the goal of the guidelines is twofold: to provide initial recommendations to rheumatologists and other care providers, as well as to provide guidance in choosing among the increasing options that we have.”
Given that newer biologic agents are now available, practitioners have had questions about how these different treatment options should be used in different clinical situations.
Carol Langford, MD, MHS, director of the Center for Vasculitis Care and Research at the Cleveland Clinic, Ohio, is one of the lead authors of the guideline focused on the management of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. She points out the ACR has targeted guideline development as an important initiative to both serve clinicians and improve patient care. “We had heard from a number of clinicians requesting that vasculitis guidelines be developed, given that these are uncommon diseases in which there can be complex therapeutic questions,” she says.
One of the inherent challenges of the project was its breadth. Vasculitis is a histopathological term indicating blood vessel inflammation, which can be seen in a wide variety of medical settings. “This is not like a guideline where you focus on one disease,” says Dr. Chung. “Many different diseases fall under the category of vasculitis, and many of them are treated very differently.” Many different disease entities are included, even if one is only considering the primary forms of vasculitis.
The manifestations can be quite different even within a specific disease. Dr. Chung provides the example of granulomatosis with polyangiitis (GPA): “There are patients with lung involvement, patients with kidney involvement, patients with sinus involvement. Thus, making recommendations for the group as a whole can be challenging, because certain therapies may benefit some more than others.”
A related challenge was the evidence base used to form the recommendations. Because of the rarity of these syndromes, it has been more difficult to conduct randomized, double-blind, controlled trials, limiting the availability of high-quality published evidence.