ACR, Vasculitis Foundation issue three new guidelines for 'expanded' vasculitis therapies
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The American College of Rheumatology has released three new vasculitis treatment guidelines — for ANCA-associated vasculitis, giant cell arteritis and Takayasu arteritis, and polyarteritis nodosa — with a fourth expected soon.
“Many rheumatologists may have limited experience caring for patients with these diseases,” Sharon A. Chung, MD, MAS, director of the vasculitis clinic at the University of California, San Francisco, and the lead investigator of the guidelines, said in a press release. “However, the treatment options for patients with vasculitis have expanded in recent years. Thus, these guidelines provide practitioners evidence-based recommendations to help navigate the treatment path for their patients.”
All three of the guidelines were developed in partnership with the Vasculitis Foundation and published in the ACR’s Arthritis & Rheumatology. A fourth guideline, on the treatment of Kawasaki disease, will be released in the coming weeks, according to the ACR. Altogether, they are the first guidelines produced and endorsed by both the ACR and the Vasculitis Foundation.
Similar to other ACR guidelines, the new vasculitis documents were developed using Grading of Recommendations Assessment, Development and Evaluation(GRADE) methodology, which creates rigorous standards for judging the quality of the literature available and assigns strengths to the recommendations, the press release said. However, because they deal with rare diseases, most of the recommendations are conditional. Documents containing the full list of recommendations, conditions and supporting evidence are available at the ACR vasculitis guidelines page.
The guideline for GCA and Takayasu arteritis includes 42 recommendations and three ungraded position statements. The recommendations and statements address clinical questions relating to the use of diagnostic testing and imaging, treatments and surgical interventions in both diseases.
The recommendations for GCA support the use of glucocorticoid-sparing immunosuppressive agents, as well as imaging to identify large vessel involvement. Recommendations for Takayasu arteritis include the use of non-glucocorticoid immunosuppressive agents with glucocorticoids as initial therapy.
The guideline for ANCA-associated vasculitis features 41 recommendations and 10 ungraded position statements for granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The recommendations cover remission induction and maintenance therapy as well as adjunctive treatment strategies in GPA, MPA and EGPA. They support the use of rituximab (Rituxan; Genentech, Biogen) for remission induction and maintenance in severe GPA and MPA, and the use of mepolizumab (Nucala, GlaxoSmithKline) in non-severe EGPA.
Lastly, the guideline for polyarteritis nodosa provides 16 recommendations and one ungraded position statement. The recommendations support early treatment of severe disease with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, as well as imaging and tissue biopsy for diagnosis. They also endorse established therapy at the start of the disease to minimize risk to the patient and identify new areas where adjunctive therapy may be warranted, according to the press release.
“Different forms of vasculitis can have similar symptoms and treatment regimens; however, each disease is distinct,” Joyce Kullman, executive director of the Vasculitis Foundation, said in the release. “These guidelines will hopefully take some of the guesswork out of determining which treatments might work best for newly diagnosed patients, or patients who have been under treatment for a while without success.”
However, both the ACR and Vasculitis Foundation cautioned that there is still much unknown about vasculitis. Chung added that she hopes the guidelines act to shine a light on what work still needs to be done.
“We identified knowledge gaps that would benefit from additional research, like comparative effectiveness trials, longitudinal studies of imaging modalities, and identification of biomarkers to inform disease activity assessments,” Chung said in the release. “We hope this fuels research in these areas to facilitate patient care.”
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ACR press release
Chung SA, et al. Arthritis Rheumatol. 2021;doi:10.1002/art.41773.
Maz M, et al. Arthritis Rheumatol. 2021;doi:10.1002/art.41774.
Chung SA, et al. Arthritis Rheumatol. 2021;doi:10.1002/art.41776.
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