New Guidelines May Help Take Guesswork Out of Treating AAV

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by Forest Ray PhD |

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The American College of Rheumatology and the Vasculitis Foundation have published new guidelines to improve the treatment and management of systemic vasculitis, a group of inflammatory disorders that includes ANCA-associated vasculitis (AAV).

The rarity of these disorders can make them challenging to diagnose and treat. The new guidelines — the first created and endorsed by both organizations — are meant to help physicians better understand and treat vasculitis.

“Many rheumatologists may have limited experience caring for patients with these diseases,” Sharon Chung, MD, 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,” she added. “Thus, these guidelines provide practitioners evidence-based recommendations to help navigate the treatment path for their patients.”

For AAV, the two groups provided 41 recommendations and 10 position statements for granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

The recommendations include guidance on inducing remission, maintenance therapy, and add-on treatment strategies for all three AAV subtypes.

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“Different forms of vasculitis can have similar symptoms and treatment regimens; however, each disease is distinct,” said Joyce Kullman, executive director of the Vasculitis Foundation. “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.”

To induce remission in patients with active, severe GPA or MPA, the researchers conditionally recommend rituximab (sold as Rituxan, among others) over cyclophosphamide, as rituximab is generally less toxic.

However, they say that cyclophosphamide may still be preferred when rituximab must be avoided or when a patient has active disease despite taking rituximab. They also acknowledge that it remains controversial whether cyclophosphamide should be favored in certain types of severe disease, and that there is limited data about the efficacy of both medications combined.

For patients with severe and active EGPA, on the other hand, the groups recommend initially using either intravenous pulse glucocorticoids or high-dose oral glucocorticoids, combined with rituximab or cyclophosphamide, to induce remission.

In their recommendations for maintenance therapy, the researchers conditionally recommend that individuals with severe GPA or MPA, who enter remission following treatment by rituximab or cyclophosphamide, use rituximab rather than methotrexate or azathioprine, as it associates with a lower relapse rate.

People with severe EGPA, however, in whom cyclophosphamide has induced remission, might favor methotrexate, azathioprine, or mycophenolate mofetil over rituximab as a maintenance therapy.

All recommendations are conditional, depending on the specifics of a given case, as the evidence for a recommendation is not strong in every setting, and an alternative may be reasonable, given a patient’s specific conditions.

“Although these recommendations provide a general guide for disease management, the patient’s clinical condition, preferences, and values should influence their treatment,” the researchers wrote.

They point out that their recommendations should not be used to restrict access to any therapy or to require that someone use certain therapies before others.

“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. “We hope this fuels research in these areas to facilitate patient care.”