Central nervous system vasculitis treatment strategies still lack strong data
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Current treatment strategies for patients with central nervous system vasculitis are not based on high-grade scientific evidence but rather expert opinion, according to a speaker at the Biologic Therapies Summit.
“The treatment strategy is based on case series, retrospective studies and expert opinion,” Rula Hajj-Ali, MD, of the Cleveland Clinic, told attendees at the hybrid meeting. “There is still a big need for classification criteria, diagnostic recommendations, universally accepted disease subcategories and validated disease activity assessment tools specifically for central nervous system (CNS) vasculitis.”
Once a diagnosis of CNS vasculitis has been made, the first-line agents many rheumatologists choose is high-dose glucocorticoids, according to Hajj-Ali. If patients do not do well after induction therapy with glucocorticoids, she recommended further investigation of the diagnosis.
“If you diagnose a patient, whether it is possible or definitive, with CNS vasculitis, and you put them into induction therapy and they don’t do well, revisit the diagnosis, because this could be something else,” Hajj-Ali said. “If they do well, use [glucocorticoids] for about 6 months and then maintain them using some kind of maintenance therapy.”
Choosing a secondary therapy comes down to several variables, including disease burden, how severe a patient’s neurological impairments are, how certain the diagnosis is, and the pathologic pattern, she added.
Regarding secondary therapeutic agents, mycophenolate can be considered when there is mild disease burden with “non-necrotizing and non-granulomatous pattern on pathology,” she said. However, Hajj-Ali cautioned that there should be a high degree of confidence in the diagnosis for patients being considered to receive mycophenolate. In certain cases, rituximab (Rituxan, Genentech) may be considered, but more studies are needed to definitively include it, she said.
Once patients using glucocorticoids achieve remission, they should be moved to maintenance therapy consisting of azathioprine or mycophenolate mofetil, according to Hajj-Ali.
Meanwhile, in cases where there is granulomatous angiitis and necrotizing vasculitis, cyclophosphamide is the therapeutic of choice, she added.
“Cyclophosphamide should be the induction agent in granulomatous angiitis and necrotizing vasculitis,” Hajj-Ali said. “Maintenance therapy is recommended, mainly with of azathioprine or mycophenolate mofetil.
“High-dose glucocorticoids are the mainstay of treatment,” she added. “Validated classification criteria, as well recommendation for treatment and diagnosis are very highly needed.”
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Hajj-Ali, R. Update on CNS vasculitis. Presented at: Biologic Therapies Summit X; May 11-13, 2023 (hybrid meeting).
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