Expanding the Therapeutic Horizon in CAA-related Inflammation: A Case Series of Rituximab Treatment
Roxana Bahani1, Veronica Moreno Gomez1, Aaron Shoskes1, Yarden Bornovski1, Stephanie Lyden1
1Neurology, University of Utah Health
Objective:
The use of rituximab is currently considered a second-line treatment for refractory or recurrent cases of cerebral amyloid angiopathy-related inflammation (CAA-ri), but its use remains uncommon and not well established. Presenting three cases of patients treated with rituximab may provide additional data to guide clinicians on treatment selection for this rare condition.
Background:
CAA-ri is a treatable, distinct subtype of cerebral amyloid angiopathy characterized by an inflammatory response to amyloid β-peptides accumulated within the vasculature of the brain and leptomeninges. This disease clinically manifests as an assortment of acute to subacute neurologic symptoms such as changes in cognition, alterations in consciousness, focal neurologic deficits, headaches, or seizures. Diagnosis is established in symptomatic patients who have MRI findings characteristic of the disease process and only after other possible causes have been excluded through extensive evaluation. Patients are treated with immunosuppressive therapy, first-line being high-dose intravenous corticosteroids followed by a gradual oral taper, though alternative agents such as rituximab have shown a promising response in select cases.
Design/Methods:
A retrospective chart review was conducted of three patients diagnosed with probable or definite CAA-ri who received rituximab for refractory disease between 2023 and 2025. Clinical presentation, MRI findings, treatment regimens, adverse effects, and short-term outcomes were reviewed.
Results:
All three patients initially improved with corticosteroids but experienced relapses, prompting initiation of rituximab. Each patient subsequently showed improvement or stabilization in both clinical symptoms and imaging findings. Two patients developed mild rash following rituximab infusion, but therapy was otherwise well tolerated. Long-term outcomes remain limited.
Conclusions:
Rituximab may be considered a therapeutic option for steroid-refractory CAA-ri. Its targeted immunosuppressive mechanism may reduce relapse risk and systemic side effects. Further studies are needed to clarify the long-term efficacy and safety of rituximab, as well as other immunosuppressive agents for treatment of CAA-ri.
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