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.

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.