A Multidisciplinary Neuro-Lupus Program for the Evaluation and Management of Patients With SLE and Cognitive Concerns

To develop and implement a standardized, comprehensive multidisciplinary program for the evaluation and management of patients with systemic lupus erythematosus (SLE) and cognitive concerns that would lead to improved diagnosis and management.

The Neuro-Lupus Program evaluated 50 patients with SLE referred for any neurocognitive concern over a 12-month period. A specific sequential work-up protocol was developed for standardized patient evaluation. Initial history and examination were performed by a dedicated lupus neurologist followed by referral for comprehensive neurocognitive testing. Patients with suspected mood or psychotic disorders were evaluated in a specialized autoimmune psychiatry clinic. MRI brain scans were performed in all patients. Patients with MRI evidence of inflammation or cognitive impairment were further assessed with CSF studies and FDG-PET/CT brain. Management of neuropsychiatric medications and immunotherapy was a collaboration between neurology, psychiatry, and rheumatology.

Full neurocognitive evaluation revealed a previously undiagnosed cognitive impairment in 46.4% of patients tested (mild 39.3%, major 7.1%), with an additional 39.3% demonstrating a normal cognitive profile with isolated weakness. MRI demonstrated white matter disease in 50% of patients and cerebral atrophy in 10%. Brain FDG-PET/CT was performed in 8 patients (16%), 7 (88%) of which were abnormal. CSF studies demonstrated normal protein and white cell counts, but 25% had elevated kappa free light chains. As a result of this program, neuropsychiatric medications were optimized in 86% of patients and 8% had an escalation in immunotherapy.

This multidisciplinary Neuro-Lupus Program model brings together neurologists, psychiatrists, rheumatologists, and neuropsychologists to standardize the approach to neurocognitive symptoms in SLE. Among the patients enrolled, this program detected previously undiagnosed cognitive impairment in the majority of those tested and was associated with changes in neuropsychiatric and immunotherapy management.