Neurocognitive Complications of Lupus: A Multidisciplinary Program for Advanced Diagnostics, Management, and Clinical Research
Douglas Wilcox1, Erin Mathieu1, Matthew Baum2, Emma Weizenbaum1, Jonathan Zurawski1, Rohit Bakshi1, Karen Costenbader3, Shamik Bhattacharyya1
1Neurology, 2Psychiatry, 3Rheumatology, Brigham and Women's Hospital
Objective:

To develop a comprehensive Neuro-Lupus Program that integrates advanced second-tier diagnostics, facilitates multidisciplinary care, and enables outcomes-based research to improve the evaluation and management of patients with neuropsychiatric lupus.

Background:
Neurocognitive symptoms occur in a majority of patients with systemic lupus erythematosus (SLE) and can be very functionally limiting. There is currently no clear standardized, multidisciplinary approach for the evaluation and management of these patients.
Design/Methods:

Patients with a diagnosis of SLE and any neurocognitive symptoms can be referred to the Neuro-Lupus Program, where they are evaluated by a dedicated lupus neurologist followed by a comprehensive neurocognitive evaluation by a clinical neuropsychologist. Patients requiring complex neuropsychiatric evaluation or management (e.g. concern for psychosis) are evaluated in a specialized autoimmune psychiatry clinic. High-resolution 7T MRI scans are performed to evaluate for subtle inflammatory changes. Patients with abnormalities on MRI or cognitive testing are further assessed with CSF studies and FDG PET-CT brain. Management of neuropsychiatric medications and immunotherapy is a collaboration between neurology, psychiatry, and rheumatology. 

Results:

We evaluated 50 patients with lupus and neurocognitive concerns in a 12-month period. Full neurocognitive evaluation revealed previously undiagnosed cognitive impairment in the majority (88%) of patients. 7T MRI brain scans demonstrated previously undescribed leptomeningeal enhancement in 31% of patients. PET scan was performed in 12% of patients, 83% of which were abnormal (10% of total cohort). No CSF studies had pleocytosis or elevated protein. In shared management with psychiatry, a neuro-psychiatric medication was started in 70% of patients. As a result of this comprehensive evaluation, 8% of patients had an escalation in immunotherapy.

Conclusions:

Our multidisciplinary Neuro-Lupus Program brings together neurologists, psychiatrists, rheumatologists, clinical neuropsychologists, and radiologists to better evaluate, manage, and understand the complex impact of neurocognitive symptoms in SLE. This program resulted in improved management of previously undiagnosed cognitive impairment and markedly impacted immunotherapy decisions.

10.1212/WNL.0000000000215504
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