Inflammatory central nervous system involvement in rheumatoid arthritis

Area of Science:

• Neurology
• Rheumatology
• Pathology

Background:

• Rheumatoid arthritis (RA) is a systemic autoimmune disease primarily affecting joints.
• Central nervous system (CNS) involvement in RA is rare but can lead to severe neurological deficits.
• Understanding the spectrum of CNS manifestations in RA is critical for timely diagnosis and management.

Observation:

• A case of seropositive rheumatoid arthritis presenting with pachymeningitis and optic atrophy.
• Review of 18 additional cases revealed characteristic neuropathologic findings: rheumatoid nodules, pachymeningitis/leptomeningitis, and vasculitis.
• CNS disease in RA can occur without active synovitis or extracranial manifestations, challenging previous assumptions.

Findings:

• Multiple neuropathologic processes, including nodules, meningitis, and vasculitis, frequently co-exist in RA-associated CNS disease.
• Patients with CNS nodules were more often asymptomatic compared to those with vasculitis or meningitis.
• Cerebrospinal fluid (CSF) analysis and computed axial tomography (CT) aided diagnosis, but biopsy or autopsy confirmed findings.

Implications:

• Inflammatory CNS disease should be considered in RA patients with unexplained neurological symptoms, after excluding infection and malignancy.
• An aggressive diagnostic approach, including invasive biopsies, may be warranted for definitive diagnosis.
• Treatment with corticosteroids and/or surgical decompression can be beneficial in managing these complex cases.