Clinical manifestations and treatment of rheumatoid pachymeningitis

Rheumatoid pachymeningitis is a rare CNS complication of rheumatoid arthritis (RA). This occurs in patients with long-standing seropositive RA with extra-articular involvement (ExRA). We reviewed the literature searching PubMed from 1971 to present using terms the “rheumatoid arthritis” and “pachymeningitis.” References from identified articles were also selected. Only 20 histopathologically proven cases have been reported. The prevalence of this disorder is unknown. We describe two patients who met American College of Rheumatology criteria for RA, with biopsy-proven pachymeningitis. ### Patient 1. A 67-year-old white man without a history of arthritis presented with acute, sensorineural hearing loss secondary to pachymeningitis confirmed on brain MRI (figure). Other RA manifestations occurred 3 months later and included synovitis and necrotizing scleritis. Erythrocyte sedimentation rate was 42 mm/h. High-titer rheumatoid factor (RF) (880 IU/mL) was detected with negative antibody to cyclic citrullinated peptide (anti-CCP). Normal or negative tests included antinuclear (ANA), antineutrophil cytoplasmic antibodies (ANCA), and antibodies to double-stranded DNA, Smith, ribonucleoprotein, Sjogren's A …