Rheumatoid Meningitis a Rare Extra-Articular Manifestation of Rheumatoid Arthritis: Report of 6 Cases and Literature Review

Abstract

Objectives. Central neurological manifestations of rheumatoid arthritis (RA) like rheumatoid meningitis (RM) are rare, little known and have a high rate of morbi-mortality. METHODS. We described six cases of RM that were directly related to RA activity after exhaustive assessment. RESULTS. They were mainly women, aged of 50 to 69. All were positive for anti-cyclic citrullinated peptide antibodies and half for rheumatoid factors. RA activity, duration, and treatments were heterogeneous including oral steroids, conventional synthetic disease modifying anti-rheumatic drugs (DMARDs) and biologic DMARDs. Symptoms were various, with acute or progressive beginning; main were: generalized or focal seizure (4/6), fever (3/6), headaches (3/6), and frontal syndrome (2/6). Imaging lesions were four leptomeningitis, one pachymeningitis, and one association of both. MRI usually showed hypersignal in various territories in T2-FLAIR (fluid attenuated inversion recovery) mode, and enhancement in T1-weighted mode after gadolinium injection. All patients had lumbar puncture that found sterile cerebrospinal fluid, no neoplasic cell, elevated cell count in 5/6 cases and elevated proteins concentration in 3/6 cases. Cerebral biopsy was possible for three patients, and definitively confirmed the diagnosis of aseptic lepto- or pachymenintis, excluding vasculitis and lymphoma. Different treatments were used like intravenous high dose steroids, immunoglobulins or biologic DMARDs, with variable clinical and imaging outcome: one death, one complete recovery, and four recoveries with sequelae. Conclusions. Clinical symptoms, imaging, lumbar puncture, and serological studies are often nonspecific, only histologic examination can confirm the diagnosis of RM. Any central neurological manifestation in RA patients, even in quiescent and ancient RA, should warn the physician.

Rheumatoid meningitis: A systematic review and meta‐analysis

“…Another one received IV steroid pulses, etanercept and RTX; the patient died of subarachnoid haemorrhage. The last one received IV steroid pulses, etanercept and adalimumab, and although her lesions stabilized in the CEMRI she was left with neurological sequelae [39]. Some other cases have incorporated an anti‐TNF agent as part of the maintenance treatment; in three patients full recovery was reported [33,40,41] and in one case a partial recovery [42].…”

“…Another one received IV steroid pulses, etanercept and RTX; the patient died of subarachnoid haemorrhage. The last one received IV steroid pulses, etanercept and adalimumab, and although her lesions stabilized in the CEMRI she was left with neurological sequelae [39].…”

Case Report: Rheumatoid meningitis with positive NMDAR antibody: A case treated with intravenous immunoglobulin

“…As a rare complication of rheumatoid arthritis (RA), rheumatoid meningitis (RM) affects the central nervous system (CNS) (1,2), such as the dura mater, pia mater, and arachnoid mater, with the main effects observed in the pia mater (1,(3)(4)(5). The first case of RM was reported approximately 70 years ago (6).…”

“…However, a practical diagnostic approach for RM has not been elucidated. Furthermore, the clinical manifestations of RM markedly vary with no specific changes in biochemical indicators, imaging, and pathology, leading to the limited effectiveness of diagnosis (1,4,5,7,8). In addition, there is no well-laid guideline for RM treatment, and high-dose corticosteroid remains the conventional induction therapy for RM (1,4).…”

“…Furthermore, the clinical manifestations of RM markedly vary with no specific changes in biochemical indicators, imaging, and pathology, leading to the limited effectiveness of diagnosis (1,4,5,7,8). In addition, there is no well-laid guideline for RM treatment, and high-dose corticosteroid remains the conventional induction therapy for RM (1,4). Notably, studies have not explored intravenous immunoglobulin (IVIg) as induction therapy for RM treatment.…”