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Rheumatoid meningitis and infection in absence of rheumatoid arthritis history: review of 31 cases

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Abstract

A 62-year-old healthy male presents with leg weakness and fever. Imaging revealed leptomeningeal enhancement (LE). After cerebrospinal fluid (CSF) cultures were negative, he was discharged with a diagnosis of aseptic meningitis, but was readmitted due to worsening symptoms. Brain biopsy suggested rheumatoid leptomeningitis associated with elevated serum rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (ACPA). Following discharge, the New York State Department of Health (NYSDOH) reported a polymerase chain reaction (PCR) on CSF and brain DNA consistent with Naegleria fowleri (NF). After dramatic improvement on steroids, the patient declined antimicrobial treatment. Upon prednisone taper, symptoms recurred which responded to rituximab (RTX). This case highlights a possible association between rheumatoid leptomeningitis (RM) onset and infection, in a patient without a history of rheumatoid arthritis (RA). Our goal is to assess whether this association is present in 69 RM cases reported since 2000. We also describe diagnosis and treatment of 31 new cases (January 2017 to March 2020). We did not identify evidence of active/latent infection in patients with RM and previous RA; however, patients without RA history appeared to have a significantly higher rate. This finding could demonstrate the necessity of evaluating for infection in de novo RM cases without antecedent RA history. We also describe characteristic clinical patterns for each group. More studies are needed to corroborate these results and expand into a possible distinct natural history of RM in each group, which might have an impact upon the clinical outcome.

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Acknowledgments

We are thankful to Lisa E. Thompson, M.D. for comments that improved the manuscript. We also thank Kimberly Mergen, M.S., and Susan Madison-Antenucci, Ph.D. at Wadsworth Center, New York State Department of Health, for assistance in technique support and PCR interpretation.

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Rheumatology, SUNY-Downstate Medical Center, Health & Hospitals Kings County, Brooklyn, NY, 11201, USA

    Milena Rodriguez Alvarez, Ajita Acharya, Nadish Ravindran & Isabel M McFarlane

  2. School of Graduate Studies, SUNY-Downstate Medical Center, Brooklyn, NY, 11203, USA

    Milena Rodriguez Alvarez & Daniel Mishan

  3. Department of Anesthesiology, SUNY-Downstate Medical Center, Brooklyn, NY, 11203, USA

    Laura Melissa Rodríguez Valencia

  4. Department of Pathology, Stony Brook University Hospital, Stony Brook, NY, 11794, USA

    Roberta Seidman

  5. Department of Health, Wadsworth Center, New York State, Albany, NY, 12237, USA

    Noel Espina

  6. Department of Internal Medicine, Division of Rheumatology, Louisiana State University Health Science Center New Orleans, New Orleans, LA, 70006, USA

    Christopher J Mesa & Luis R Espinoza

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  1. Milena Rodriguez Alvarez
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  2. Laura Melissa Rodríguez Valencia
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Correspondence to Milena Rodriguez Alvarez.

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Rodriguez Alvarez, M., Rodríguez Valencia, L.M., Seidman, R. et al. Rheumatoid meningitis and infection in absence of rheumatoid arthritis history: review of 31 cases. Clin Rheumatol 39, 3833–3845 (2020). https://doi.org/10.1007/s10067-020-05221-1

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