Abstract
We report the clinical and autopsy findings of a 71-year-old Japanese woman with rheumatoid meningitis. This patient developed subacute meningitis during an inactive stage of rheumatoid arthritis (RA), and despite intensive examinations no causative agents or underlying disease could be identified except for RA. Based on persistent hypocomplementaemia and increased serum levels of immune complexes she was suspected of having vasculitis, and was treated with intravenous methylprednisolone (1000 mg/day for 3 days) followed by oral prednisolone. Soon after beginning treatment with corticosteroid her symptoms improved, in parallel with a decrease in cell counts and interleukin-6 in the cerebrospinal fluid. During tapering of oral prednisolone she died of a subarachnoid haemorrhage which was ascribed to a relapse of the meningitis. Autopsy demonstrated infiltration of mononuclear cells, including plasma cells, in the leptomeninges, mainly around small vessels, leading to a definite diagnosis of rheumatoid meningitis. When RA patients manifest intractable meningitis with a subacute course, this disease is important as a possible diagnosis even if the arthritis is inactive, and intensive treatment, including corticosteroid and immunosuppressants, should be positively considered as a therapeutic option as soon as possible because of the poor prognosis.
Similar content being viewed by others
Abbreviations
- RA:
-
Rheumatoid arthritis
- IL:
-
Interleukin
- DMARD:
-
Disease-modifying antirheumatic drugs
- RF:
-
Rheumatoid factor
- RAHA:
-
Rheumatoid arthritis haemagglutination
- CSF:
-
Cerebrospinal fluid
- TNF:
-
Tumour necrosis factor
- MRI:
-
Magnetic resonance imaging
References
Bathon JM, Moreland LW, DiBartolomeo AG (1989) Inflammatory central nervous system involvement in rheumatoid arthritis. Semin Arthritis Rheum 18:258–266
Yucel AE, Kart H, Aydin P et al. (2001) Pachymeningitis and optic neuritis in rheumatoid arthritis: successful treatment with cyclophosphamide. Clin Rheumatol 20:136–139
Arnett FC, Edworthy SM, Bloch DA et al. (1988) The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 31:315–324
Steinbrocker O, Traeger CH, Batterman RC (1949) Therapeutic criteria in rheumatoid arthritis. JAMA 140:659–662
Hochberg MC, Chang RW, Dwosh I, Lindsey S, Pincus T, Wolfe F (1992) The American College of Rheumatology 1991 revised criteria for the classification of global functional status in rheumatoid arthritis. Arthritis Rheum 35:498–502
Scott DG, Bacon PA, Allen C, Elson CJ, Wallington T (1981) IgG rheumatoid factor, complement and immune complexes in rheumatoid synovitis and vasculitis: comparative and serial studies during cytotoxic therapy. Clin Exp Immunol 43:54–63
Markenson JA, McDougal JS, Tsairis P, Lockshin MD, Christian CL (1979) Rheumatoid meningitis: a localized immune process. Ann Intern Med 90: 786–789
Choy EH, Panayi GS (2001) Cytokine pathways and joint inflammation in rheumatoid arthritis. N Engl J Med 344:907–916
Maher FA (1954) Dural nodules in rheumatoid arthritis. Arch Pathol 58:354–359
Ellman P, Cudkowicz L, Elwood JS (1954) Widespread serous membrane involvement by rheumatoid nodules. J Clin Pathol 7:239–244
Steiner JW, Gelbloom AJ (1959) Intracranial manifestations in two cases of systemic rheumatoid disease. Arthritis Rheum 2:537–545
Johnson RL, Smyth CJ, Holt GW, Lubchenco A, Valentine E (1959) Steroid therapy and vascular lesions in rheumatoid arthritis. Arthritis Rheum 2:224–249
Contin JU, Oka M (1966) Unusual cardiac, pulmonary and meningeal involvement in rheumatoid arthritis. Dis Chest 49:552–555
Ouyang R, Mitchell DM, Rozdilsky B (1967) Central nervous system involvement in rheumatoid disease. Report of a case. Neurology 17:1099–1105
Ramos M, Mandybur TI (1975) Cerebral vasculitis in rheumatoid arthritis. Arch Neurol 32:271–275
Sunter JP (1977) Rheumatoid disease with involvement of the leptomeninges presenting as symptomatic epilepsy. Beitr Pathol 161:194–202
Watson P, Fekete J, Deck J (1977) Central nervous system vasculitis in rheumatoid arthritis. Can J Neurol Sci 4:269–272
Schachenmayr W, Friede RL (1978) Dural involvement in rheumatoid arthritis. Acta Neuropathol (Berl) 42:65–66
Kim RC (1980) Rheumatoid disease with encephalopathy. Ann Neurol 7:86–91
Kim RC, Collins GH, Parisi JE (1982) Rheumatoid nodule formation within the choroid plexus. Report of a second case. Arch Pathol Lab Med 106:83–84
Rodnan GP (1984) Clinical pathological conference. J Rheumatol 11:855–861
Spurlock RG, Richman AV (1983) Rheumatoid meningitis. A case report and review of the literature. Arch Pathol Lab Med 107:129–131
Jackson CG, Chess RL, Ward JR (1984) A case of rheumatoid nodule formation within the central nervous system and review of the literature. J Rheumatol 11:237–240
Hino H, Aoto K (1995) Rheumatoid meningitis. Shinkeinaika 42:70–72 [in Japanese]
Nishikawa M, Sakamoto H, Kishi H et al. (1996) A case of rheumatoid factor-positive pachymeningitis. No To Shinkei 48:735–739 [in Japanese with English abstract]
Otsuka M, Fujiwara T, Kuwata Y, Yamada S, Ueki A (1997) A case of rheumatoid pachymenigitis. Rinsho Shinkeigaku 37:834–840 [in Japanese with English abstract]
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kato, T., Hoshi, Ki., Sekijima, Y. et al. Rheumatoid meningitis: an autopsy report and review of the literature. Clin Rheumatol 22, 475–480 (2003). https://doi.org/10.1007/s10067-003-0788-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-003-0788-0