Rheumatoid arthritis with pachymeningitis - a case presentation and review of the literature

Abstract

Rheumatoid meningitis (RM) is a rare central nervous system (CNS) manifestation of rheumatoid arthritis (RA) with a wide spectrum of symptoms. We present a review of the literature with a rare illustrative case of a 61-year-old man with a history of seropositive rheumatoid arthritis (RA) who presented headaches, stroke-like symptoms and seizures. MRI revealed the leptomeningeal enhancement in the right hemisphere. As cerebromeningeal fluid showed increased level of protein and was positive for Candida mannan, the initial clinical diagnosis was fungal meningitis. Despite the antifungal treatment the patient's clinical condition did not improve. Detailed laboratory, radiologic and histopathological diagnostics enabled the diagnosis of RM. In conclusion is worth to highlight that presentation of RM is variable and complex, diagnosing it is a big dilemma which is why it must be considered in the differential in a patient with long-standing seropositive RA.

Keywords: central system involvement; rheumatoid arthritis; rheumatoid meningitis; stroke-like symptoms.

Fig. 1

MRI scan of the brain on first admission to the hospital. The study description was prepared by prof. Edyta Szurowska. Axial TIRM dark fluid sequence – cerebral cortex oedema in the frontoparietal region of the right hemisphere with clamping grooves of brain and increase of their signal (A); axial DWI sequence – minor foci of diffusion reduction in the right parietal lobe (B); axial, contrast-enhanced T1 –dependent images- distinct enhancement of dura mater and pia mater in frontoparietal area (C).

Fig. 2

Histopathology of cerebro-meningeal biopsy. The study description was prepared by prof. Ewa Iżycka-Świeszewska. Intense chronic inflammation with vasculitis and granulation tissue within leptomeninges and subarachnoid space as well as slight vasculitis of cortical vessels (HE, 100 ×) (A); fibrosing granulation tissue with histiocytic multinucleated cell necrotizing granuloma and hyalinization of blood vessels walls (HE, 200 ×) (B); inflammatory infiltrate made of foamy and multinucleated histiocytes, lymphocytes and plasmocytes (HE, 400 ×) (C); chronic meningitis with active vasculitis in form of dense intraparietal and perivascular lympho-plasmocytic infiltrates, old extravasations with hemosiderin- laden macrophages and collagen fibers deposition (HE, 400 ×) (D).

Fig. 3

MRI scan of the brain after 6 months of immunosuppressive treatment. The study description was prepared by prof. Edyta Szurowska. Axial FLAIR sequence – regression of cerebral cortex oedema placed in the frontoparietal region of the right hemisphere, grooves of brain of proper width, visible only band-like area of gliosis in the area of surgical access (A); axial, contrast-enhanced T1 – dependent images – distinct regression of meninges enhancement in frontoparietal area, minor enhancement of post-biopsy site (B).

Fig. 4

Results of data searching.