Meningoencephalitis in relapsing polychondritis

Abstract

Rationale: Aseptic meningoencephalitis is a rare central nervous system complication of relapsing polychondritis (RP). Patient: We report a 61-year-old Japanese male patient with spiking fever and impaired consciousness. Neurological examination revealed meningealirritation, and cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with elevated protein (199 mg/dL) and interleukin-6 (3810 pg/mL). Serological analysis showed high levels of anti-type II collagen antibodies, and the result of auricular biopsy was consistent with the diagnosis of RP showing cartilage degeneration surrounded by inflammatory cell infiltrations. Diagnosis: A clinical diagnosis of RP was made according to the diagnostic criteria established by MacAdams et al. Intervention: Steroid pulse therapy (methylprednisolone 1000 mg, consecutive 3 days) followed by oral prednisolone (60 mg/day) resolved the patient's high fever and disturbance of consciousness. Outcomes: The patient rapidly improved after steroid treatments and has a normal quality of life under the maintenance dose of steroid plus methotrexate (4 mg/week). Lessons: RP-associated meningoencephalitis is a rare complication with significant morbidity and mortality. It should be considered and differentiated in patients with RP with unexplained spiking fever and impaired consciousness. In addition, the assessment of cerebrospinal fluid interleukin-6 levels may be useful to investigate the disease activity of RP-related meningoencephalitis. Further prospective studies are required to confirm this result.

Relapsing Polychondritis and Aseptic Meningoencephalitis

“…Relapsing polychondritis (RP) is a rare rheumatic disease that causes recurrent and repetitive inflammation, mainly in the hyaline cartilage throughout the whole body ( 1 ). The ear cartilage is mainly damaged in the early stage of the disease ( 2 ).…”

“…The ear cartilage is mainly damaged in the early stage of the disease ( 2 ). Although the cause of RP has yet to be elucidated, an autoimmune mechanism involving collagen is suspected ( 1 , 2 ). RP-associated central and peripheral neuropathies occur in 3% of patients ( 2 ).…”

Relapsing polychondritis presenting as otitis externa

“…Results showed spongiotic dermatitis with lymphocytes and plasma cells suggestive of relapsing polychondritis (HD 21). Paraspinal muscle biopsy also showed a focus of plasma cells in the connective tissue surrounding The Southwest Respiratory and Critical Care Chronicles 2023;11 (46): [44][45][46][47][48][49][50][51][52] Several HLA alleles have been associated with RP, including HLA-DR4, which supports an autoimmune process. 4 Cellular immunity may also have a role, with T cells demonstrating reactivity towards collagen.…”

“…The Southwest Respiratory and Critical Care Chronicles 2023;11 (46): [44][45][46][47][48][49][50][51][52] monotherapy were associated with recurrence. 40 However, certain manifestations portend a worse prognosis.…”

“…49,50 Several other cases also document limbic encephalitis, meningitis, and characteristic atrophy of the hippocampi seen on MRI imaging. 49,[51][52][53] It has been proposed that when CNS symptoms are present that hippocampal and other white matter changes on MRI imaging have a crucial role in monitoring the disease progression. 54 Studies suggest that dementia and mental changes associated with RP were reversible with treatment, [55][56][57] as was meningitis.…”