Relapsing Polychondritis with Meningoencephalitis Refractory to Immunosuppressant Therapy

Meningoencephalitis is a rare complication of relapsing polychondritis. We report a case of a 25-year-old male who presented with visual hallucinations and symptoms of depression and anxiety, white matter changes on MRI, and CSF lymphocytosis, along with inflammatory chondritis seen in his auricle cartilage biopsy. Eventually he was given the diagnosis of RP presenting with meningoencephalitis based on CSF analysis, brain MRI findings, negative serologies, and neurologic exam findings. The patient's clinical state did not improve despite being on IV methylprednisolone for a period of 7 days; afterwards he was switched to oral prednisone with no clinical improvement. As a result, he was given cyclophosphamide and rituximab, respectively, without benefit. He also underwent craniectomy with VP shunt due to worsening hydrocephalus and a brain biopsy was done to confirm the diagnosis. He is currently on methotrexate and steroid dependent with a goal to taper down. Even though all 19 reported cases of meningoencephalitis with RP in the literature did respond to immunosuppressive therapy, in our case, however the patient did not respond to immunosuppressive treatment and currently is in mute dementia status after three years of treatment.