Relapsing Polychondritis with Meningoencephalitis Refractory to Immunosuppressant Therapy

Abstract

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.

Relapsing Polychondritis as a Cause of Sudden and Unexpected Death With Central Nervous System Involvement

“…Similar to the 2020 publication, Fujiki et al 7 reported 2 cases of nonherpetic nonparaneoplastic limbic encephalitis associated with RP in 2004 that were successfully evaluated and monitored with MRI, focusing specifically on the medial temporal lobe regions. In contrast to the report by Al-Tabbaa and Habal, 5 both patients were successfully treated with high-dose intravenous corticosteroids and later weaned off. However, later, MRI results showed atrophy and damage to the medial temporal lobe and dilation of the temporal horns of the lateral ventricle, indicative of irreversible ischemic injury.…”

“…The unique and variable presentation of RP in the CNS is best appreciated when contrasting the case reports, diagnostic tools, and therapy responses published in the literature. In 2018, Al-Tabbaa and Habal 5 reported the case of a 25-year-old man who developed meningoencephalitis secondary to RP that was refractory to all high-dose immunosuppressive medications. Of the 19 prior cases of RP with meningoencephalitis reported to date, this was the first report to demonstrate a lack of response to immunosuppressive therapy.…”

“…1,3,4 In even rarer cases, central nervous system involvement has been documented, resulting in a mix of profound and severe complications that can be challenging for healthcare professionals to manage. [5][6][7][8] Of all clinical presentations associated with RP, pulmonary infection, systemic vasculitis, airway collapse, and renal failure represent the most concerning and common causes of death in RP patients. 8 First described in 1923 by Jackson-Wartenhorst-then termed polychondopathia-this condition would later go on to be acknowledged as RP by Pearson et al in 1960, building on the initial assumption of an autoimmune etiology.…”

“…In rare cases, paradoxical involvement of noncartilaginous tissues, such as the heart, kidney, and bone marrow, can become involved, leading to life-threatening conditions 1,3,4 . In even rarer cases, central nervous system involvement has been documented, resulting in a mix of profound and severe complications that can be challenging for healthcare professionals to manage 5–8 . Of all clinical presentations associated with RP, pulmonary infection, systemic vasculitis, airway collapse, and renal failure represent the most concerning and common causes of death in RP patients 8 …”

Overlapping Clinical Syndromes in Patients with Glial Fibrillary Acidic Protein IgG

“…RP is a rare multisystem disorder affecting cartilaginous tissue and the CNS. The phenotype of RP in the CNS commonly manifests as meningoencephalitis and dementia [10][11][12][13][14][15][16]. Case 2 had abnormal mental behavior, memory loss, headache, and abnormal MRI, consistent with the features of RPrelated encephalitis.…”