title: Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center.
abstract: Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Hospital, Bucheon, Korea. chjeon@gmail.com.
authors.forename: Chan Hong
authors.identifier: http://orcid.org/0000-0002-2430-7264
authors.initials: CH
authors.lastname: Jeon
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging; D002493:Central Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology; D060825:Cognitive Dysfunction / Q000175:diagnosis / Q000209:etiology; D003704:Dementia / Q000175:diagnosis / Q000209:etiology; D004569:Electroencephalography; D005260:Female; D006801:Humans; D020363:Limbic Encephalitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000150:complications / Q000175:diagnosis; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000150:complications / Q000175:diagnosis*; D000072078:Positron Emission Tomography Computed Tomography; D013256:Steroids / Q000627:therapeutic use
chemical_list: D013256:Steroids
keywords: Dementia; Limbic Encephalitis; Meningoencephalitis; Relapsing Polychondritis