Central nervous system involvement in relapsing polychondritis

Sampaio, Lúzia; Silva, Lígia Torres; Mariz, Eva; Ventura, Francisco

doi:10.1016/j.jbspin.2010.05.015

Cited by 12 publications

(17 citation statements)

References 6 publications

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“…Although CNS involvement due to vasculitis is rare, RP has been reported to be associated with aseptic meningitis, meningoencephalitis, encephalitis, and ischemic stroke (3). To our knowledge, there have been 18 cases of RP-associated meningoencephalitis and encephalitis demonstrated clinically and on neuroimaging in the English literature (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). The clinical features of the previously reported cases and the present case are summarized in Table. Among these cases, the age of onset was between 29 and 73 years (mean, 55 years).…”

Section: Discussionmentioning

confidence: 76%

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

et al. 2015

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We herein report the case of a 39-year-old man who developed bilateral auricular chondritis, conjunctivitis, and central neurological symptoms. He was diagnosed with encephalitis associated with relapsing polychondritis (RP) based on the findings of an ear cartilage biopsy, cerebrospinal fluid examination and magnetic resonance imaging. Although oral prednisolone (60 mg/day) was administered, the initial steroid therapy did not improve his symptoms. In contrast, methylprednisolone (mPSL) pulse therapy followed by prednisolone gradually ameliorated his condition. There were no episodes of recurrence during the two-year follow-up period. A review of the literature revealed that meningoencephalitis and encephalitis are rare, but important, complications of RP responsive to mPSL pulse therapy.

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Section: Discussionmentioning

confidence: 76%

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

et al. 2015

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“…Neurological involvement of RP is rare and affects about 3% of patients but it is an important cause of death ( 3 ). The pathogenesis of CNS involvement in RP is still unknown but seems to be related with autoimmunity.…”

Section: Discussionmentioning

confidence: 99%

“…Steroids in high dose or pulse range are the mainstay of treatment. For patients who are refractory to steroid or are requiring long-term steroid therapy, immunosuppressive or cytotoxic treatments are indicated ( 3 7 12 ). There have been a few reports in which treatment with TNF blocking agent, infliximab has been effective to CNS involvement resistant to steroid and cytotoxic agents ( 9 20 ).…”

Section: Discussionmentioning

confidence: 99%

Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center

Jeon

2016

J Korean Med Sci

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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.

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“…Other reports of peripheral nervous system involvement include hemiplegia, ataxia, myelitis, and polyneuritis. Although CNS involvement is uncommon, there have been reports of aseptic meningitis, meningoencephalitis, encephalitis, ischemic stroke, seizures (focal and generalized), confusion, hallucinations, headache, and cerebral aneurysm (27,28). The pathogenesis of neurologic involvement is thought to be secondary to vasculitis.…”

Section: Nervous System Involvementmentioning

confidence: 99%

Relapsing Polychondritis in the Department of Defense Population and Review of the Literature

Mathew

Battafarano

Morris

2012

Seminars in Arthritis and Rheumatism

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The incidence, demographic data, and organ involvement in our RPC patients were similar to previous studies. The diagnosis of RPC was determined primarily on physical examination and symptom-driven diagnostic testing. There was no notable pattern by rheumatologists for monitoring the progression of tracheobronchial tree or large vessel involvement. Interpreting flow volume loops is recommended with pulmonary function testing to detect early laryngotracheal involvement. Computed tomography of the chest is also recommended to monitor for vascular and tracheobronchial tree involvement.

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Central nervous system involvement in relapsing polychondritis

Cited by 12 publications

References 6 publications

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

Relapsing Polychondritis with Encephalitis: A Case Report and Literature Review

Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center

Relapsing Polychondritis in the Department of Defense Population and Review of the Literature

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