Kondo Takeshi, Fukuta Mamiko, Takemoto Ayumu, Takami Yuichiro, Sato Motoki, Takahashi Noriyuki, Suzuki Tomio, Sato Juichi, Atsuta Naoki, Sobue Gen, Takahashi Yukitoshi, Ban Nobutaro
Department of General Medicine/Family & Community Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Center for Medical Education, Nagoya University Hospital, Nagoya, Japan.
Nagoya J Med Sci. 2014 Aug;76(3-4):361-8.
Central nervous system (CNS) manifestations are rare complications of relapsing polychondritis (RP). The majority of patients respond well to glucocorticoid therapy, but need to maintain it. Some patients are refractory to initial glucocorticoid therapy and to additional immunosuppressants, and end up with an outcome worse than at therapy initiation. The standardized therapeutic protocol for this condition has not been established. The effects of anti-tumor necrosis factor (TNF) -α agents have been reported recently. We experienced a patient with RP and limbic encephalitis who was refractory to initial high-dose glucocorticoid, but subsequently responded to infliximab and did not show deterioration of signs and symptoms after stopping therapy. We report this case together with a systematic literature review. This is the first case report of RP with CNS manifestations successfully treated by an anti-TNF-α agent without recurrence after discontinuation.
中枢神经系统（CNS）表现是复发性多软骨炎（RP）的罕见并发症。大多数患者对糖皮质激素治疗反应良好，但需要维持治疗。一些患者对初始糖皮质激素治疗及额外的免疫抑制剂治疗无效，最终结局比治疗开始时更差。针对这种情况的标准化治疗方案尚未确立。近期有关于抗肿瘤坏死因子（TNF）-α药物疗效的报道。我们诊治了一名患有RP和边缘性脑炎的患者，该患者对初始大剂量糖皮质激素治疗无效，但随后对英夫利昔单抗有反应，且停药后未出现症状和体征恶化。我们报告该病例并进行系统的文献综述。这是首例有中枢神经系统表现的RP患者经抗TNF-α药物成功治疗且停药后无复发的病例报告。
