Limbic encephalitis associated with relapsing polychondritis responded to infliximab and maintained its condition without recurrence after discontinuation: a case report and review of the literature.
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.
Department of General Medicine/Family & Community Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Takeshi
T
Kondo
Center for Medical Education, Nagoya University Hospital, Nagoya, Japan.
Mamiko
M
Fukuta
Department of General Medicine, Nagoya University Hospital, Nagoya, Japan.
Ayumu
A
Takemoto
Department of General Medicine, Nagoya University Hospital, Nagoya, Japan.
Yuichiro
Y
Takami
Department of General Medicine, Nagoya University Hospital, Nagoya, Japan.
Motoki
M
Sato
Department of General Medicine/Family & Community Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Noriyuki
N
Takahashi
Department of General Medicine, Nagoya University Hospital, Nagoya, Japan.
Tomio
T
Suzuki
Department of General Medicine/Family & Community Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Juichi
J
Sato
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Naoki
N
Atsuta
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Gen
G
Sobue
Department of Pediatrics, National Epilepsy Center, Shizuoka, Japan.
Yukitoshi
Y
Takahashi
Department of General Medicine/Family & Community Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Nobutaro
N
Ban
anti-tumor necrosis factor-alpha agent; infliximab; limbic encephalitis; relapsing polychondritis; therapy discontinuation