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Case Reports : A Case of Eosinophilic Polymyositis Treated with Immunosuppressants
최정호 ( Jung Ho Choi ),강지현 ( Ji Hyoun Kang ),온리휘 ( Lihui Wen ),이정원 ( Jeong Won Lee ),이경은 ( Kyung Eun Lee ),박동진 ( Dong Jin Park ),박용욱 ( Yong Wook Park ),김태종 ( Tae Jong Kim ),이신석 ( Shin Seok Lee ) 대한류마티스학회 2015 대한류마티스학회지 Vol.22 No.5
저자들은 사지의 부종, 근육통 및 근력저하를 주소로 내원한 환자를 호산구성 다발근염 진단 하에 고용량 글루코코르티코이드 치료를 시행하였으나, 이에 충분한 반응을 보이지 않아 면역억제제를 추가로 사용하여 성공적으로 치료한 호산구성 다발근염 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Eosinophilic polymyositis is a rare form of inflammatory muscle disease associated with peripheral blood and/or tissue eosinophilia. Most patients respond to glucocorticoids, however some show poor prognosis, leading to mortality. A 28-year-old female was admitted to our hospital because of myalgia and motor weakness of the upper and lower extremities. Laboratory findings showed significantly elevated levels of muscle enzymes and inflammatory lesions in both thigh muscles were demonstrated on magnetic resonance imaging. A diagnosis of eosinophilic polymyositis was based on histological findings, which showed diffuse eosinophilic infiltration into perivascular spaces in the endomysium and perimysium, and necrosis of myofibers. High-dose methylprednisolone treatment improved the clinical symptoms and muscle enzymes. However, on tapering the glucocorticoid dose, clinical and laboratory findings were exacerbated. After the addition of methotrexate and azathioprine, the symptoms and muscle enzymes recovered without relapse. Here, we report on a case of eosinophilic polymyositis, which responded to immunosuppressants. (J Rheum Dis 2015;22:308-312)
전신홍반루푸스 환자에서 발생한 급성 염증성 탈수초성 다발성 신경병증 1예
이정원 ( Jeong Won Lee ),강지현 ( Ji Hyoun Kang ),홍형주 ( Hyoung Ju Hong ),주선미 ( Sun Mi Ju ),이경은 ( Kyung Eun Lee ),온리휘 ( Lihui Wen ),박동진 ( Dong Jin Park ),김태종 ( Tae Jong Kim ),박용욱 ( Yong Wook Park ),이신석 ( Shi 대한류마티스학회 2014 대한류마티스학회지 Vol.21 No.3
AIDP는 진행하는 양측성의 근력약화 및 심부건 반사의 저하나 소실을 특징으로 하는 질환으로, 루푸스의 신경정신증상의 하나로 발생한다. 진단을 위하여 신경학적 진찰, 뇌척수액 검사, 영상 검사 및 신경전도검사가 필요하고 치료는 일반적인 AIDP와 달리 코르티코스테로이드와 면역 억제제의 사용이 효과적이다. 저자들은 루푸스의 질병활성 없이 경과 관찰하던 환자에서 갑자기 발생한 사지의 근력 악화에 대하여 신경학적 진찰과 신경전도검사를 통해 AIDP로 진단하고 고용량 코르티코스테로이드와 시클로포스타미드를 투여하여 성공적으로 치료한 증례를 국내에서 처음으로 경험하였기에 문헌고찰과 함께 보고하는 바이다. Neuropsychiatric manifestations in patients with systemic lupus erythematosus are fairly common, with a prevalence of 37∼95%. Among 19 neuropsychiatric manifestations, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is quite rare, and is characterized by progressive, symmetric muscle weakness accompanied by absent or depressed deep tendon reflexes. Generally, plasma exchange and intravenous immunoglobulin are the main treatment modalities. Here, we report a case of AIDP in a 29-year-old SLE patient, who was fully recovered with a treatment of high-dose glucocorticoid and immunosuppressive agents. Ours case suggests that AIDP should be treated differently in SLE patients to avoid disastrous results.