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Abstract：
Satoyoshi syndrome is a multisystemic rare disease of unknown etiology, although an autoimmune basis is presumed. Its main symptoms are: painful muscle spasms, diarrhea, alopecia and skeletal abnormalities. Clinical course without treatment may result in serious disability or death. A review of treatment and its response is still pending.
Sixty-four cases of Satoyoshi syndrome were published between 1967 and 2018. 47 cases described the treatment administered. Drugs used can be divided into two main groups of treatment: muscle relaxants/anticonvulsants, and corticosteroids/immunosuppressants. Dantrolene improved muscle symptoms in 13 out of 15 cases, but not any other symptoms of the disease. Other muscle relaxants or anticonvulsant drugs showed little or no effect. 28 out of 30 cases responded to a regimen that included costicosteroids. Other immunosuppressive drugs including cyclosporine, mycophenolate mofetil, azathioprine, methotrexate, tacrolimus and cyclophosphamide were used to decrease corticosteroid dose or improve efficacy. Immunoglobulin therapy was used in nine patients and four of them obtained a favorable response.
Corticosteroids was the most widely treatment employed with the best results in Satoyoshi syndrome. Further studies are needed to determine optimal dose and duration of corticosteroids as well as the role of other immunosuppressants and immunoglobulin therapy. Genetic or autoimmune markers will be useful to guide future therapies.
Sixty-four cases of Satoyoshi syndrome were published between 1967 and 2018. 47 cases described the treatment administered. Drugs used can be divided into two main groups of treatment: muscle relaxants/anticonvulsants, and corticosteroids/immunosuppressants. Dantrolene improved muscle symptoms in 13 out of 15 cases, but not any other symptoms of the disease. Other muscle relaxants or anticonvulsant drugs showed little or no effect. 28 out of 30 cases responded to a regimen that included costicosteroids. Other immunosuppressive drugs including cyclosporine, mycophenolate mofetil, azathioprine, methotrexate, tacrolimus and cyclophosphamide were used to decrease corticosteroid dose or improve efficacy. Immunoglobulin therapy was used in nine patients and four of them obtained a favorable response.
Corticosteroids was the most widely treatment employed with the best results in Satoyoshi syndrome. Further studies are needed to determine optimal dose and duration of corticosteroids as well as the role of other immunosuppressants and immunoglobulin therapy. Genetic or autoimmune markers will be useful to guide future therapies.
摘要：
Satoyoshi综合征是一种病因不明的多系统罕见疾病，尽管推测是自身免疫基础。其主要症状是：肌肉痉挛疼痛，腹泻,脱发和骨骼异常。未经治疗的临床过程可能导致严重的残疾或死亡。对治疗及其反应的审查仍在等待中。
1967年至2018年间发表了64例Satoyoshi综合征。47例描述了给药的治疗。使用的药物可以分为两个主要的治疗组：肌肉松弛药/抗惊厥药，和皮质类固醇/免疫抑制剂。丹曲林改善了15例中的13例肌肉症状，但没有任何其他症状的疾病。其他肌肉松弛剂或抗惊厥药物几乎没有或没有效果。30例病例中有28例对包括皮质类固醇的方案有反应。其他免疫抑制药物，包括环孢素，霉酚酸酯，硫唑嘌呤，甲氨蝶呤,他克莫司和环磷酰胺用于减少皮质类固醇剂量或提高疗效。9例患者使用了免疫球蛋白治疗，其中4例获得了良好的反应。
糖皮质激素是Satoyoshi综合征中使用最广泛的治疗方法，效果最好。需要进一步的研究来确定皮质类固醇的最佳剂量和持续时间以及其他免疫抑制剂和免疫球蛋白疗法的作用。遗传或自身免疫标志物将有助于指导未来的治疗。
1967年至2018年间发表了64例Satoyoshi综合征。47例描述了给药的治疗。使用的药物可以分为两个主要的治疗组：肌肉松弛药/抗惊厥药，和皮质类固醇/免疫抑制剂。丹曲林改善了15例中的13例肌肉症状，但没有任何其他症状的疾病。其他肌肉松弛剂或抗惊厥药物几乎没有或没有效果。30例病例中有28例对包括皮质类固醇的方案有反应。其他免疫抑制药物，包括环孢素，霉酚酸酯，硫唑嘌呤，甲氨蝶呤,他克莫司和环磷酰胺用于减少皮质类固醇剂量或提高疗效。9例患者使用了免疫球蛋白治疗，其中4例获得了良好的反应。
糖皮质激素是Satoyoshi综合征中使用最广泛的治疗方法，效果最好。需要进一步的研究来确定皮质类固醇的最佳剂量和持续时间以及其他免疫抑制剂和免疫球蛋白疗法的作用。遗传或自身免疫标志物将有助于指导未来的治疗。
