SARAH Network of Rehabilitation Hospitals, Brasilia, Brasil.
Programa de PósGraduação em Neurologia, Universidade Federal Fluminense, Niterói, Brazil.
Rheumatology (Oxford). 2023 Jul 5;62(7):2343-2351. doi: 10.1093/rheumatology/kead067.
Satoyoshi syndrome is a rare multisystem disease of presumed autoimmune aetiology. We carried out a systematic review to evaluate the available evidence to support that autoimmune hypothesis.
We searched for Satoyoshi syndrome cases in PubMed, the Web of Science and Scopus up to January 2022, using keywords 'Satoyoshi syndrome' or 'Komuragaeri disease'. Data on symptoms, associated autoimmune diseases, presence of autoantibodies and response to treatment were collected.
A total of 77 patients from 57 articles published between 1967 and 2021 were included; 59 patients were women. The mean age at diagnosis was 21.2 years. All cases had painful muscular spasms and alopecia. Frequent manifestations included: diarrhoea, malabsorption, growth retardation, amenorrhoea and bone deformity. Satoyoshi syndrome was associated with other autoimmune diseases: myasthenia gravis, autoimmune thyroiditis, idiopathic thrombocytopenic purpura, atopic dermatitis, bronchial and lupus erythematosus. Autoantibody determinations were performed in 39 patients, of which 27 had positive results. The most frequently detected autoantibodies were ANAs. Other less frequently found autoantibodies were: anti-acetylcholine receptor antibodies, anti-DNA antibodies, antithyroid antibodies, anti-glutamic acid decarboxylase (anti-GAD) and anti-gliadin antibodies. Pharmacological treatment was reported in 50 patients. Most of them improved with CS, immunosuppressants and immunoglobulins, or a combination of these medications.
Satoyoshi syndrome is associated with other autoimmune diseases and a variety of autoantibodies. Improvement after CS or other immunosuppressant treatment was observed in 90% of cases. These data support an autoimmune aetiology for Satoyoshi syndrome. More studies including systematic determination of autoantibodies in all patients with Satoyoshi syndrome will help us advance in our understanding of this disease.
Satoyoshi 综合征是一种罕见的多系统疾病，病因可能为自身免疫。我们进行了一项系统评价，以评估支持自身免疫假说的现有证据。
我们在 PubMed、Web of Science 和 Scopus 中检索了 Satoyoshi 综合征病例，检索时间截至 2022 年 1 月，使用的关键词是“Satoyoshi 综合征”或“Komuragaeri 病”。收集了症状、相关自身免疫性疾病、自身抗体存在情况和治疗反应等数据。
共纳入 57 篇发表于 1967 年至 2021 年的文章中的 77 例患者，其中 59 例为女性。诊断时的平均年龄为 21.2 岁。所有患者均有疼痛性肌肉痉挛和脱发。常见表现包括腹泻、吸收不良、生长迟缓、闭经和骨骼畸形。 Satoyoshi 综合征与其他自身免疫性疾病有关，包括重症肌无力、自身免疫性甲状腺炎、特发性血小板减少性紫癜、特应性皮炎、支气管哮喘和红斑狼疮。39 例患者进行了自身抗体检测，其中 27 例结果阳性。最常检测到的自身抗体是抗核抗体。其他较少见的自身抗体包括：抗乙酰胆碱受体抗体、抗 DNA 抗体、抗甲状腺抗体、抗谷氨酸脱羧酶（anti-GAD）抗体和抗麦胶蛋白抗体。50 例患者报告了药物治疗情况。大多数患者在接受 CS、免疫抑制剂和免疫球蛋白治疗或这些药物联合治疗后症状改善。
Satoyoshi 综合征与其他自身免疫性疾病和多种自身抗体有关。90%的病例在接受 CS 或其他免疫抑制剂治疗后病情改善。这些数据支持 Satoyoshi 综合征的自身免疫病因。对所有 Satoyoshi 综合征患者进行系统的自身抗体检测的更多研究将有助于我们深入了解这种疾病。
