六种自身免疫性疾病并存的空前复杂性:病例报告

Q3 Medicine
Jihed Anoun, Nourhen Guedri, Imen Ben Hassine, Wafa Baya, Anis Mzabi, Fatma Ben Fredj
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引用次数: 0

摘要

引言自身免疫性疾病通常具有相互关联性,但在一名患者身上同时出现多种自身免疫性疾病的情况并不常见。多重自身免疫综合征的特征是一个人至少患有三种不同的自身免疫疾病。本报告概述了一名中年女性的病例,她被诊断患有自身免疫性甲状腺炎、斯约格伦综合征、硬皮病、自身免疫性肝炎、原发性胆汁性肝硬化和抗合成酶综合征。此外,该报告还包括一篇文献综述,涵盖了涉及五种或五种以上自身免疫性疾病的多种自身免疫综合征:一名 57 岁的女性患者,既往无病史,因发热、全身肌肉无力、进行性劳力性呼吸困难、炎性多关节痛、吞咽困难和口干就诊。临床检查显示,患者肩胛骨和骨盆腰部肌肉无力,远端肌肉无力,腕部滑膜炎,具有 "技工之手 "的特征。实验室检查显示有细胞溶解、胆汁淤积、肌酶升高、高丙种球蛋白血症和促甲状腺激素升高。免疫测定显示抗核抗体、抗组蛋白-t-RNA合成酶、抗斯约格伦综合征相关抗原A、抗核糖核酸聚合酶-III-RP155、抗纤连蛋白、抗线粒体、抗肝/肾微粒体1型、抗糖蛋白210和抗甲状腺过氧化物酶抗体阳性。进一步检查后,诊断结果为多种自身免疫综合征,包括自身免疫性甲状腺炎、斯约格伦综合征、硬皮病、自身免疫性肝炎、原发性胆汁性肝硬化和抗合成酶综合征。患者接受了静脉注射免疫球蛋白、皮质类固醇、硫唑嘌呤和熊去氧胆酸治疗,取得了良好的临床和生物学效果:结论:该患者同时患有六种不同的自身免疫性疾病,因此被归类为第二型多重自身免疫综合征。抗异烟酸酶综合征的确定使该病例与众不同。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Unprecedented complexity of six coexisting autoimmune diseases: A case report.

Introduction: Autoimmune disorders often exhibit interconnectedness, although encountering multiple autoimmune conditions in a single patient is uncommon. Multiple autoimmune syndrome is characterized by the presence of at least three distinct autoimmune diseases in an individual. This report outlines the case of a middle-aged woman diagnosed with autoimmune thyroiditis, Sjögren's syndrome, scleroderma, autoimmune hepatitis, primary biliary cirrhosis, and antisynthetase syndrome. Additionally, it includes a literature review encompassing multiple autoimmune syndromes involving five or more autoimmune diseases.

Observation: A 57-year-old woman, with no previous medical history, presented with fever, extensive muscle weakness, progressive exertional dyspnea, inflammatory polyarthralgia, dysphagia, and dry mouth. Clinical examination revealed muscular deficit in the scapular and pelvic girdles, distal muscular deficit, synovitis in the wrists, and features indicative of "mechanic's hand". Laboratory examinations showed cytolysis, cholestasis, elevated muscle enzymes, hypergammaglobulinemia and elevated thyroid stimulating hormone. Immunoassays showed positive results for antinuclear antibodies, anti-histidyl-t-RNA synthetase, anti-Sjögren's-syndrome-related antigen A, anti-ribonucleic-acid-polymerase-III-RP155, anti-fibrillarin, anti-mitochondrial, anti-liver/kidney microsomal type 1, anti-glycoprotein 210, and anti-thyroid peroxidase antibodies. Further investigations led to the diagnosis of a multiple autoimmune syndrome involving autoimmune thyroiditis, Sjögren's syndrome, scleroderma, autoimmune hepatitis, primary biliary cirrhosis, and antisynthetase syndrome. The patient received treatment with intravenous immunoglobulins, corticosteroids, azathioprine, and ursodeoxycholic acid, which resulted in favorable clinical and biological outcomes.

Conclusion: This patient presented with six concurrent distinct autoimmune disorders, categorizing this case as a type two multiple autoimmune syndrome. The identification of antisynthetase syndrome notably distinguishes this case.

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来源期刊
Tunisie Medicale
Tunisie Medicale Medicine-Medicine (all)
CiteScore
1.00
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发文量
72
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