AbstractObjectiveThe present study aimed to investigate progranulin autoantibodies in systemic sclerosis and autoimmune connective tissue disorders. Progranulin is a physiologic tumor necrosis factor (TNF) antagonist. Progranulin antibodies decrease progranulin levels.MethodsSerum samples of 123 patients with systemic sclerosis and various autoimmune connective tissue disorders (Sjoegren's syndrome [SjS], mixed connective tissue disorder, polymyositis [PM] and dermatomyositis [DM], antiphospholipid syndrome [APLS], and undifferentiated connective tissue disease [UCTD]) were tested for progranulin antibodies using enzyme‐linked immunosorbent assay.ResultsProgranulin antibodies were found in 34 of 123 (27.6%) patients at least once during their disease. In detail, 2 of 8 (25%) patients with limited cutaneous systemic sclerosis, 10 of 31 (32.3%) patients with diffuse cutaneous systemic sclerosis, 9 of 22 (40.9%) patients with SjS, 1 of 3 (33.33%) patients with mixed connective tissue disease, 4 of 33 (12.1%) patients with DM or PM, 6 of 15 (40%) patients with APLS, and 2 of 11 (18.2%) patients with UCTD were positive for progranulin antibodies during the course of disease.ConclusionsProgranulin antibodies are frequently present in patients with systemic sclerosis and other autoimmune connective tissue disorders. Despite the lack of specificity for a given autoimmune disease, progranulin antibodies might not only indicate a potential subtype but also play a pathogenic role in patients with autoimmune connective disorders. Given the important role of TNF‐α in inflammatory processes in autoimmune connective tissue disorders, progranulin antibodies might support the proinflammatory environment by neutralizing the TNF blocker progranulin.

中文翻译：

---

颗粒体蛋白前体自身抗体在系统性硬化症和自身免疫性结缔组织疾病中的作用：初步研究

摘要客观的本研究旨在研究系统性硬化症和自身免疫性结缔组织疾病中的颗粒体蛋白前体自身抗体。颗粒体蛋白前体是一种生理性肿瘤坏死因子 (TNF) 拮抗剂。颗粒体蛋白前体抗体可降低颗粒体蛋白前体水平。方法123 例系统性硬化症和各种自身免疫性结缔组织疾病（干燥综合征 [SjS]、混合结缔组织病、多发性肌炎 [PM] 和皮肌炎 [DM]、抗磷脂综合征 [APLS] 和未分化结缔组织病 [UCTD] 患者的血清样本）使用酶联免疫吸附测定法测试了颗粒体蛋白前体抗体。结果123 名患者中有 34 名 (27.6%) 在患病期间至少发现过一次颗粒体蛋白前体抗体。具体而言，8 名局限性皮肤系统性硬化症患者中的 2 名（25%）、31 名弥漫性皮肤系统性硬化症患者中的 10 名（32.3%）、22 名 SjS 患者中的 9 名（40.9%）、3 名患者中的 1 名（33.33%）混合性结缔组织病患者、33 名 DM 或 PM 患者中的 4 名（12.1%）、15 名 APLS 患者中的 6 名（40%）和 11 名 UCTD 患者中的 2 名（18.2%）在治疗过程中颗粒体蛋白前体抗体呈阳性。疾病。结论颗粒体蛋白前体抗体经常出现在患有系统性硬化症和其他自身免疫性结缔组织疾病的患者中。尽管对特定自身免疫性疾病缺乏特异性，颗粒体蛋白前体抗体不仅可能表明潜在的亚型，而且在自身免疫性结缔性疾病患者中发挥致病作用。鉴于 TNF-α 在自身免疫性结缔组织疾病炎症过程中的重要作用，颗粒体蛋白前体抗体可能通过中和 TNF 阻滞剂颗粒体蛋白前体来支持促炎环境。