Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, and Department of Clinical and Experimental Sciences, University of Brescia, Italy.
Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, Italy.
Clin Exp Rheumatol. 2023 Aug;41(8):1589-1598. doi: 10.55563/clinexprheumatol/gezm3b. Epub 2023 Feb 10.
In systemic sclerosis (SSc) American patients, anti-Th/To antibodies were reported to be associated with interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Few data in European patients are available, so we aimed at describing the clinical associations of anti-Th/To antibodies, focusing on ILD outcome, organ damage and mortality in an Italian single-centre cohort.
Case-control study: anti-Th/To+ SSc patients vs. anti-topoisomerase (anti-topo)1+, anticentromere (ACA)+ and quadruple-negative (anti-topo 1-, ACA-, anti-RNAP3-, anti-Th/To-) SSc patients (1:3; matched for sex and age at SSc onset). Organ damage was assessed with the SCTC-Damage Index.
Thirteen anti-Th/To+ patients were evaluated: 100% had limited cutaneous involvement; 46% digital ulcers; none had PAH, synovitis, joint contractures. As compared to anti-topo 1+ and quadruple-negative patients, anti-Th/To+ patients developed less frequently ILD (40% vs. 85% and 84%), that required less immunosuppression (8% vs. 41% and 44%), and rarely had functional worsening (15.4% at 5 years), without development of long-term complications (no need for O2, pulmonary hypertension, death). In anti-Th/To+ patients, the Damage Index was lower than in anti-topo 1+ and quadruple-negative patients at various timepoints, and remained low during the long-term follow-up (median: 16 years). The 5- and 10-year survival of anti-Th/To+ patients was 92% and 72%, respectively, and did not differ from those of the SSc matched patients; none of the anti-Th/To+ patients died due to SSc, while mortality was mainly related to cancer.
In this study, anti-Th/To+ patients showed a mild SSc phenotype, characterised by low organ damage, favourable ILD outcome and good survival.
在系统性硬化症（SSc）美国患者中，抗-Th/To 抗体被报道与间质性肺病（ILD）和肺动脉高压（PAH）相关。在欧洲患者中，相关数据较少，因此我们旨在描述意大利单中心队列中抗-Th/To 抗体的临床关联，重点是ILD 结局、器官损伤和死亡率。
病例对照研究：抗-Th/To+ SSc 患者与抗拓扑异构酶（抗-topo）1+、抗着丝粒（ACA）+和四重阴性（抗-topo 1-、ACA-、抗-RNAP3-、抗-Th/To-）SSc 患者（1:3；按 SSc 发病时的性别和年龄匹配）。器官损伤采用 SCTC-Damage Index 进行评估。
评估了 13 例抗-Th/To+患者：100%为局限性皮肤受累；46%有指端溃疡；无 PAH、滑膜炎、关节挛缩。与抗-topo 1+和四重阴性患者相比，抗-Th/To+患者发生 ILD 的频率较低（40%比 85%和 84%），需要的免疫抑制较少（8%比 41%和 44%），且功能恶化罕见（5 年时为 15.4%），无长期并发症（无需吸氧、肺动脉高压、死亡）。在抗-Th/To+患者中，在不同时间点，损伤指数均低于抗-topo 1+和四重阴性患者，且在长期随访期间保持较低水平（中位数：16 年）。抗-Th/To+患者的 5 年和 10 年生存率分别为 92%和 72%，与 SSc 匹配患者无差异；抗-Th/To+患者均未因 SSc 死亡，而死亡率主要与癌症相关。
在这项研究中，抗-Th/To+患者表现出一种轻度的 SSc 表型，其特征为低器官损伤、有利的ILD 结局和良好的生存。
