抗拓扑异构酶型抗体阳性的局限性硬皮病患者更可能发展为弥漫性硬皮病

作者：Kranenburg P,et al.

翻译：北医三院 金银姬，校对：武东

摘要：目的：局限性硬皮病（lcSSc）与抗着丝点抗体(ACAs)及缓和的病程相关，而弥漫性系统性硬化症（dcSSc）与抗拓扑异构酶型抗体 (ATAs)及更严重的病程相关。然而，ATA也可见于lcSSc。关于其亚型的生存及器官受累目前暂不明确。本研究的目的为明确lcSSc ATA阳性患者与 lcSSc ATA阴性或dcSSc ATA阳性患者之间是否存在生存及器官受累差异。此外，评估lcSSc转变为dcSSc的具体情况。

方法：使用Nijmegen系统性硬化症数据库，最长随访15年。Kaplan Meier分析生存及器官受累，包括间质性肺病、肺动脉高压、心脏受累和硬皮病肾危象。通过Cox比例风险模型调整混杂因素。

结果：本研究共纳入460名患者：58 (13%) lcSSc ATA阳性患者, 237 (52%) lcSSc ATA阴性患者和78 (17%) dcSSc ATA阳性患者。其中lcSSc ATA阳性患者的累积生存率为75%，lcSSc ATA阴性患者累积生存率为58%，dcSSc ATA阳性患者累积生存率为53%。间质性肺病在lcSSc ATA阳性患者(49%)中较lcSSc ATA阴性患者(25%)更普遍，但低于dcSSc ATA阳性的患者(60%)。48名患者发展为dcSSc:24名ATA阴性，24 名ATA阳性(P < 0.001)。

结论：在生存及器官受累方面，lcSSc ATA阳性患者不同于 lcSSc ATA阴性患者及dcSSc ATA阳性患者。ATA阳性的lcSSc患者较ATA阴性的lcSSc相比，更有可能发展为dcSSc。

附原文：Abstract OBJECTIVE: LcSSc is associated with ACAs and a mild course, whereas dcSSc is associated with anti-topoisomerase antibodies (ATAs) and a more severe course. However, ATAs are also present in lcSSc. Little is known about survival and organ involvement in this subgroup. The aim of this study is to determine whether survival and organ involvement of lcSSc ATA-positive patients differs from lcSSc ATA-negative or dcSSc ATA-positive patients. Furthermore, transition from lcSSc to dcSSc was evaluated. METHODS: Data from The Nijmegen Systemic Sclerosis cohort were used, with up to 15 years of follow-up. Kaplan-Meier analysis was performed for survival and organ involvement, including interstitial lung disease, pulmonary arterial hypertension, cardiac involvement and Scleroderma Renal Crises. Cox proportional hazard modelling was performed to adjust for confounders. RESULTS:A total of 460 patients were included: 58 (13%) lcSSc ATA-positive patients, 237 (52%) lcSSc ATA-negative patients and 78 (17%) dcSSc ATA-positive patients. Cumulative survival in lcSSc ATA-positive patients was 75%, in lcSSc ATA-negative patients 58% and in dcSSc ATA-positive patients 53%. Interstitial lung disease was more prevalent in lcSSc ATA-positive patients (49%) than in lcSSc ATA-negative patients (25%), but less than in dcSSc ATA-positive patients (60%). Forty-eight patients developed dcSSc: 24 ATA-negative and 24 ATA-positive (P < 0.001). conclusion:lcssc ata-positive patients differ from lcssc ata-negative patients and dcssc ata-positive patients concerning survival and organ involvement. lcssc patients who are ata-positive are more likely to develop dcssc than lcssc patients who are ata negative.

引自：Kranenburg P, van den Hombergh WM, Knaapen-Hans HK, et al. Survival and organ involvement in patients with limited cutaneous systemic sclerosis and anti-topoisomerase-I antibodies: determined by skin subtype or auto-antibody subtype? A long-term follow-up study. Rheumatology (Oxford). 2016 Aug 12. pii: kew298. [Epub ahead of print]