硬皮症相關症狀及抗體之關聯性調查：台灣單一中心的照護經驗

The association of symptoms of systemic sclerosis with systemic sclerosis-specific antibodies: a Single Medical Center Experience in Taiwan

目的：從單一醫療中心的資料中，計算系統性硬皮症特異性抗體和其相關症狀的患病率。
方法：我們回顧了從2018年1月1日至2022年12月31間來自三軍總醫院風濕免疫科之患者資料，並從中挑選出診斷為系統性硬皮症之患者資料。我們從以上患者中挑選出限制性系統性硬皮症（lcSSc）之病患、系統性皮症合併肺動脈高壓（SSc-PAH）之病患、及系統性硬皮症合併肺纖維化（SSc-ILD）之病患。本研究亦挑選出抗中心體抗體（ACA）陽性之病患和抗拓撲異構酶I抗體（anti-topoisomerase I Ab）陽性之患者，針對以上症狀及抗體進行分組討論。本研究旨在計算抗體及其相關症狀中的患病率。
結果：我們收納了48名診斷為系統性硬皮症的患者的資料。研究顯示，在限制性系統性硬皮症（lcSSc）病患中，抗中心體抗體（ACA）陽性患者的比例高於非限制性系統性硬皮症病患（non lcSSc）（50% vs. 13.88%; p = 0.018）。但抗中心體抗體（ACA）和系統性硬皮症合併肺動脈高壓（SSc-PAH）之間沒有統計學上的顯著性差異（50% vs. 21.74%; p = 0.410）。研究亦顯示，在無系統性硬皮症合併肺纖維化（SSc non-ILD）之病患中，抗中心體抗體（ACA）的比例高於系統性硬皮症合併肺纖維化（SSc-ILD）之病患（37.93% vs 0%; p = 0.002）。硬皮症合併肺纖維化（SSc-ILD）病患之抗拓撲異構酶I抗體（anti-topoisomerase I Ab）比率亦高於無硬皮症合併和肺纖維化（SSc non-ILD）病患之抗拓撲異構酶I抗體（anti-topoisomerase I Ab）比率（47.37% vs 13.79%; p =0.019）。
結論：我們發現抗中心體抗體（ACA）在限制性系統性硬皮症（lcSSc）及無系統性硬皮症合併肺纖維化（non SSc-ILD）患者中的盛行率較高，但在系統性硬皮症合併肺動脈高壓（SSc-ILD）和抗中心體抗體（ACA）之間沒有統計上的顯著差異。此外，本研究發現在系統性硬皮症合併肺纖維化（SSc-ILD）患者中抗拓撲異構酶I抗體（anti-topoisomerase I Ab）比率較高。

Objectives: We aim to determine the prevalence of systemic sclerosis-specific antibodies and related symptoms in systemic sclerosis (SSc) from a single medical center data.
Methods: We performed a single center cohort study. Patients with SSc meeting 2013 ACR-EULAR criteria between 2018 January and 2022 December were included. Clinical characteristics and laboratory results were retrieved from electronic medical records.
Results: A total of 48 patients with SSc were included. Anti-centromere antibody (ACA) was detected more frequently in patients with limited cutaneous systemic sclerosis (lcSSc) than diffuse cutaneous systemic sclerosis (dcSSc) (50% vs. 13.88%; p=0.018). We also found a higher prevalence of ACA in the SSc non-ILD group compared to the SSc-ILD group (37.93% vs. 0%; p=0.002) and a statistically higher prevalence of anti-topo I Ab in the SSc-ILD group compared to the SSc non-ILD group (47.37% vs. 13.79%; p=0.019).
Conclusions: We identified a higher prevalence of ACA among lcSSc and SSc non-ILD patients, but no statistically significant difference between ACA and SSc-PAH patients. Additionally, the study found a higher prevalence of anti-topo I Ab among SSc-ILD patients. These findings suggest a possible association between SSc-specific antibodies and related symptoms.