Anti-CENP-A陽性患者中（伴或伴隨Anti-Ro52抗體）罹患系統性硬化症、肺動脈高壓及間質性肺病的風險

Risk of Systemic Sclerosis, Pulmonary Arterial Hypertension, and Interstitial Lung Disease in Anti-CENP-A Positive Patients with or without Anti-Ro52 Antibodies

目的：著絲點蛋白A（Anti-CENP-A）抗體經常用於懷疑患有系統性硬化症（SSc）的患者檢測。然而，Anti-CENP-A與重疊症候群、間質性肺病（ILD）、肺動脈高壓（PAH）和癌症之間的關係，特別是在合併抗Ro52抗體的情況下，仍不明確。
方法：本回顧性研究分析了2022年1月1日至2022年12月31日期間於台灣的一個多院區醫療體系接受Anti-CENP-A抗體檢測的疑似SSc患者的數據，追蹤至2023年12月31日。利用傾向分數配對（PSM）減少干擾因子影響，配對變量為年齡與性別。次組分析則評估抗Ro52共同陽性對疾病結果的影響。
結果：共納入564名患者，其中112名（19.9%）Anti-CENP-A檢測陽性。在Anti-CENP-A陽性患者中，33名（29.5%）被診斷為SSc，而陰性患者中僅為13.5%。Anti-CENP-A陽性組的PAH發病率顯著高於陰性組（11.6%對1.6%，p<0.001），而ILD和癌症的發病率無顯著差異。次組分析顯示，同時Anti-CENP-A和抗Ro52陽性的患者ILD風險高於僅Anti-CENP-A陽性的患者（20.7%對3.6%，p=0.009）。PSM（1:1）配對後，形成兩個各110名患者的配對組，進一步確認Anti-CENP-A陽性與SSc和PAH較高風險相關。
結論：Anti-CENP-A抗體是亞洲患者SSc的重要生物標誌，並可能暗示PAH的較高風險。雖然ILD和癌症風險未顯著升高，但抗Ro52與Anti-CENP-A雙重陽性可強化ILD風險評估。仍需進一步的大規模研究來驗證這些發現。

Objective: Anti-centromere protein A (Anti-CENP-A) antibody is frequently tested in patients suspected of systemic sclerosis (SSc). However, the relationship between Anti-CENP-A and overlapping syndrome, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and cancer, particularly in combination with anti-Ro52 antibodies, remains unclear.
Methods: This retrospective study analyzed data from patients suspected of having SSc and tested for Anti- CENP-A antibodies at a multi-site hospital system in Taiwan between January 1, 2022, and December 31, 2022, with follow-up until December 31, 2023. Propensity score matching (PSM) minimized confounding, with age and sex as variables. Subgroup analyses evaluated the impact of anti-Ro52 co-positivity.
Results: A total of 564 patients were included, of whom 112 (19.9%) tested positive for Anti-CENP-A. Among Anti-CENP-A-positive patients, 33 (29.5%) were diagnosed with SSc compared to 13.5% in negative patients. PAH prevalence was significantly higher in the Anti-CENP-A-positive group (11.6% vs. 1.6%, p<0.001), whereas no significant differences were observed for ILD or cancer. Subgroup analysis revealed that patients positive for both Anti-CENP-A and anti-Ro52 had a higher risk of ILD compared to those positive for Anti-CENP-A alone (20.7% vs. 3.6%, p=0.009). PSM (1:1) resulted in two matched cohorts of 110 patients each, confirming the association of Anti-CENP-A positivity with higher risks of SSc and PAH.
Conclusions: Anti-CENP-A antibodies are significant biomarkers for SSc in Asian patients and may indicate an increased risk of PAH. Although ILD and cancer risks were not significantly elevated, the coexistence of anti-Ro52 and Anti-CENP-A may refine ILD risk assessments. Further large-scale studies are warranted.