Uludağ Ömer, Çene Erhan, Gurel Erdem, Çetin Çiğdem, Bektaş Murat, Yalçınkaya Yasemin, Diz-Küçükkaya Reyhan, Gül Ahmet, Inanç Murat, Artim-Esen Bahar
Division of Rheumatology Department of Internal Medicine, Istanbul Faculty of Medicine, 64041Istanbul University, Istanbul, Turkey.
Department of Statistics, Faculty of Arts and Sciences, 563618Yildiz Technical University, Istanbul, Turkey.
Lupus. 2022 Apr;31(4):433-442. doi: 10.1177/09612033221079781. Epub 2022 Feb 15.
To identify the different clinical phenotypes of antiphospholipid syndrome (APS) by using cluster analysis and describe cumulative damage of disease clusters.
This retrospective study includes patients with APS (±systemic lupus erythematosus (SLE)). Two-step cluster analysis was applied by considering clinical data. Damage was calculated for all patients by applying damage index for APS (DIAPS).
A total of 237 patients (198 females; median age of 43 years; median follow-up of 9.5 years) were classified into four clusters. Cluster 1 ( = 74) consisted of older patients with arterial-predominant thrombosis, livedo reticularis, and increased cardiovascular risk; cluster 2 ( = 70) of SLE+APS patients with thrombocytopenia and heart valve disease; cluster 3 ( = 59) of patients with venous-predominant thrombosis, less extra-criteria manifestations; and cluster 4 ( = 34) of patients with only pregnancy morbidity with lower frequency of extra-criteria features and cardiovascular risk. Patients with SLE+APS ( = 123) had the highest mean DIAPS. Regarding clusters, 1 and 2 had high cumulative damage. While cumulative survival rates of clusters did not differ, cluster 2 and 3 had lower survival rates at further years. There was no correlation between DIAPS and mortality.
SLE+APS patients with extra-criteria manifestations and older APS patients with arterial thrombosis and increased cardiovascular risk have higher cumulative damage. Effective treatment of SLE disease activity and control of cardiovascular risk may help to reduce cumulative damage in these patients.
通过聚类分析确定抗磷脂综合征（APS）的不同临床表型，并描述疾病聚类的累积损伤情况。
这项回顾性研究纳入了患有APS（±系统性红斑狼疮（SLE））的患者。通过考虑临床数据应用两步聚类分析。对所有患者应用APS损伤指数（DIAPS）计算损伤情况。
共237例患者（198例女性；中位年龄43岁；中位随访9.5年）被分为四个聚类。聚类1（n = 74）由以动脉为主的血栓形成、网状青斑和心血管风险增加的老年患者组成；聚类2（n = 70）为患有血小板减少症和心脏瓣膜病的SLE+APS患者；聚类3（n = 59）为以静脉为主的血栓形成、额外标准表现较少的患者；聚类4（n = 34）为仅患有妊娠并发症、额外标准特征和心血管风险发生率较低的患者。SLE+APS患者（n = 123）的平均DIAPS最高。关于聚类，聚类1和2有较高的累积损伤。虽然聚类的累积生存率没有差异，但聚类2和3在随后几年的生存率较低。DIAPS与死亡率之间没有相关性。
具有额外标准表现的SLE+APS患者以及患有动脉血栓形成和心血管风险增加的老年APS患者有较高的累积损伤。有效治疗SLE疾病活动并控制心血管风险可能有助于减少这些患者的累积损伤。
