系统性风湿性疾病患者收缩期肺动脉压进展的结局和危险因素：韩国登记处的随访结果
Outcomes and Risk Factors of Systolic Pulmonary Artery Pressure Progression in Patients with Systemic Rheumatic Diseases: Follow-up Results from a Korean Registry.
作者信息
Seo Mi Ryoung, Yeo Jina, Ryu Hee Jung, Choi Hyo-Jin, Ko Kwang-Pil, Baek Han Joo
机构信息
Department of Internal Medicine, Division of Rheumatology, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea.
Department of Preventive Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, South Korea.
出版信息
Arch Rheumatol. 2020 Apr 27;35(4):558-567. doi: 10.46497/ArchRheumatol.2020.7812. eCollection 2020 Dec.
OBJECTIVES
This study aims to investigate the outcomes and risk factors associated with the progression of systolic pulmonary artery pressure (sPAP) in patients with systemic rheumatic diseases.
PATIENTS AND METHODS
A total of 532 patients (73 males, 459 females; median age 49 years; interquartile range (IQR), 36 to 62 years) registered with the Registry of Pulmonary Hypertension Associated with Rheumatic Diseases were included. Mortality curves were constructed using the Kaplan- Meier method and comparisons were performed using the log-rank test. A paired t-test was performed to evaluate the patients with markedly elevated sPAP between baseline and follow-up.
RESULTS
The average follow-up duration was 31 months (IQR, 9 to 60 months). Of the patients, 196 had follow-up echocardiographs at least one year later. We defined the sPAP over 60 mmHg as markedly elevated. Patients in the increased sPAP above 60 mmHg at follow-up and persistently markedly elevated sPAP were associated with worse outcomes in all-cause mortality and pulmonary arterial hypertension-related mortality (p<0.001). In patients with systemic sclerosis, the majority of patients remained static within their pressure group or rose progressively: the patients with markedly elevated sPAP at follow-up were higher than those at baseline (32% versus 15%, p<0.01). In patients with mixed connective tissue disease (MCTD) or rheumatoid arthritis (RA), the majority of patients remained static within their pressure group or gradually improved: the patients with markedly elevated sPAP at follow-up were lower than those at baseline (RA=14% versus 29%, MCTD=5% versus 16%, p<0.05).
CONCLUSION
Persistently high sPAP or increase of sPAP over 60 mmHg at follow-up was associated with increased mortality. There were some differences in the progression of sPAP according to the underlying rheumatic diseases.
目的
本研究旨在调查系统性风湿性疾病患者收缩期肺动脉压（sPAP）进展的相关结局和危险因素。
患者与方法
纳入了532例登记在风湿性疾病相关肺动脉高压登记处的患者（73例男性，459例女性；中位年龄49岁；四分位间距（IQR）为36至62岁）。采用Kaplan-Meier法构建死亡率曲线，并使用对数秩检验进行比较。采用配对t检验评估基线和随访之间sPAP显著升高的患者。
结果
平均随访时间为31个月（IQR为9至60个月）。其中196例患者在至少一年后进行了随访超声心动图检查。我们将sPAP超过60 mmHg定义为显著升高。随访时sPAP升高超过60 mmHg以及sPAP持续显著升高的患者，其全因死亡率和肺动脉高压相关死亡率的结局较差（p<0.001）。在系统性硬化症患者中，大多数患者在其压力组内保持稳定或逐渐升高：随访时sPAP显著升高的患者高于基线时（32%对15%，p<0.01）。在混合性结缔组织病（MCTD）或类风湿关节炎（RA）患者中，大多数患者在其压力组内保持稳定或逐渐改善：随访时sPAP显著升高的患者低于基线时（RA=14%对29%，MCTD=5%对16%，p<0.05）。
结论
随访时sPAP持续升高或升高超过60 mmHg与死亡率增加相关。根据潜在的风湿性疾病，sPAP的进展存在一些差异。
Zotero 插件