Department of Internal Medicine, Soonchunhyang University Hospital Bucheon, Bucheon, Gyeonggi-do, Korea.
Int J Rheum Dis. 2012 Oct;15(5):e80-9. doi: 10.1111/j.1756-185X.2012.01815.x. Epub 2012 Sep 27.
The REgistry of Pulmonary Hypertension Associated with Rheumatic Disease (REOPARD) was established in Korea. The baseline data are described from the second year of the registry's operation.
Patients with a connective tissue disease (CTD) who met the modified definition of the WHO group I pulmonary arterial hypertension (PAH) were enrolled. PAH was defined as a systolic pulmonary arterial pressure> 40 mmHg by echocardiography or mean pulmonary arterial pressure> 25 mmHg by right heart catheterization. Hemodynamic parameters and clinical data such as demographics, functional class, underlying disease, organ involvement, laboratory tests and current treatment were recorded.
A total of 321 patients were enrolled during the 2-year study period from 2008 to 2010. The mean age of the patients at registration was 51.9 years and 87.5% were female. Most patients were diagnosed by echocardiography and only 24 patients (7.5%) underwent cardiac catheterization. Exertional dyspnea was present in 63.6% of patients and 31.8% were New York Heart Association class III or IV. Among the patients, systemic lupus erythematosus accounted for 35.3%, systemic sclerosis 28.3%, rheumatoid arthritis 7.8%, overlap syndrome 9.0%, and mixed connective tissue disease 5.9%. There were no significant differences in hemodynamics, functional class, diffusing capacity and N-terminal pro-brain natriuretic peptide levels between the disease subgroups. Treatments consisted of calcium antagonists (57.0%), endothelin antagonists (32.7%), prostanoids (27.1%), phosphodiesterase-5 inhibitors (14.3%) and combinations (37.4%).
Compared with previous studies, the results showed some differences: underlying diseases, functional status and treatments. This may be due to differences in ethnic background and diagnostic methods of our study.
韩国建立了肺动脉高压与风湿性疾病登记处（REOPARD）。本文描述了该登记处开展第二年的基线数据。
登记处纳入符合世界卫生组织（WHO）I 组肺动脉高压（PAH）改良定义的结缔组织病（CTD）患者。PAH 定义为超声心动图提示收缩期肺动脉压>40mmHg 或右心导管检查提示平均肺动脉压>25mmHg。记录血流动力学参数和临床数据，包括人口统计学、功能状态、基础疾病、器官受累、实验室检查和当前治疗情况。
2008 年至 2010 年期间，该登记处开展了为期 2 年的研究，共纳入 321 例患者。登记时患者的平均年龄为 51.9 岁，87.5%为女性。大多数患者通过超声心动图诊断，仅有 24 例（7.5%）患者行右心导管检查。63.6%的患者有劳力性呼吸困难，31.8%为纽约心脏病协会（NYHA）心功能 III 或 IV 级。患者中系统性红斑狼疮占 35.3%，系统性硬化症占 28.3%，类风湿关节炎占 7.8%，重叠综合征占 9.0%，混合性结缔组织病占 5.9%。各疾病亚组间的血流动力学、功能状态、弥散量和 N 末端脑钠肽前体水平无显著差异。治疗包括钙通道阻滞剂（57.0%）、内皮素受体拮抗剂（32.7%）、前列环素（27.1%）、磷酸二酯酶-5 抑制剂（14.3%）和联合治疗（37.4%）。
与既往研究相比，本研究结果存在一些差异，包括基础疾病、功能状态和治疗方法。这可能与研究人群的种族背景和诊断方法不同有关。
