Santos Estrella Paul V, Lin Yih Chang, Navarra Sandra V
Department of Rheumatology, Allergy and Immunology Center, St. Luke's Medical Center, 279 E. Rodriguez Blvd., Quezon City 1102, Philippines.
Mod Rheumatol. 2007;17(3):224-7. doi: 10.1007/s10165-007-0569-8. Epub 2007 Jun 20.
We describe the clinical features, therapies, and clinical course of pulmonary arterial hypertension (PAH) in a group of Filipinos with connective tissue diseases (CTDs). We retrospectively reviewed the records of patients diagnosed with PAH by a two-dimensional echocardiogram as a tricuspid regurgitant jet of more than 25 mmHg. All patients had underlying CTDs, defined by the American College of Rheumatology criteria, and were seen at the rheumatology clinics of the University of Santo Tomas Hospital and the St. Luke's Medical Center, Philippines. Of the 33 patients (32 women) included in the analysis, there were 14 patients with systemic lupus erythematosus (SLE), 12 with scleroderma, 5 with mixed connective tissue disease (MCTD), 1 with primary antiphospholipid syndrome (APS), and 1 with dermatomyositis. The average age at PAH diagnosis was 38 +/- 14 years (mean +/- SD), and the mean duration of illness from CTD to PAH diagnosis was 53 +/- 52 months. Twelve patients had died at the time of this report, with a median duration of 15 months (range 1-57 months) from PAH diagnosis to mortality: six of these had scleroderma, five with SLE, and one with APS. The following therapies were used in this group of patients: low molecular weight heparin, warfarin, calcium-channel blockers, aspirin, cyclophosphamide, bosentan, iloprost, and sildenafil. We have described the clinical profile of PAH in a group of Filipino patients with CTDs, most commonly SLE. Various forms of pharmacologic therapies were used among these patients. Mortality remains high, particularly among those with underlying scleroderma. Early recognition and treatment are crucial in order to provide a better outcome for these patients.
我们描述了一组患有结缔组织病（CTD）的菲律宾人肺动脉高压（PAH）的临床特征、治疗方法及临床病程。我们回顾性分析了通过二维超声心动图诊断为PAH（三尖瓣反流速度超过25 mmHg）患者的病历。所有患者均符合美国风湿病学会标准定义的潜在CTD，且均在菲律宾圣托马斯大学医院和圣卢克医疗中心的风湿病门诊就诊。纳入分析的33例患者（32例女性）中，14例患有系统性红斑狼疮（SLE），12例患有硬皮病，5例患有混合性结缔组织病（MCTD），1例患有原发性抗磷脂综合征（APS），1例患有皮肌炎。PAH诊断时的平均年龄为38±14岁（均值±标准差），从CTD诊断到PAH诊断的平均病程为53±52个月。在本报告发布时，已有12例患者死亡，从PAH诊断到死亡的中位时间为15个月（范围1 - 57个月）：其中6例患有硬皮病，5例患有SLE，1例患有APS。该组患者采用了以下治疗方法：低分子量肝素、华法林、钙通道阻滞剂、阿司匹林、环磷酰胺、波生坦、伊洛前列素和西地那非。我们描述了一组患有CTD的菲律宾患者（最常见的是SLE）中PAH的临床特征。这些患者使用了多种形式的药物治疗。死亡率仍然很高，尤其是在患有潜在硬皮病的患者中。早期识别和治疗对于为这些患者提供更好的预后至关重要。
