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肺动脉高压(PAH)是结缔组织病(CTD)死亡的重要因素之一。容易合并PAH的常见CTD包括系统性红斑狼疮、系统性硬化症、混合性结缔组织病以及原发性干燥综合征等。应针对具有PAH相关高危因素患者进行积极筛查,从而实现早期诊断。强调CTD相关PAH全面评估,重视针对PAH治疗同时,还应通过糖皮质激素联合免疫抑制剂积极控制CTD,从而实现“双重达标”治疗目标,以期改善预后,提高患者生活质量。
Pulmonary hypertension (PAH) is one of the important factors in the death of connective tissue disease (CTD). Common CTDs that easily incorporate PAH include systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease and primary Sjogren’s syndrome. Patients with high risk factors associated with PAH should be actively screened for early diagnosis. Emphasize CTD-related PAH comprehensive assessment, emphasis on PAH treatment, but also by glucocorticoid and immunosuppressive agents should actively control CTD, so as to achieve “double standard” treatment goals, with a view to improving the prognosis and improve patient quality of life.