摘要:背景：肺动脉高压(PAH)是系统性硬化症(SSc)死亡的主要原因。推荐使用超声心动图(ECHO)进行年度筛查。我们根据一个大型的澳大利亚SSc队列中提出了PAH筛查方案的方法，该队列中提出了sscl -PAH的流行病学以及对PAH筛查指南的影响因素的评估。

        方法：在所有纳入SSc纵向队列研究的患者中，均确定了PAH筛选的患者特征和结果。通过对澳大利亚风湿病学家的调查，评估了对PAH筛查指南的认可。摘要统计、卡方检验、单变量和多变量logistic回归分析了风险因素与PAH的关系。

        结果：在1636例SSc患者中，194例(11.9%)通过右心导管证实了PAH。其中160例是通过筛查发现的。每年的PAH发病率为1.4%。后续诊断的PAH患者和原发诊断的PAH患者更有可能扩散SSc(p = 0.03),根据一个世界卫生组织(世卫组织)关于PAH的诊断(p = 0.01) ，其六分钟步行试验的完成情况更好(p = 0.03), 平均肺动脉压更低(p = 0.009)，平均肺血管阻力更低(p = 0.006)和较少的心包积液(p = 0.03)。在澳大利亚的风湿病学家中，使用基于效仿的算法对其中不到一半的患者进行了10年以上的疾病随访是很困难的。

        结论：PAH是SSc的常见并发症。医生对于SSc患者PAH的筛查执行较差。确定可修改的筛查障碍可以提高患者的依从性和影响最终的患者预后。

        附原文：

        Abstract Background Pulmonaryarterial hypertension (PAH) is the leading cause of death in systemic sclerosis(SSc). Annual screening with echocardiogram (ECHO) is recommended. We presentthe methodological aspects of a PAH screening programme in a large AustralianSSc cohort, the epidemiology of SSc-PAH in this cohort, and an evaluation offactors influencing physician adherence to PAH screening guidelines. Methods Patientcharacteristics and results of PAH screening were determined in all patientsenrolled in a SSc longitudinal cohort study. Adherence to PAH screeningguidelines was assessed by a survey of Australian rheumatologists. Summarystatistics, chi-square tests, univariate and multivariable logistic regressionwere used to determine the associations of risk factors with PAH. Results Among 1636 patientswith SSc, 194 (11.9%) had PAH proven by right-heart catheter. Of these, 160were detected by screening. The annual incidence of PAH was 1.4%. Patients withPAH diagnosed on subsequent screens, compared with patients in whom PAH wasdiagnosed on first screen, were more likely to have diffuse SSc (p=0.03),be in a better World Health Organisation (WHO) Functional Class at PAHdiagnosis (p=0.01)and have less advanced PAH evidenced by higher mean six-minute walk distance (p=0.03),lower mean pulmonary arterial pressure (p=0.009),lower mean pulmonary vascular resistance (p=0.006)and fewer non-trivial pericardial effusions (p=0.03).Adherence to annual PAH screening using an ECHO-based algorithm was poor amongAustralian rheumatologists, with less than half screening their patients withSSc of more than ten years disease duration. Conclusion PAH is a common complication of SSc. Physicianadherence to PAH screening recommendations remains poor. Identifying modifiablebarriers to screening may improve adherence and ultimately patient outcomes.

        引自：Morrisroe K1,2,Stevens W2,Sahhar J3,Rabusa C2,Nikpour M4,5,Proudman S6,7;AustralianSclerodermaInterest Group (ASIG). Epidemiologyand disease characteristics ofsystemic sclerosis-relatedpulmonaryarterial hypertension: results from a real-lifescreeningprogramme.Arthritis Res Ther.2017 Mar 7;19(1):42.doi: 10.1186/s13075-017-1250-z.