摘要:背景：肺動脈高壓(PAH)是係統性硬化症(SSc)死亡的主要原因。推薦使用超聲心動圖(ECHO)進行年度篩查。我們根據一個大型的澳大利亞SSc隊列中提出了PAH篩查方案的方法，該隊列中提出了sscl -PAH的流行病學以及對PAH篩查指南的影響因素的評估。
方法：在所有納入SSc縱向隊列研究的患者中，均確定了PAH篩選的患者特征和結果。通過對澳大利亞風濕病學家的調查，評估了對PAH篩查指南的認可。摘要統計、卡方檢驗、單變量和多變量logistic回歸分析了風險因素與PAH的關係。
結果：在1636例SSc患者中，194例(11.9%)通過右心導管證實了PAH。其中160例是通過篩查發現的。每年的PAH發病率為1.4%。後續診斷的PAH患者和原發診斷的PAH患者更有可能擴散SSc(p = 0.03),根據一個世界衛生組織(世衛組織)關於PAH的診斷(p = 0.01) ，其六分鍾步行試驗的完成情況更好(p = 0.03), 平均肺動脈壓更低(p = 0.009)，平均肺血管阻力更低(p = 0.006)和較少的心包積液(p = 0.03)。在澳大利亞的風濕病學家中，使用基於效仿的算法對其中不到一半的患者進行了10年以上的疾病隨訪是很困難的。
結論：PAH是SSc的常見並發症。醫生對於SSc患者PAH的篩查執行較差。確定可修改的篩查障礙可以提高患者的依從性和影響最終的患者預後。
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.
