Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Department of Cardiology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Clin Exp Rheumatol. 2024 Aug;42(8):1590-1597. doi: 10.55563/clinexprheumatol/gzo4r2. Epub 2024 Mar 15.
This study compares the performance of three composite pulmonary arterial hypertension (PAH) screening tools in a real-life SSc cohort, according to both the previous 2015 ESC/ERS guideline and the recent 2022 ESC/ERS guideline haemodynamic criteria.
Consecutive SSc patients without a previous diagnosis of pulmonary hypertension (PH) were screened for PAH using the European Society of Cardiology/European Respiratory Society (ESC/ERS), DETECT, and Australian Scleroderma Interest Group (ASIG) algorithms. Right heart catheterisation (RHC) referral performances for PAH were compared according to the 2022 ESC/ERS PAH criteria.
Thirty-five of the 81 patients required RHC; 15 (18.5%) according to ESC/ERS, 27 (33.3%) according to DETECT, and 25 (31%) according to ASIG. The final diagnoses were no-PH in 17 patients, WHO group 1 PH (PAH) in 8 patients, WHO group 2 PH in 8 patients, and WHO group 3 PH in 2 patients. When the hemodynamic criteria of the previous ESC/ERS guideline were applied, only one patient was diagnosed with PAH. The sensitivities of the algorithms for the diagnosis of PAH were 62.5% for ESC/ERS, 75% for DETECT, 87.5% for ASIG according to the 2022 ESC/ERS guideline definition, and 100% for all according to the previous ESC/ERS guideline.
With the recent criteria, PAH diagnosis in patients with SSc increased by 1.8-fold. Current algorithms for screening PAH are less sensitive with these revised criteria. Although the ASIG algorithm seems more sensitive, it can still miss the diagnosis. The multimodal/algorithmic approach seems to be the best option for predicting PAH.
本研究根据 2015 年 ESC/ERS 指南和最近的 2022 ESC/ERS 指南血流动力学标准，比较三种复合肺动脉高压（PAH）筛查工具在真实 SSc 队列中的表现。
对未诊断为肺动脉高压（PH）的连续 SSc 患者进行欧洲心脏病学会/欧洲呼吸学会（ESC/ERS）、DETECT 和澳大利亚硬皮病兴趣小组（ASIG）算法的 PAH 筛查。根据 2022 ESC/ERS PAH 标准，比较右心导管检查（RHC）对 PAH 的转诊表现。
81 例患者中有 35 例需要 RHC；根据 ESC/ERS，15 例（18.5%）需要 RHC，27 例（33.3%）根据 DETECT，25 例（31%）根据 ASIG。最终诊断为 17 例无 PH，8 例 WHO 组 1 PH（PAH），8 例 WHO 组 2 PH，2 例 WHO 组 3 PH。当应用之前 ESC/ERS 指南的血流动力学标准时，只有 1 例患者被诊断为 PAH。根据 2022 ESC/ERS 指南的定义，算法对 PAH 的诊断敏感性分别为 ESC/ERS 为 62.5%，DETECT 为 75%，ASIG 为 87.5%，所有标准均为 100%。
根据最近的标准，SSc 患者的 PAH 诊断增加了 1.8 倍。目前用于筛查 PAH 的算法对这些修订标准的敏感性较低。尽管 ASIG 算法似乎更敏感，但它仍然可能遗漏诊断。多模式/算法方法似乎是预测 PAH 的最佳选择。
