Department of Rheumatology, The first affiliated hospital of Nanjing Medical University, Nanjing, China.
Clin Rheumatol. 2019 Dec;38(12):3619-3626. doi: 10.1007/s10067-019-04690-3. Epub 2019 Aug 5.
To explore the long-term prognostic value of a simplified risk assessment strategy based on the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines in Chinese patients with connective tissue disease (CTD) associated with pulmonary arterial hypertension (PAH).
We identified 50 CTD-PAH patients diagnosed by right heart catheterization. A retrospective chart review was completed to assess their clinical presentation and laboratory test results. A simplified version of the risk stratification model proposed by the 2015 ESC/ERS PH guidelines was applied, which included the WHO functional class, the 6-minute walking distance test, N-terminal pro-B-type natriuretic peptide plasma levels, pericardial effusion, right atrial pressure (RAP), cardiac index (CI), and mixed venous oxygen saturation (SvO). The risk grades were defined as follows: low risk = at least 3 low-risk variables and no high-risk variables; high risk = at least 2 high-risk variables, including SvO or CI; and intermediate risk = when the above definitions of low or high risk were not fulfilled. The study endpoint was 3-year all-cause mortality.
Twenty patients were defined as a low-risk group, while 30 were classified into a combined intermediate-high-risk group at the baseline assessment. All 20 patients in the low-risk group remained in the low-risk group at follow-up, 20 patients in the intermediate-high-risk group were downgraded to the low-risk group, and eight patients remained in the intermediate-high-risk group at the follow-up assessment. Patients in the intermediate-high-risk group exhibited higher 3-year mortality than the low-risk group at baseline (26% vs 14%, P = 0.0384). Compared with patients who remained in the intermediate-high-risk group, patients who were downgraded to the low-risk group showed lower 3-year mortality (P = 0.0281).
A simplified risk stratification model based on the 2015 ESC/ERS PH guidelines helped to identify CTD-PAH patients with poor long-term prognosis , which was useful in evaluating the severity and treatment response of patients with CTD-PAH.Key Point•This study showed that the simplified version of the 2015 ESC/ERS risk stratification model could help identify Chinese CTD-PAH patients with poor prognosis at diagnosis and after treatment initiation.
探讨基于 2015 年欧洲心脏病学会（ESC）/欧洲呼吸学会（ERS）肺动脉高压（PH）指南的简化风险评估策略在伴有肺动脉高压（PAH）的结缔组织病（CTD）患者中的长期预后价值。
我们纳入了 50 例经右心导管检查诊断为 CTD-PAH 的患者。回顾性分析其临床表现和实验室检查结果。应用 2015 年 ESC/ERS PH 指南提出的简化风险分层模型，该模型包括世界卫生组织（WHO）功能分级、6 分钟步行试验、氨基末端脑钠肽前体（NT-proBNP）血浆水平、心包积液、右心房压（RAP）、心指数（CI）和混合静脉血氧饱和度（SvO）。风险等级定义如下：低危=至少 3 个低危变量且无高危变量；高危=至少 2 个高危变量，包括 SvO 或 CI；中危=不满足低危或高危的上述定义。研究终点为 3 年全因死亡率。
基线评估时，20 例患者被定义为低危组，30 例患者被归类为中高危合并组。低危组的 20 例患者在随访中均保持在低危组，中高危合并组的 20 例患者降级为低危组，8 例患者在随访评估时仍处于中高危合并组。中高危合并组患者在基线时的 3 年死亡率高于低危组（26%比 14%，P=0.0384）。与仍处于中高危合并组的患者相比，降级为低危组的患者 3 年死亡率较低（P=0.0281）。
基于 2015 年 ESC/ERS PH 指南的简化风险分层模型有助于识别 CTD-PAH 患者的不良长期预后，可用于评估 CTD-PAH 患者的严重程度和治疗反应。
本研究表明，2015 年 ESC/ERS 风险分层模型的简化版本可以帮助诊断时和治疗开始后识别预后不良的中国 CTD-PAH 患者。
