Tackling the challenges of systemic sclerosis-associated pulmonary hypertension: one step forward.
Tackling the challenges of systemic sclerosis-associated pulmonary hypertension: one step forward.
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应对系统性硬化症相关肺动脉高压的挑战：向前迈出一步。
DOI：
10.1002/art.38031
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发表时间：
2013
影响因子：
--
通讯作者：
Shafiq,Majid
中科院分区：
文献类型：
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作者：
Hassoun,PaulM;Shafiq,Majid
无论是否与毛细血管前肺血管疾病（肺动脉高压[PAH]）或间质性肺病（ILD）相关，系统性硬化症（SSc）相关的肺动脉高压（PH）都是SSc患者死亡的主要原因之一（1）。Lefevre及其同事进行的系统综述和荟萃分析（见本期《关节炎与风湿病》的其他部分）涉及一个重要而棘手的临床问题。作者对所有可用的队列研究（n 22）进行了荟萃分析，其中报告了SSc相关PH的生存和/或预后因素，并计算了总计2，244例患者的汇总生存率。他们还检查了决定生存的预后因素，以及生存是否根据所考虑的时代而变化。这些结果清楚地代表了我们对SSc相关PH的不断增长的理解的有用补充。本研究在很大程度上扩展了之前的单中心观察结果（3，4）以及大型登记研究的结果（5，6），这些结果表明该人群的生存率通常较差，ILD相关PH患者的3年生存率低于PAH患者（7，8）。结果还证实了先前已知的预后因素在这种疾病中的价值，例如PAH诊断时的年龄、肺血管阻力（PVR）和心包积液（9）。然而，Lefevre et al还指出，当汇总几项研究的数据时，以前认为在特发性PAH中重要但在SSc相关PH中不重要的特定预后标志物（如右心房压力和6分钟步行距离）具有预后意义（10）。因此，目前的研究似乎消除了一些以前持有的误解，例如6分钟步行试验作为该人群预后因素的有限价值（11）。虽然这项研究的结果总体上没有特别的惊喜，但作者对不同研究中合并的患者进行了深入的分析和仔细的表型分析，特别是他们对SSc相关PAH和ILD相关PH的谨慎区分，值得称赞。生存数据显示为这些明显不同的综合征的合并结果和单独结果。当汇总结果时，结果的异质性在很大程度上可以通过ILD的存在来解释。因此，该荟萃分析证实了之前几项小型研究（8，12）提出的重要发现，即这两种实体是不同的，并且合并ILD或PH的SSc患者的生存率显著低于合并PAH的SSc患者。然而，关于这些发现的一个警告是，在这两种SSc综合征之间做出明确区分并不总是容易的，因为大多数患者可能有一定程度的间质纤维化。因此，尽管一些患者可能很容易被描述为主要为PAH特征或主要为ILD相关PH特征，但其他患者可能更难以分类（例如，患者的总肺容量为60%的预测值，并且在成像上具有一定程度的间质纤维化）。和PVR是SSc相关PAH患者生存期的显著预测因子，但在ILD患者中并非如此，其中单次呼吸一氧化碳弥散量（DLCO）（和心包积液）是生存期的更强预测因子。这些结果表明，与特发性PAH一样，血管损伤程度是SSc并发PAH患者生存的主要决定因素。DLCO（而不是血流动力学因素）的减少作为预测...
Whether it is related to precapillary pulmonary vascular disease (pulmonary arterial hypertension [PAH]) or interstitial lung disease (ILD), systemic sclerosis (SSc)–associated pulmonary hypertension (PH) is one of the leading causes of death in patients with SSc (1). The systematic review and meta-analysis conducted by Lefevre and colleagues, which appears elsewhere in this issue of Arthritis & Rheumatism (2), deals with an important and vexing clinical issue. The authors performed a meta-analysis of all available cohort studies (n 22) in which survival and/or prognostic factors in SSc-associated PH were reported and calculated pooled survival rates for a total of 2,244 patients. They also examined prognostic factors determining survival and whether survival varied according to the epoch considered. The results clearly represent a useful addition to our growing understanding of SSc-associated PH. This study largely extends previous single-center observations (3, 4) as well as findings from large registries (5, 6), which suggest that survival in this population is generally poor, with worse 3-year survival in patients with ILD-associated PH compared with that in patients with PAH (7, 8). The results also confirm the value of previously known prognostic factors in this disease, such as age at the time of PAH diagnosis, pulmonary vascular resistance (PVR), and pericardial effusion (9). However, Lefevre et al also note that specific prognostic markers previously thought to be important in idiopathic PAH (10) but not in SSc-related PH, such as right atrial pressure and the distance covered in a 6-minute walk, had prognostic significance when the data from several studies were pooled. Thus, the current study seems to dispel some previously held misconceptions, such as the limited value of a 6-minute walk test as a prognostic factor in this population (11). Although the findings of this study overall offer no particular surprises, the authors should be commended for their in-depth analysis and careful phenotyping of patients pooled from different studies, particularly their cautious distinction between SSc-associated PAH and ILD-related PH. The survival data were presented as both pooled results and separate results for these clearly different syndromes. When the results were pooled, the heterogeneity of the results was largely explained by the presence of ILD. Thus, this metaanalysis confirms the important finding suggested by several previous smaller studies (8, 12), that these 2 entities are distinct, and that survival among patients with SSc complicated by ILD or PH is significantly worse than that among patients with SSc complicated by PAH. A word of caution about these findings, however, is that it is not always easy to make a clear distinction between these 2 SSc syndromes, because most patients might have some degree of interstitial fibrosis. Therefore, although some patients may easily be characterized as having a predominantly PAH profile or a predominantly ILD-related PH profile, others may be more difficult to categorize (eg, a patient with total lung capacity of 60% predicted and some degree of interstitial fibrosis on imaging).Hemodynamic parameters such as right atrial pressure, the cardiac index, and PVR were significant predictors of survival among patients with SSc-related PAH but not in those with ILD, in whom single-breath diffusing capacity for carbon monoxide (DLCO)(and pericardial effusion) was a stronger predictor of survival. These results suggest that the degree of vascular impairment is the main determinant of survival in SSc complicated by PAH, as it is in idiopathic PAH. The decrease in DLCO (rather than hemodynamic factors) as a predictor …