Sauer Gesa M, Käs Florian, Mihai Carmen-Marina, Elhai Muriel, Dobrota Rucsandra, Becker Mike O, Muraru Sinziana, Hoffmann-Vold Anna-Maria, Distler Oliver, Bruni Cosimo
Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Department of Rheumatology, Oslo University Hospital, University of Zurich, Oslo, Norway.
Rheumatology (Oxford). 2025 Aug 1. doi: 10.1093/rheumatology/keaf410.
Combined pulmonary fibrosis and emphysema (CPFE) predicts unfavourable outcomes in systemic sclerosis (SSc). CPFE and emphysema are associated with pulmonary function tests (PFTs) abnormalities. As screening algorithms for pulmonary hypertension include PFTs, we aimed to assess whether CPFE and emphysema affect the performance of the DETECT algorithm to select patients for right heart catheterization (RHC).
SSc patients from our referral center, with available chest tomography images to identify emphysema or interstitial lung disease (ILD) and data to calculate the DETECT score were included. Baseline visit was set as the first visit with available information. Patients with any form of pulmonary hypertension at baseline were excluded. We tested the association of CPFE (and ILD and emphysema separately) with the DETECT score, DETECT score positivity and false positivity, using regression models adjusted for selected covariates.
Among 550 eligible cases, ILD was detected in 232 (42%) and emphysema in 59 (11%) patients, resulting in a 7% prevalence of CPFE. The DETECT score was higher and on average above the threshold for referral to RHC in emphysema and CPFE patients. After adjustment, both emphysema and CPFE predicted positive DETECT scores, while ILD did not. Nevertheless, we found no association between CPFE nor emphysema with false positive DETECT scores.
In SSc, CPFE and emphysema are associated with higher and more frequently positive DETECT score. As neither CPFE nor emphysema were associated with false positive DETECT results, our data support the validity of the DETECT algorithm also in these SSc subgroups.
合并性肺纤维化和肺气肿（CPFE）可预测系统性硬化症（SSc）的不良预后。CPFE和肺气肿与肺功能测试（PFT）异常相关。由于肺动脉高压的筛查算法包括PFT，我们旨在评估CPFE和肺气肿是否会影响DETECT算法在选择患者进行右心导管检查（RHC）时的性能。
纳入来自我们转诊中心的SSc患者，这些患者有可用的胸部断层扫描图像以识别肺气肿或间质性肺疾病（ILD），并有数据可计算DETECT评分。将基线访视设定为首次有可用信息的访视。排除基线时患有任何形式肺动脉高压的患者。我们使用针对选定协变量进行调整的回归模型，测试了CPFE（以及分别的ILD和肺气肿）与DETECT评分、DETECT评分阳性和假阳性之间的关联。
在550例符合条件的病例中，232例（42%）检测到ILD，59例（11%）检测到肺气肿，CPFE患病率为7%。DETECT评分在肺气肿和CPFE患者中更高，且平均高于转诊至RHC的阈值。调整后，肺气肿和CPFE均预测DETECT评分阳性，而ILD则不然。然而，我们发现CPFE和肺气肿与DETECT评分假阳性之间均无关联。
在SSc中，CPFE和肺气肿与更高且更频繁的DETECT评分阳性相关。由于CPFE和肺气肿均与DETECT结果假阳性无关，我们的数据支持DETECT算法在这些SSc亚组中的有效性。
