Oslo University Hospital, Rikshospitalet, and University of Oslo, Oslo, Norway.
Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Arthritis Rheumatol. 2015 May;67(8):2205-12. doi: 10.1002/art.39166.
Systemic sclerosis (SSc) carries a high risk of progressive interstitial lung disease (ILD), but tools for stratifying individual risk are scarce. The purpose of this study was to assess detailed data from serial lung fibrosis measurements and paired pulmonary function tests (PFTs) as outcome prediction tools in a prospective cohort of SSc patients.
Paired PFTs and high-resolution computed tomography (HRCT) scans were obtained at baseline and at followup in 305 SSc patients who met the American College of Rheumatology/European League Against Rheumatism 2013 classification criteria. The extent of fibrosis was scored on 10 sections from every HRCT scan and expressed as the percentage of the total lung volume.
Baseline HRCT analyses revealed 3 SSc subgroups: those with >20% lung fibrosis (n = 40), those with 1-20% fibrosis (n = 157), and those with no fibrosis (n = 108). At followup HRCT (mean of 3.1 years later), all 108 group 3 patients were still free of fibrosis. In group 2 patients, 146 continued to have 1-20% fibrosis (group 2a), whereas 11 (marked by short disease duration of 1.3 years) had experienced progression to >20% fibrosis (group 2b). The annual fibrosis progression rate differed across the 4 groups: 0.9% in group 1, 0.7% in group 2a, 5.9% in group 2b, and 0% in group 3. The annual fibrosis progression rate correlated with the total decline in the forced vital capacity (FVC) (7.1%, 5.7%, 8.7%, and 2.9% in groups 1, 2a, 2b, and 3, respectively), but not the diffusing capacity for carbon monoxide (DLco) (8.4%, 7.7%, 7.7%, and 8.6%, respectively). Multivariate analyses identified anticentromere antibodies (odds ratio [OR] 4.7) and baseline DLco (OR 1.04) as predictors of no fibrosis at followup and baseline fibrosis (OR 1.3) and FVC (OR 0.96) as predictors of >20% fibrosis at followup.
These prospective cohort data suggest that HRCT performed at baseline predicts the development of fibrosis, the rate of progression of fibrosis, and the decline in pulmonary function in SSc.
系统性硬化症（SSc）并发进行性间质性肺病（ILD）的风险较高，但个体风险分层的工具却很匮乏。本研究旨在评估 305 例符合美国风湿病学会/欧洲抗风湿病联盟 2013 年分类标准的 SSc 患者的连续肺纤维化测量和配对肺功能测试（PFT）的详细数据，作为前瞻性队列的结局预测工具。
305 例 SSc 患者在基线和随访时均接受了配对的 PFT 和高分辨率计算机断层扫描（HRCT）检查。对每例 HRCT 扫描的 10 个部分进行纤维化程度评分，并表示为肺总量的百分比。
基线 HRCT 分析显示，SSc 患者分为 3 个亚组：纤维化>20%（n=40）、纤维化 1-20%（n=157）和无纤维化（n=108）。在后续 HRCT（平均随访 3.1 年）中，所有 108 例 3 组患者仍无纤维化。在 2 组患者中，146 例继续存在 1-20%的纤维化（2a 组），而 11 例（病程短至 1.3 年）纤维化进展至>20%（2b 组）。4 组的纤维化年进展率不同：1 组为 0.9%，2a 组为 0.7%，2b 组为 5.9%，3 组为 0%。纤维化年进展率与用力肺活量（FVC）的总下降量相关（分别为 1 组 7.1%、2a 组 5.7%、2b 组 8.7%和 3 组 2.9%），但与一氧化碳弥散量（DLco）无关（分别为 8.4%、7.7%、7.7%和 8.6%）。多变量分析发现，抗着丝点抗体（优势比[OR]4.7）和基线 DLco（OR 1.04）是随访时无纤维化的预测因素，基线纤维化（OR 1.3）和 FVC（OR 0.96）是随访时纤维化>20%的预测因素。
这些前瞻性队列数据表明，基线时的 HRCT 可预测 SSc 纤维化的发生、纤维化的进展速度以及肺功能的下降。
