Department of Rheumatology.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Am J Respir Crit Care Med. 2019 Nov 15;200(10):1258-1266. doi: 10.1164/rccm.201903-0486OC.
Interstitial lung disease (ILD) represents a major challenge in systemic sclerosis (SSc), but there are no precise, population-based data on its overall impact, limiting opportunities for screening and management strategies. Evaluate impact of ILD in a unique, nationwide, population-based SSc cohort. ILD was assessed prospectively in the Norwegian SSc (Nor-SSc) cohort, including all 815 patients with SSc resident in the country from 2000 to 2012. Lung high-resolution computed tomography (HRCT) scans were available for fibrosis quantification at baseline ( = 650, 80%) and follow-up. Pulmonary function tests were assessed at baseline ( = 703, 86%) and follow-up. Vital status and standardized mortality ratios (SMRs) were estimated at study end (2018) in the 630 incident Nor-SSc cases and 15 individually matched control subjects. Cumulative survival rates were computed. At baseline, 50% of the subjects with SSc ( = 324) had ILD by HRCT and 46% displayed pulmonary function declines consistent with ILD progression. Mortality correlated with extent of lung fibrosis as SMR increased from 2.2 with no fibrosis to 8.0 with greater than 25% fibrosis. SMR was inversely related to baseline FVC% and increased at all FVC levels below 100%. In patients with normal-range baseline FVC (80-100%), the 5- and 10-year survival rates correlated with presence or absence of lung fibrosis, being 83% and 80%, respectively, with no fibrosis and 69% and 56%, respectively, with lung fibrosis ( = 0.03). The mere presence of ILD at baseline appears to affect outcome in SSc, suggesting that all patients with SSc should undergo a baseline pulmonary function test and lung HRCT screening to diagnose ILD early and tailor further management.
间质性肺病 (ILD) 是系统性硬化症 (SSc) 的主要挑战，但由于缺乏针对其总体影响的精确、基于人群的数据，限制了筛查和管理策略的机会。在一个独特的、全国性的、基于人群的 SSc 队列中评估 ILD 的影响。ILD 在挪威 SSc（Nor-SSc）队列中进行了前瞻性评估，包括 2000 年至 2012 年期间居住在该国的所有 815 例 SSc 患者。在基线（ = 650，80%）和随访时进行肺部高分辨率计算机断层扫描（HRCT）扫描以评估纤维化程度。在基线（ = 703，86%）和随访时评估肺功能测试。在研究结束时（2018 年），在 630 例新确诊的 Nor-SSc 病例和 15 名个体匹配的对照中估计了生存状态和标准化死亡率比（SMR）。计算累积生存率。在基线时，50%的 SSc 患者（ = 324）通过 HRCT 显示 ILD，46%的患者显示与 ILD 进展一致的肺功能下降。死亡率与肺纤维化程度相关，SMR 从无纤维化的 2.2 增加到大于 25%纤维化的 8.0。SMR 与基线 FVC%呈负相关，在低于 100%的所有 FVC 水平下均增加。在基线 FVC（80-100%）正常范围内的患者中，5 年和 10 年生存率与是否存在肺纤维化相关，无纤维化的生存率分别为 83%和 80%，肺纤维化的生存率分别为 69%和 56%（ = 0.03）。基线时仅存在 ILD 似乎会影响 SSc 的结局，这表明所有 SSc 患者都应进行基线肺功能测试和肺部 HRCT 筛查，以早期诊断 ILD，并调整进一步的管理。
