Division of Pulmonary Critical Care and Sleep Medicine, Brody School of Medicine, East Carolina University, Greenville, NC.
Division of Pulmonary Critical Care and Sleep Medicine, Brody School of Medicine, East Carolina University, Greenville, NC; Department of Bioinformatics and Biostatistics, School of Public Health and Information Sciences, University of Louisville, Louisville, KY.
Chest. 2024 Apr;165(4):892-907. doi: 10.1016/j.chest.2023.10.021. Epub 2023 Oct 23.
Different patterns of fibrosis on high-resolution CT scans (HRCT) have been associated with reduced survival in some interstitial lung diseases. Nothing is known about HRCT scan patterns and survival in sarcoidosis.
Will a detailed description of the extent and pattern of HRCT scan fibrosis in patients with stage IV pulmonary sarcoidosis impact pulmonary function and survival?
Two hundred forty patients with stage IV sarcoidosis at two large tertiary institutions were studied. The earliest HRCT scan with fibrosis was reviewed for extent of fibrosis (< 10%, 10%-20%, and > 20%) and presence of bronchiectasis, upper lobe fibrocystic changes, basal subpleural honeycombing, ground-glass opacities (GGOs), large bullae, and mycetomas. Presence of sarcoidosis-associated pulmonary hypertension (SAPH) and pulmonary function testing performed within 1 year of HRCT were recorded. Patients were followed up until last clinic visit, death, or lung transplantation.
The mean age was 58.4 years. Seventy-four percent were Black, 63% were female, and mean follow-up was 7.4 years. Death or LT occurred in 53 patients (22%). Thirty-one percent had > 20% fibrosis, 25% had 10%-20% fibrosis, and 44% had < 10% fibrosis. The most common HRCT abnormalities were bronchiectasis (76%), upper lobe fibrocystic changes (36%), and GGOs (28%). Twelve percent had basal subpleural honeycombing, and 32% had SAPH. Patients with > 20% fibrosis had more severe pulmonary impairment, were more likely to have SAPH (53%), and had worse survival (44% mortality; P < .001). Upper lobe fibrocystic changes, basal subpleural honeycombing, and large bullae were associated with worse pulmonary function and worse survival. Patients with basal subpleural honeycombing had the worst pulmonary function and survival (55% mortality; P < .001). GGOs were associated with worse pulmonary function but not worse survival, and mycetomas were associated with worse survival but not worse pulmonary function. A Cox proportional hazards model indicated that basal subpleural honeycombing (hazard ratio, 7.95), diffusion capacity for carbon monoxide < 40% (HR, 5.67) and White race (hazard ratio, 2.61) were independent predictors of reduced survival.
HRCT scan features of fibrotic pulmonary sarcoidosis had an impact on pulmonary function and survival. Presence of >20% fibrosis and basal subpleural honeycombing are predictive of worse pulmonary function and worse survival in patients with stage IV pulmonary sarcoidosis.
高分辨率 CT 扫描（HRCT）上不同的纤维化模式与某些间质性肺疾病的生存率降低有关。关于结节病的 HRCT 扫描模式和生存率，目前尚不清楚。
在 IV 期肺结节病患者中，详细描述 HRCT 扫描纤维化的程度和模式是否会影响肺功能和生存？
在两家大型三级医疗机构研究了 240 例 IV 期结节病患者。回顾最早的纤维化 HRCT 扫描，以评估纤维化程度（<10%、10%-20%和>20%）以及支气管扩张、上叶纤维囊性改变、基底胸膜下蜂窝状改变、磨玻璃影（GGO）、大疱和真菌球的存在。记录 HRCT 检查后 1 年内出现的结节病相关肺动脉高压（SAPH）和肺功能检查情况。患者随访至最后一次就诊、死亡或肺移植。
平均年龄为 58.4 岁。74%为黑人，63%为女性，平均随访时间为 7.4 年。53 例（22%）患者死亡或需要进行肺移植。31%的患者纤维化程度>20%，25%的患者纤维化程度为 10%-20%，44%的患者纤维化程度<10%。最常见的 HRCT 异常包括支气管扩张（76%）、上叶纤维囊性改变（36%）和 GGO（28%）。12%的患者有基底胸膜下蜂窝状改变，32%的患者有 SAPH。纤维化程度>20%的患者肺功能损害更严重，SAPH 发生率更高（53%），生存率更差（44%死亡率；P<.001）。上叶纤维囊性改变、基底胸膜下蜂窝状改变和大疱与更差的肺功能和生存率相关。基底胸膜下蜂窝状改变患者的肺功能和生存率最差（55%死亡率；P<.001）。GGO 与更差的肺功能相关，但与生存率无关，真菌球与生存率相关，但与肺功能无关。Cox 比例风险模型表明，基底胸膜下蜂窝状改变（风险比，7.95）、一氧化碳弥散量<40%（HR，5.67）和白种人（HR，2.61）是生存率降低的独立预测因素。
结节病纤维化性肺部 HRCT 扫描特征对肺功能和生存率有影响。IV 期肺结节病患者纤维化程度>20%和基底胸膜下蜂窝状改变与肺功能下降和生存率降低相关。
