Dipartimento Cardio-Toraco-Vascolare, University of Milan-Bicocca, Respiratory Unit, San Gerardo Hospital, Monza, Italy.
Department of Radiology, Mayo Clinic, Rochester, MN, USA.
Respir Med. 2018 Sep;142:53-59. doi: 10.1016/j.rmed.2018.07.014. Epub 2018 Jul 25.
Whether current diagnostic computed tomography (CT) patterns in idiopathic pulmonary fibrosis (IPF) represent distinct clinical phenotypes or simply temporal evolution of the same underlying radiologic process is unknown. We studied IPF patients presenting with 'possible' or 'inconsistent' usual interstitial pneumonia (UIP) CT patterns and characterized the frequency and timing of evolution to 'consistent' UIP and its effect on survival.
Consecutive IPF patients seen at our institution from 1/1/2005 through 12/31/2013 were assessed for study inclusion. Presenting and serial CT scans were reviewed by two expert radiologists. Baseline and interval clinical data were collated.
Ninety one patients (mean age 67.4 years, 59% male) met study criteria with 'possible' and 'inconsistent' UIP CT patterns present in 58 (64%) and 33 (36%) cases, respectively. Twenty nine (32%) transitioned to a 'consistent' UIP pattern over a median of 57 months (interquartile range 33-78 months). Decline in pulmonary function was statistically significant on interval follow-up for those with or without pattern evolution, but no different between them in terms of degree. Evolution to 'consistent' UIP did not confer worse survival from the date of disease diagnosis or date of first CT with 'consistent' UIP pattern.
A portion of IPF patients with initial 'possible' or 'inconsistent' UIP CT pattern will go on to develop 'consistent' UIP CT pattern over months to years. Despite this, there appeared to be similar risk and cause of death in those with or without pattern evolution, suggesting similar morbidity across the radiologic spectrum in IPF.
特发性肺纤维化（IPF）患者的现行诊断计算机断层扫描（CT）模式是代表不同的临床表型，还是同一潜在放射学过程的时间演变尚不清楚。我们研究了表现为“可能”或“不一致”寻常型间质性肺炎（UIP）CT 模式的 IPF 患者，并描述了向“一致”UIP 演变的频率和时间及其对生存的影响。
对我院 2005 年 1 月 1 日至 2013 年 12 月 31 日期间就诊的连续 IPF 患者进行评估以确定是否符合纳入研究标准。由两位专家放射科医生对患者的首发和系列 CT 扫描进行评估。整理基线和间隔临床数据。
91 例患者（平均年龄 67.4 岁，59%为男性）符合研究标准，其中 58 例（64%）和 33 例（36%）患者分别存在“可能”和“不一致”UIP CT 模式。在中位数为 57 个月（33-78 个月）的时间内，有 29 例（32%）转变为“一致”UIP 模式。在有或没有模式演变的患者中，随访期间的肺功能下降均具有统计学意义，但在程度上没有差异。从疾病诊断之日或首次出现“一致”UIP 模式的 CT 之日起，向“一致”UIP 模式演变并没有导致更差的生存。
部分初始“可能”或“不一致”UIP CT 模式的 IPF 患者在数月至数年内将发展为“一致”UIP CT 模式。尽管如此，在有或没有模式演变的患者中，其死亡风险和原因似乎相似，提示在 IPF 的放射学谱中存在相似的发病率。
