Department of Internal Medicine V, Comprehensive Pneumology Centre (CPC-M), Member of the German Centre for Lung Research (DZL), Ludwig-Maximilian University Munich, Marchioninistrasse 15, 81377, Munich, Germany.
IBE - Institute for Medical Information Processing, Biometry and Epidemiology, Ludwig-Maximilian University Munich, Munich, Germany.
Lung. 2018 Aug;196(4):401-408. doi: 10.1007/s00408-018-0123-9. Epub 2018 May 14.
Idiopathic pulmonary fibrosis (IPF) is considered a disease of older patients, being rare in patients ≤ 50 years. Still, IPF can occur in younger patients, but this particular patient group is not well characterised so far. The aim of this study was to compare the diagnostic certainty, clinical features, comorbidities and survival in young versus older IPF patients.
We reviewed our medical records from February 2011 until February 2015, to identify IPF patients, who were then classified as young (≤ 50 years) or older IPF (> 50 years). Radiographic and histological findings, lung function parameters, comorbidities, disease progression and survival were analysed and compared between the two groups.
Of 440 patients with interstitial lung disease, 129 patients with IPF were identified, including 30 (23.3%) ≤50 years and 99 (76.7%) > 50 years. There were no differences between age groups in baseline demographics; younger patients were less likely to have a confirmed diagnosis by high-resolution computed tomography (p = 0.014), more likely to require a biopsy (p = 0.08) and less likely to have received antifibrotic therapy (p = 0.006). Despite an overall limited prognosis, younger patients had a significantly better median survival after diagnosis (p = 0.0375), with a significantly higher proportion of older patients dying due to respiratory failure (p = 0.0383).
IPF patients under the age of 50 years have similar features and clinical course compared to older IPF patients. These patients should be diagnosed by adopting a multidisciplinary team approach, potentially benefitting from earlier intervention with effective antifibrotic therapy.
特发性肺纤维化（IPF）被认为是一种老年患者的疾病，在≤50 岁的患者中很少见。尽管如此，IPF 仍可发生在年轻患者中，但迄今为止，这一特定患者群体尚未得到充分描述。本研究旨在比较年轻与老年 IPF 患者的诊断确定性、临床特征、合并症和生存率。
我们回顾了 2011 年 2 月至 2015 年 2 月期间的病历，以确定 IPF 患者，然后将其分为年轻（≤50 岁）或老年 IPF（>50 岁）。比较两组之间的影像学和组织学发现、肺功能参数、合并症、疾病进展和生存率。
在 440 例间质性肺病患者中，有 129 例被诊断为 IPF，其中 30 例（23.3%）年龄≤50 岁，99 例（76.7%）年龄>50 岁。两组患者的基线人口统计学特征无差异；年轻患者通过高分辨率计算机断层扫描（HRCT）确诊的可能性较小（p=0.014），更有可能需要进行活检（p=0.08），且接受抗纤维化治疗的可能性较小（p=0.006）。尽管总体预后有限，但年轻患者在诊断后的中位生存期明显更长（p=0.0375），而更多的老年患者死于呼吸衰竭（p=0.0383）。
年龄在 50 岁以下的 IPF 患者与老年 IPF 患者具有相似的特征和临床过程。这些患者应通过多学科团队方法进行诊断，可能受益于更早地采用有效的抗纤维化治疗进行干预。
