Department of Respiratory Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.
BMJ Open Respir Res. 2023 Nov;10(1). doi: 10.1136/bmjresp-2023-001864.
Few prospective cohort studies with relatively large numbers of patients with non-idiopathic pulmonary fibrosis (non-IPF) of idiopathic interstitial pneumonia (IIP) have been described. We aimed to assess disease progression and cause of death for patients with non-IPF IIPs or IPF under real-life conditions.
Data were analysed for a prospective multi-institutional cohort of 528 IIP patients enrolled in Japan between September 2013 and April 2016. Diagnosis of IPF versus non-IPF IIPs was based on central multidisciplinary discussion, and follow-up surveillance was performed for up to 5 years after patient registration. Survival and acute exacerbation (AE) were assessed.
IPF was the most common diagnosis (58.0%), followed by unclassifiable IIPs (35.8%) and others (6.2%). The 5-year survival rate for non-IPF IIP and IPF groups was 72.8% and 53.7%, respectively, with chronic respiratory failure being the primary cause of death in both groups. AE was the second most common cause of death for both non-IPF IIP (24.1%) and IPF (23.5%) patients. The cumulative incidence of AE did not differ significantly between the two groups (p=0.36), with a 1-year incidence rate of 7.4% and 9.0% in non-IPF IIP and IPF patients, respectively. We found that 30.2% and 39.4% of non-IPF IIP and IPF patients, respectively, who experienced AE died within 3 months after an AE event, whereas 55.8% and 66.7% of such patients, respectively, died within 5 years after registration.
Closer monitoring of disease progression and palliative care interventions after AE are important for non-IPF IIP patients as well as for IPF patients.
描述了为数不多的几项针对特发性间质性肺炎（IIP）中非特发性肺纤维化（非 IPF）患者的前瞻性队列研究，这些研究的患者数量相对较多。我们旨在评估真实环境下非特发性 IPF 患者和特发性纤维化（IPF）患者的疾病进展和死亡原因。
对 2013 年 9 月至 2016 年 4 月期间在日本入组的 528 例 IIP 患者的前瞻性多机构队列进行数据分析。IPF 与非特发性 IPF 的诊断基于中心多学科讨论，在患者登记后对其进行长达 5 年的随访监测。评估生存和急性加重（AE）情况。
最常见的诊断是特发性纤维化（58.0%），其次是非特发性 IIP（35.8%）和其他（6.2%）。非特发性 IPF 和特发性纤维化组的 5 年生存率分别为 72.8%和 53.7%，两组的主要死亡原因为慢性呼吸衰竭。AE 是非特发性 IPF（24.1%）和特发性纤维化（23.5%）患者的第二大常见死亡原因。两组间 AE 的累积发生率无显著差异（p=0.36），非特发性 IPF 和特发性纤维化患者的 1 年发生率分别为 7.4%和 9.0%。我们发现，30.2%和 39.4%的非特发性 IPF 和特发性纤维化患者分别在 AE 后 3 个月内死亡，而 55.8%和 66.7%的患者分别在登记后 5 年内死亡。
AE 后更密切地监测疾病进展和姑息治疗干预对于非特发性 IPF 患者和特发性纤维化患者同样重要。
