Department of Respiratory Medicine, Gentofte University Hospital, Hellerup, Hovedstaden, Denmark
Centre for Rare Lung Diseases, Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark.
BMJ Open Respir Res. 2022 Jul;9(1). doi: 10.1136/bmjresp-2022-001276.
The diagnosis of idiopathic pulmonary fibrosis (IPF) is often delayed up to several years. The objective of this study was to assess the impact of the diagnostic delay on progression-free survival, quality of life and hospitalisation rates.
A total of 264 incident patients with IPF were included immediately after their diagnosis and followed for up to 5 years, with regular collection of clinical data, quality-of-life questionnaires and assessment of disease progression. Hospitalisation data were extracted from electronic patient records. Analyses were performed on the entire cohort and strata according to forced vital capacity (FVC) at diagnosis.
A long diagnostic delay (>1 year) was associated with worse progression-free survival compared with a short diagnostic delay (<1 year) (HR: 1.70, 95% CI: 1.18 to 2.46, p=0.004) especially in patients with mild disease at the time of diagnosis (FVC>80% predicted). Mean total scores of the St. George's respiratory questionnaire (SGRQ), a derived IPF-specific version of the SGRQ and the chronic obstructive pulmonary disease assessment test (CAT) were consistently higher in patients with long diagnostic delays, indicating worse quality of life. Mean hospitalisation rates were higher during the first year after diagnosis (Incidence rate ratio [IRR]: 3.28, 95% CI: 1.35 to 8.55, p=0.01) and during the entire follow-up (IRR: 1.74, 95% CI: 1.01 to 3.02, p=0.04).
A diagnostic delay of more than 1 year negatively impacts progression-free survival, quality of life and hospitalisation rates in patients with IPF. These findings highlight the importance of an early diagnosis for proper management of IPF.
NCT02755441.
特发性肺纤维化（IPF）的诊断常常延迟数年。本研究旨在评估诊断延迟对无进展生存期、生活质量和住院率的影响。
共纳入 264 例 IPF 新发病例，在诊断后立即进行随访，最长随访 5 年，定期收集临床数据、生活质量问卷和疾病进展评估。住院数据从电子病历中提取。对整个队列和按诊断时用力肺活量（FVC）分层进行分析。
与短时间诊断延迟（<1 年）相比，长时间诊断延迟（>1 年）与无进展生存期较差相关（HR：1.70，95%CI：1.18 至 2.46，p=0.004），特别是在诊断时疾病较轻的患者（FVC>80%预计值）。圣乔治呼吸问卷（SGRQ）、SGRQ 的衍生 IPF 特定版本和慢性阻塞性肺疾病评估测试（CAT）的总评分在诊断延迟较长的患者中始终较高，表明生活质量较差。诊断后第一年的平均住院率较高（发病率比 [IRR]：3.28，95%CI：1.35 至 8.55，p=0.01），整个随访期间的平均住院率也较高（IRR：1.74，95%CI：1.01 至 3.02，p=0.04）。
诊断延迟超过 1 年对 IPF 患者的无进展生存期、生活质量和住院率产生负面影响。这些发现强调了早期诊断对适当管理 IPF 的重要性。
NCT02755441。
