The prevalence of pulmonary fibrosis combined with emphysema in patients with lung cancer

Background and objective: Combined pulmonary fibrosis and emphysema (CPFE) is a unique disorder of the upper lobe, whereas emphysema is usually associated with lower lobe fibrosis. Although CPFE might increase the risk of lung cancer, the prevalence of CPFE in patients with lung cancer and the incidence of lung cancer in patients with CPFE are unknown. The objective of this study was to determine the prevalence of CPFE in lung cancer patients and to assess the clinical features of these patients. Methods: A total of 1143 patients with lung cancer were reviewed. Based on HRCT performed at diagnosis of lung cancer, patients were categorized into four groups: normal, emphysema, fibrosis and CPFE. The clinical characteristics of patients with CPFE were compared with those of the other groups. Results: CPFE, emphysema and fibrosis were identified in 101 (8.9%), 404 (35.3%) and 15 (1.3%) patients with lung cancer, respectively. The median overall survival of CPFE patients (n = 101, 10.8 months) was significantly less than that of normal patients (n = 623, 53.0 months) or that of patients with emphysema alone (n = 404, 21.9 months). Acute lung injury occurred in 20 (19.8%) patients with CPFE. Conclusions: CPFE is more prevalent than fibrosis in patients with lung cancer, and patients with CPFE had a poorer prognosis in the present study. Further investigation is therefore necessary to elucidate whether CPFE is an independent risk factor for lung cancer.     

Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema

Background and objective

This study evaluated whether patients with combined pulmonary fibrosis and emphysema (CPFE) have an increased likelihood of pulmonary hypertension (PHT) when compared with idiopathic pulmonary fibrosis (IPF) patients without emphysema.

Methods

Two consecutive IPF populations having undergone transthoracic echocardiography were examined (n = 223 and n = 162). Emphysema and interstitial lung disease (ILD) extent were quantified visually; ILD extent was also quantified by a software tool, CALIPER. Echocardiographic criteria categorized PHT risk.

Results

The prevalence of an increased PHT likelihood was 29% and 31% in each CPFE cohort. Survival at 12 months was 60% across both CPFE cohorts with no significantly worsened outcome identified when compared with IPF patients without emphysema. Using logistic regression models in both cohorts, total computed tomography (CT) disease extent (ILD and emphysema) predicted the likelihood of PHT. After adjustment for total disease extent, CPFE had no stronger association with PHT likelihood than IPF patients without emphysema.

Conclusion

Our findings indicate that the reported association between CPFE and PHT is explained by the summed baseline CT extents of ILD and emphysema. Once baseline severity is taken into account, CPFE is not selectively associated with a malignant microvascular phenotype, when compared with IPF patients without emphysema.

     

Clinical characteristics of combined pulmonary fibrosis and emphysema

Background and objective: Patients with combined pulmonary fibrosis and emphysema (CPFE) are sometimes seen, and we speculate that these patients have some different clinical characteristics from COPD patients. This study clarifies the clinical characteristics of CPFE patients. Methods: This was a retrospective study of 47 stable patients with concurrent emphysema and diffuse parenchymal lung disease with fibrosis, based on the findings of chest CT (CPFE patients). The clinical characteristics of CPFE patients were compared with those of emphysema‐dominant COPD patients without parenchymal lung disease (COPD patients). Results: Forty‐six of the 47 CPFE patients were male. Paraseptal emphysema was particularly common in the CPFE group. Honeycombing, ground‐glass opacities and reticular opacities were present in 75.6%, 62.2% and 84.4% of CPFE patients, respectively. Twenty‐two of the 47 CPFE patients (46.8%) had lung cancer. Pulmonary function tests showed that the CPFE group had milder airflow limitation and lower diffusing capacity than the COPD group. Desaturation during 6‐min walking test in CPFE patients tended to be more severe than in COPD patients, if the level of FEV₁ or 6MWD was equal. Conclusions: CPFE patients had some different clinical characteristics in comparison with COPD patients and may also have a high prevalence of lung cancer.     

多层螺旋CT肺密度测定与肺气肿患者肺功能的相关性研究

目的探讨多层螺旋CT肺密度测定与肺气肿患者肺功能的相互关系。方法对45例肺气肿患者及40例对照组人员的CT肺密度和肺功能进行测定,并将CT肺密度测定结果与肺功能结果进行对照研究。结果肺气肿患者CT呼、吸气相VD与TLC、FEV1及FEV1/FVC呈显著正相关（P〈0.01）,而与RV/TLC呈显著负相关（P〈0.01）。吸气相全肺MLD与PFT指标无相关性（P〉0.05）。结论对于肺气肿疾病多层螺旋CT肺密度测定与肺功能检测有着密切的关系。     

Sequential occurrence of combined pulmonary fibrosis and emphysema syndrome in a non‐smoker female patient

The combined pulmonary fibrosis and emphysema (CPFE) syndrome is a unique and an under‐recognized disorder characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes of the lung. It occurs predominantly in males and almost exclusively in smokers. This rare combination of a restrictive and an obstructive mechanical defect carries a poorer prognosis than either of the two components. We present a case of CPFE syndrome in a non‐smoker female patient who developed lower lobe emphysema subsequent to development of interstitial fibrosis. The case was remarkable for the extreme rarity of several presenting features, namely, a lower lobe occurrence of emphysema subsequent to pre‐existent interstitial fibrosis, female gender and absence of a history of smoking.     

Clinical and high resolution computed tomography characteristics of patients with rheumatoid arthritis lung disease

Introduction: Rheumatoid arthritis (RA) is a systemic disease of unknown cause. A variety of pulmonary disorders have been described in association with RA. Among the most common are interstitial lung disease (ILD) and bronchiectasis. Objectives: This study aims to determine the characteristics of RA patients with lung disease in relation to clinical characteristics, pulmonary function test (PFT) and high resolution computed tomography (HRCT) thorax. Method: This is a 6‐months cross‐sectional study involving 63 consecutive RA patients in an outpatient rheumatology clinic. Patients had a mean disease duration of 5 years and above. Disease activity and severity was assessed by Disease Activity Score 28 (DAS28), Health Assessment Questionnaire (HAQ) and Rheumatoid Arthritis Articular Damage (RAAD) score. Full pulmonary function test (PFT) and HRCT of thorax were performed. Conclusions: The prevalence of RA‐ILD is 44% and 67% of patients are asymptomatic. There was significant higher proportion (68%) in patients of Chinese ethnicity who have ILD. Diffusion capacity of carbon monoxide was abnormal in all patients and forced expiratory flow (FEF)_25–75% was low in 16%. Restrictive pattern was 66.7% by PFT. The most common HRCT findings were reticulation (46%) followed by ground glass opacities (38.1%) and bronchiectasis (28.6%). There was no association between ILD and male gender, duration of the disease, smoking, rheumatoid factor, extra‐articular manifestations, disease activity or severity.     

特发性肺纤维化患者的肺功能及CT影像学相关性分析

目的研究特发性肺纤维化(IPF)患者的肺功能与螺旋CT影像学诊断的相关性。方法分析40例临床诊断IPF患者的肺功能和螺旋CT检查结果。入选的所有病例均在1周之内行肺功能测定和胸部CT扫描。结果螺旋CT的磨玻璃样密度影与一秒末用力呼气率(FEV1/FVC)有负相关性(轻度);弥漫性线网状阴影与一氧化碳弥散量(DLco)和一氧化碳弥散率(DLco/VA)有显著负相关性;蜂窝影与FVC、FEV1、TLC、DLco和DLco/VA均具有一定的负相关性。结论不同程度的IPF患者肺功能参数与螺旋CT影像学征象存在一定的相关性。两种检查方法联合应用对于提高临床工作中IPF检出率及预测IPF的进展、预后及疗效有一定参考价值。     

Radiographic fibrosis score predicts survival in systemic sclerosis‐associated interstitial lung disease

Background and objective

Interstitial lung disease (ILD ) is a common pulmonary manifestation of systemic sclerosis (SSc ). It is unknown whether radiographic fibrosis score predicts mortality in SSc ‐associated ILD (SSc‐ILD ). We retrospectively analysed patients with SSc‐ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality.

Methods

We identified SSc‐ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high‐resolution computed tomography (HRCT ) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model.

Results

The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT . The median follow‐up period was 56.6 months, and over the follow‐up period 15 patients died. The 5‐year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not.

Conclusion

A high radiographic fibrosis score was a poor prognostic factor in SSc‐ILD . More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.

     

Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis

Background and objective:   Although pulmonary rehabilitation is effective for patients with COPD, its efficacy in patients with IPF is unknown. The purpose of this study was to evaluate the effects of pulmonary rehabilitation in IPF.
Methods:   Thirty patients diagnosed with IPF, according to the consensus statement, were randomly assigned to the rehabilitation group or the control group. The pulmonary rehabilitation mainly consisted of a 10-week programme of exercise training. Pulmonary function, blood gas analysis, 6MWD, dyspnoea rating with the baseline dyspnoea index and health-related quality of life score on the St George's Respiratory Questionnaire were evaluated at baseline and after the programme.
Results:   Assessment of efficacy was carried out on 13 patients who completed the programme and 15 patients in the control group. There were no significant effects of the programme on measures of pulmonary function, values of arterial blood gas analysis or dyspnoea rating. Although there were some differences in the baseline 6MWD and total health-related quality of life score which were not statistically significant, marked improvements were observed in the 6MWD (mean difference 46.3 m (95% CI: 8.3–84.4), P  < 0.05) and the total health-related quality of life score (−6.1 (95% CI: −11.7 to −0.5), P  < 0.05).
Conclusions:   Pulmonary rehabilitation improves both exercise capacity and health-related quality of life in patients with IPF.     

特发性肺纤维化合并肺气肿与未合并肺气肿患者的临床差异

目的探讨单纯特发性肺纤维化（IPF）和肺纤维化合并肺气肿（CPFE）患者的临床差异。方法选取我院2007年8月至2012年8月收治的13例CPFE患者作为观察对象,设为观察组,选择同期13例单纯IPF患者作为对照,设为对照组。对比两组患者的临床资料、PaO2、肺功能、BALF细胞成分等指标和临床用药的差异。结果观察组吸烟患者占92.3%.吸烟指数为（29.7±18. 6）包年,均高于 对照组[53.8%.t=7.89. P〈0.05;（17.8±16.4）包年,t =1.73.P〈0.05J。两组PaO,和肺活 量%pred差异无统计学意义（t值分别为0. 21、1. 29. P值均〉0.0日,观察组FEV,/FVC和DLCO%pred 均低于对照组[（77.9±7.7）%vs （85.6±8. 9） %,t = 2. 36. P 〈 0. 05; （44. 1±12.3） % vs （54.3±16.7） %. t = 1.77, P 〈0. 05J.而肺总量% pred显著高于对照组[（77. 3±12.2） % vs （64.4± 12.4） %. t =2.67. P 〈0. 01]。两组患者BALF所有细胞成分相似,差异均无统计学意义（ P值均〉 0.05） .用药情况基本」致。结论吸烟可能是CPFE患者的重要危险因素.CPFE患者弥散功能显著下降且肺容积异常。     

Multiple breath washout: A new and promising lung function test for patients with idiopathic pulmonary fibrosis

Background and objective

Idiopathic pulmonary fibrosis (IPF) is a devastating progressive lung disease affecting the parenchyma. Nitrogen multiple‐breath washout (N₂‐MBW) is a lung function test that measures ventilation inhomogeneity, a biomarker of small airway disease. We assessed clinical properties of N₂‐MBW in IPF.

Methods

In this prospective cohort pilot study, 25 IPF patients and 25 healthy controls were assessed at baseline and 10 patients at median 6.2 months later. Outcomes included the lung clearance index (LCI) from N₂‐MBW, forced vital capacity (FVC) from spirometry, diffusion capacity of the lungs for carbon monoxide (DL_CO), bronchiectasis score from computed tomography scans, the Gender–Age–Physiology (GAP score for IPF) stage and death or lung transplantation (LTx). Study end points were feasibility, repeatability, discriminative capacity and correlation with disease severity and structural lung damage.

Results

All patients were able to perform N₂‐MBW. LCI was repeatable and reproducible. Median (interquartile range (IQR)) LCI in IPF was 11.6 (10.1–13.8) in IPF versus 7.3 (6.9–8.4) in controls (P < 0.0001). LCI correlated with DL_CO corrected for haemoglobin (corrDL_CO; r = −0.49, P = 0.016), bronchiectasis score (r = 0.45, P = 0.024) and the GAP stage (r = 0.59, P = 0.002), but not with FVC. FVC was not related to bronchiectasis. During follow‐up, six patients died and one received LTx. LCI correlated with the latter compound outcome: hazard ratio (95% CI) was 2.43 (1.26; 4.69) per one LCI SD from the patient population.

Conclusion

N₂‐MBW is a feasible, reliable and valid lung function test in IPF. LCI correlates with diffusion impairment, structural airway damage and clinical disease severity. LCI is a promising surveillance tool in IPF that may predict mortality.

     

Cytokine profile of bronchoalveolar lavage fluid in patients with combined pulmonary fibrosis and emphysema

Background and objective: Combined pulmonary fibrosis and emphysema (CPFE) is characterized by upper lobe emphysema together with lower lobe fibrosis. The aim of this study was to examine whether cytokine levels in the alveolar space are associated with emphysematous changes superimposed on pulmonary fibrosis. Methods: Consecutive patients (n = 102), diagnosed with pulmonary fibrosis were retrospectively evaluated. Cytokine levels and differential cell counts in bronchoalveolar lavage (BAL) fluid, pulmonary function, computed tomography (CT) scores and levels of serum markers were compared between patients with or without emphysema. Results: Among the 102 patients (14 females, mean age 68 years), 38 (37%) had evidence of upper lobe emphysema on computed tomography (CT). Levels of epithelial neutrophil activating peptide 78 (ENA‐78/CXCL5) and interleukin (IL)‐8/CXCL8 in BAL fluid were significantly higher in patients with emphysema. Vital capacity (VC, % predicted) was greater, and ratio of forced expiratory volume in 1 s/forced vital capacity and diffusing capacity of carbon monoxide (DL_CO)/alveolar volume (V_A) were lower in patients with emphysema. CXCL8 and CXCL5 levels were associated with percentage or absolute numbers of neutrophils in BAL fluid. In addition, CXCL8 levels were inversely correlated with VC and DL_CO/V_A, and positively correlated with composite physiological index (CPI) and the extent of areas of low attenuation on CT. Conclusions: Increased CXC chemokine levels in the airspaces may be associated with emphysematous lung changes in patients with pulmonary fibrosis.     

肺纤维化合并肺气肿综合征肺功能及与高分辨率CT定量相关性分析

目的:分析肺纤维化合并肺气肿(CPFE)综合征患者肺功能及与高分辨率CT(HRCT)定量相关性。方法:本研究为病例对照研究,采用非随机对照的方法收集2011年10月至2021年10月于兰州大学第一医院住院治疗的CPFE综合征患者239例和同期收治的特发性肺纤维化(IPF)患者294例及肺气肿患者287例,根据肺部纤维化...