Forced vital capacity, total lung capacity and diffusion capacity in idiopathic pulmonary fibrosis

Objective. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease characterized by predominant upper lobe pulmonary fibrosis of unknown etiology. However, the prognosis of IPPFE has not been discussed. We investigated the clinical characteristics and prognostic factors of IPPFE and idiopathic pulmonary fibrosis (IPF). Methods. We performed a retrospective cohort study on 375 consecutive idiopathic interstitial pneumonia patients between April 2004 and December 2014. Among them, we diagnosed IPPFE and IPF patients using high-resolution computed tomography radiological criteria. Results. Twenty-nine IPPFE patients (9 males, 20 females) and 67 IPF patients (54 males, 13 females) were enrolled. IPPFE patients were significantly more likely to be females and nonsmokers and had lower body mass index, lower values of predicted percentage of forced vital capacity (%FVC), and a higher residual volume-to-total lung capacity ratio than IPF patients. Survival analysis revealed that they had significantly poorer prognosis than IPF patients in GAP (gender, age, and physiology) stages II + III. %FVC and GAP index independently predict mortality in patients with IPPFE. Conclusions. Patients with IPPFE showed poorer prognosis in the advanced stage than patients with IPF. %FVC and GAP index are independent predictors of survival in patients with IPPFE.

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Prognostic impact of malignant diseases in idiopathic pulmonary fibrosis

Scientific Reports ◽

10.1038/s41598-020-75276-2 ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Hong Yeul Lee ◽

Jaeyoung Cho ◽

Nakwon Kwak ◽

Jinwoo Lee ◽

Young Sik Park ◽

...

Keyword(s):

Lung Cancer ◽

Carbon Monoxide ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Medical Records ◽

Vital Capacity ◽

Prognostic Impact ◽

Malignant Diseases ◽

Diffusion Capacity ◽

And Diffusion

Abstract No studies on idiopathic pulmonary fibrosis (IPF) have investigated the prognostic impact of extrapulmonary cancers in patients with IPF. We aimed to determine the prognostic impact of malignancies in patients with IPF. We retrospectively reviewed the medical records of patients diagnosed with IPF between 2001 and 2015. Patients were divided into three groups: IPF without cancer (n = 440), IPF with lung cancer (n = 69), and IPF with extrapulmonary cancer (n = 70). Of the 579 patients with IPF, 139 (24%) had cancer; the three most common types were lung (11.9%), gastric (2.4%), and colorectal (1.9%). Survival was significantly worse in patients with lung cancer than in those without cancer (hazard ratio [HR] = 1.83, 95% confidence interval [CI], 1.35–2.48) or those with extrapulmonary cancer (HR = 1.70, 95% CI, 1.14–2.54). The rate of hospitalisation for cancer-related complications was significantly higher in IPF patients with lung cancer than in those with extrapulmonary cancer. The annual rates of decline in percent predicted forced vital capacity and diffusion capacity for carbon monoxide did not differ among the groups. Physicians should pay attention to the development and progression of cancer and its prognostic impact in patients with IPF.

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Effect of nintedanib on decline in forced vital capacity (FVC) over time in the INPULSIS trials in patients with idiopathic pulmonary fibrosis (IPF)

Pneumologie ◽

10.1055/s-0037-1598505 ◽

2017 ◽

Vol 71 (S 01) ◽

pp. S1-S125

Author(s):

S Gläser ◽

TM Maher ◽

KR Flaherty ◽

A Azuma ◽

V Cottin ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity ◽

Over Time

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Analysis of patients with idiopathic pulmonary fibrosis (IPF) with percent predicted forced vital capacity (FVC) < 50% treated with pirfenidone in RECAP

Pneumologie ◽

10.1055/s-0037-1598500 ◽

2017 ◽

Vol 71 (S 01) ◽

pp. S1-S125

Author(s):

U Costabel ◽

C Albera ◽

KU Kirchgaessler ◽

F Gilberg ◽

U Petzinger ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity

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Quantitative CT analysis using functional imaging is superior in describing disease progression in idiopathic pulmonary fibrosis compared to forced vital capacity

Respiratory Research ◽

10.1186/s12931-018-0918-5 ◽

2018 ◽

Vol 19 (1) ◽

Cited By ~ 8

Author(s):

J. Clukers ◽

M. Lanclus ◽

B. Mignot ◽

C. Van Holsbeke ◽

J. Roseman ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Disease Progression ◽

Functional Imaging ◽

Forced Vital Capacity ◽

Vital Capacity ◽

Quantitative Ct ◽

Ct Analysis ◽

Quantitative Ct Analysis

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Long-term Effectiveness of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Is Independent of Baseline Forced Vital Capacity

CHEST Journal ◽

10.1016/j.chest.2016.08.552 ◽

2016 ◽

Vol 150 (4) ◽

pp. 538A ◽

Cited By ~ 1

Author(s):

Paul Noble ◽

Carlo Albera ◽

Klaus-Uwe Kirchgaessler ◽

Frank Gilberg ◽

Ute Petzinger ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Vital Capacity

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Impact of Lung Biopsy on Lung Function in Idiopathic Pulmonary Fibrosis

Respiration ◽

10.1159/000509557 ◽

2020 ◽

pp. 1-8

Author(s):

Pierre-Henri Aussedat ◽

Nader Chebib ◽

Kais Ahmad ◽

Jean-Charles Glerant ◽

Gabrielle Drevet ◽

...

Keyword(s):

Lung Function ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Forced Vital Capacity ◽

Lung Biopsy ◽

Vital Capacity ◽

Forced Expiratory Volume ◽

Surgical Lung Biopsy ◽

Before And After ◽

The Impact

Background: Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). Objectives: The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. Methods: This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV1), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. Results: In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (p < 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV1, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC <50% at baseline. Conclusion: In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. Summary at a Glance: This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.

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