scholarly journals Prognostic impact of malignant diseases in idiopathic pulmonary fibrosis

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.

scholarly journals A score without diffusion capacity of the lung for carbon monoxide for estimating survival in idiopathic pulmonary fibrosis

Medicine ◽  
2020 ◽  
Vol 99 (25) ◽  
pp. e20739
Author(s):  
Cesar Yoshito Fukuda ◽  
Maria Raquel Soares ◽  
Carlos Alberto de Castro Pereira
Keyword(s):  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Diffusion Capacity

scholarly journals The efficacy of nintedanib in 158 patients with idiopathic pulmonary fibrosis in real-world settings: A multicenter retrospective study

2021 ◽  
Vol 9 ◽  
pp. 205031212110233
Author(s):  
Masaki Dobashi ◽  
Hisashi Tanaka ◽  
Kageaki Taima ◽  
Masamichi Itoga ◽  
Yoshiko Ishioka ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Real World ◽  
Forced Vital Capacity ◽  
Medical Records ◽  
Vital Capacity ◽  
Comparable Efficacy ◽  
Diffusing Capacity ◽  
Before And After ◽  
Severe Group

Background: The INPULSIS trials revealed that nintedanib reduced the decline in lung function in patients with idiopathic pulmonary fibrosis. We aimed to evaluate the efficacy and safety of nintedanib in Japanese idiopathic pulmonary fibrosis patients in real-world settings. Method: Medical records of idiopathic pulmonary fibrosis patients, who received treatment with nintedanib in five institutions between July 2015 and June 2017, were reviewed. Patients with % forced vital capacity ⩾50% and % predicted diffusing capacity of the lung carbon monoxide ⩾30% were classified as the moderate group and those with more impaired lung functions as the severe group. Result: Among 158 patients analyzed, 132 (84.6%) were classified as the moderate group and 26 (15.4%) as the severe group. In the moderate group, changes in forced vital capacity in 12 months were significantly different between before and after nintedanib administration (−253 ± 163 vs −125 ± 235 mL; p = 0.0027). In contrast, changes in forced vital capacity in 12 months were not significantly changed by nintedanib treatment in the severe group (−353 ± 250 vs −112 ± 341 mL; p = 0.2374). Incidence of acute exacerbation was higher in the severe group than in the moderate group (30.8% vs 18.9%). The overall survival of the moderate and the severe groups was 17.2 and 10.1 months. Conclusion: In real-world practice, nintedanib showed comparable efficacy to those observed in previous trials. In the severe group, the efficacy of nintedanib might be limited.

scholarly journals Progranulin And Activin A Concentrations Are Elevated In Serum From Patients With Acute Exacerbations of Idiopathic Pulmonary Fibrosis

2021 ◽  
Author(s):  
Tian Xie ◽  
Lizhen Han ◽  
Yongxing Chen ◽  
Haihong Wu
Keyword(s):  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Activin A ◽  
Control Group ◽  
Diffusion Capacity ◽  
Arterial Blood Gas ◽  
Arterial Blood ◽  
Acute Exacerbations ◽  
Gas Measurements

Abstract Purpose To examine the serum concentrations of Progranulin (PGRN) and activin A in patients with acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) in a pilot study. Methods Twenty-one patients with AE-IPF were compared with 23 patients with stable idiopathic pulmonary fibrosis (IPF) as a control group. Serum PGRN and activin A levels, arterial blood gas measurements and lung function were determined in these two groups. Students t-test was used to compare the differences in PGRN and activin A levels between the two groups, and Spearman correlation coefficient was used to examine the relationship between serum PGRN and activin A levels with carbon monoxide diffusion capacity in patients with IPF. Results Peripheral blood PGRN and activin A levels in patients with AE-IPF were 83.7+10.0 and 14.2+1.7 ng/ml (mean+SD), respectively, higher than those in the control group 61.0+5.8, and 5.8+1.0 (p<0.001). PGRN and activin A levels were significantly negatively correlated with carbon monoxide diffusion capacity (r=-0.857 p<0.001) and r=-0.757 (p<0.001). Conclusion: PGRN and activin A may be involved in the pathogenesis of AE-IPF. They may be possible markers of activity in AE-IPF.

scholarly journals Functional and prognostic effects when emphysema complicates idiopathic pulmonary fibrosis

2017 ◽  
Vol 50 (1) ◽  
pp. 1700379 ◽  
Author(s):  
Joseph Jacob ◽  
Brian J. Bartholmai ◽  
Srinivasan Rajagopalan ◽  
Maria Kokosi ◽  
Toby M. Maher ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Smoking Status ◽  
Independent Effect ◽  
Gas Transfer ◽  
Prognostic Impact ◽  
Impact On Survival ◽  
Ct Analysis ◽  
Functional Indices

This study aimed to investigate whether the combination of fibrosis and emphysema has a greater effect than the sum of its parts on functional indices and outcome in idiopathic pulmonary fibrosis (IPF), using visual and computer-based (CALIPER) computed tomography (CT) analysis.Consecutive patients (n=272) with a multidisciplinary IPF diagnosis had the extent of interstitial lung disease (ILD) scored visually and by CALIPER. Visually scored emphysema was subcategorised as isolated or mixed with fibrotic lung. The CT scores were evaluated against functional indices forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide (DLCO), transfer coefficient of the lung for carbon monoxide (KCO), composite physiologic index (CPI)) and mortality.The presence and extent of emphysema had no impact on survival. Results were maintained following correction for age, gender, smoking status and baseline severity usingDLCO, and combined visual emphysema and ILD extent. Visual emphysema quantitation indicated that relative preservation of lung volumes (FVC) resulted from tractionally dilated airways within fibrotic lung, ventilating areas of admixed emphysema (p<0.0001), with no independent effect on FVC from isolated emphysema. Conversely, only isolated emphysema (p<0.0001) reduced gas transfer (DLCO).There is no prognostic impact of emphysema in IPF, beyond that explained by the additive extents of both fibrosis and emphysema. With respect to the location of pulmonary fibrosis, emphysema distribution determines the functional effects of emphysema.

scholarly journals Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Myung Jin Song ◽  
Sang Hoon Lee ◽  
Ji Ye Jung ◽  
Young Ae Kang ◽  
Moo Suk Park ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Tertiary Care ◽  
Care Hospital ◽  
Diffusion Capacity ◽  
Autoantibody Status ◽  
The Mean ◽  
One Year ◽  
Positive Groups

Abstract Background Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear. Methods We retrospectively reviewed the medical records of patients with IPF treated at a tertiary care hospital in South Korea. The autoantibody status was defined as positive if we detected autoantibodies meeting the serological domain criteria for interstitial pneumonia with autoimmune features or anti-neutrophil cytoplasmic antibodies. Results We included 142 patients with IPF treated with pirfenidone for over six months (93 were autoantibody-positive and 49 were autoantibody-negative). The mean age was 69.5 ± 7.3 years, and 77.5% of the patients were male. The adjusted mean changes over one year were − 34.4 and − 112.2 mL (p = 0.168) in forced vital capacity (FVC), and − 0.53 and − 0.72 mL/mmHg/min (p = 0.356) in the lungs diffusion capacity for carbon monoxide (DLCO) in the autoantibody-negative and autoantibody-positive groups, respectively. Conclusions Reductions in FVC and DLCO were similar in autoantibody-positive and autoantibody-negative patients with IPF treated with pirfenidone. Pirfenidone is effective in attenuating the progression of IPF, irrespective of the autoantibody status.

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