scholarly journals Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Makoto Shioya ◽  
Mitsuo Otsuka ◽  
Gen Yamada ◽  
Yasuaki Umeda ◽  
Kimiyuki Ikeda ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Total Lung Capacity ◽  
Unknown Etiology ◽  
Advanced Stage ◽  
High Resolution Computed Tomography ◽  
Pleuroparenchymal Fibroelastosis ◽  
Radiological Criteria ◽  
Lung Capacity

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.

scholarly journals Automatic quantitative computed tomography measurement of longitudinal lung volume loss in interstitial lung diseases

2022 ◽  
Author(s):  
Salim A. Si-Mohamed ◽  
Mouhamad Nasser ◽  
Marion Colevray ◽  
Olivier Nempont ◽  
Pierre-Jean Lartaud ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Vital Capacity ◽  
Pulmonary Function Tests ◽  
Interstitial Lung Diseases ◽  
Volume Loss ◽  
Lung Capacity ◽  
Lung Ct ◽  
Ct Volume

Abstract Objectives To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and explore a prognostic value of annual CTvol loss in IPF. Methods We conducted in an expert center a retrospective study between 2005 and 2018 on consecutive patients with ILD. CTvol was measured automatically using commercial software based on a deep learning algorithm. In the first group, Spearman correlation coefficients (r) between forced vital capacity (FVC), total lung capacity (TLC), and CTvol were calculated. In a second group, annual CTvol loss was calculated using linear regression analysis and compared with the Mann–Whitney test. In a last group of IPF patients, annual CTvol loss was calculated between baseline and 1-year CTs for investigating with the Youden index a prognostic value of major adverse event at 3 years. Univariate and log-rank tests were calculated. Results In total, 560 patients (4610 CTs) were analyzed. For 1171 CTs, CTvol was correlated with FVC (r: 0.86) and TLC (r: 0.84) (p < 0.0001). In 408 patients (3332 CT), median annual CTvol loss was 155.7 mL in IPF versus 50.7 mL in non-IPF (p < 0.0001) over 5.03 years. In 73 IPF patients, a relative annual CTvol loss of 7.9% was associated with major adverse events (log-rank, p < 0.0001) in univariate analysis (p < 0.001). Conclusions Automated lung CT volume may be an alternative or a complementary biomarker to pulmonary function tests for the assessment of lung volume loss in ILD. Key Points • There is a good correlation between lung CT volume and forced vital capacity, as well as for with total lung capacity measurements (r of 0.86 and 0.84 respectively, p < 0.0001). • Median annual CT volume loss is significantly higher in patients with idiopathic pulmonary fibrosis than in patients with other fibrotic interstitial lung diseases (155.7 versus 50.7 mL, p < 0.0001). • In idiopathic pulmonary fibrosis, a relative annual CT volume loss higher than 9.4% is associated with a significantly reduced mean survival time at 2.0 years versus 2.8 years (log-rank, p < 0.0001).

scholarly journals The airway response to deep inspirations decreases with COPD severity and is associated with airway distensibility assessed by computed tomography

2008 ◽  
Vol 105 (3) ◽  
pp. 832-838 ◽  
Author(s):  
Nicola Scichilone ◽  
Alba La Sala ◽  
Maria Bellia ◽  
Katherine Fallano ◽  
Alkis Togias ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Vital Capacity ◽  
Total Lung Capacity ◽  
Chronic Obstructive ◽  
High Resolution Computed Tomography ◽  
Lung Inflation ◽  
Lung Capacity ◽  
Gold Stage ◽  
Computed Tomography Scans

In patients with mild chronic obstructive pulmonary disease (COPD), the effect of deep inspirations (DIs) to reverse methacholine-induced bronchoconstriction is largely attenuated. In this study, we tested the hypothesis that the effectiveness of DI is reduced with increasing disease severity and that this is associated with a reduction in the ability of DI to distend the airways. Fifteen subjects [Global Initiative for Chronic Obstructive Lung Disease (GOLD) stage I–II: n = 7; GOLD stage III–IV: n = 8] underwent methacholine bronchoprovocation in the absence of DI, followed by DI. The effectiveness of DI was assessed by their ability to improve inspiratory vital capacity and forced expiratory volume in 1 s (FEV1). To evaluate airway distensibility, two sets of high-resolution computed tomography scans [at residual volume (RV) and at total lung capacity] were obtained before the challenge. In addition, mean parenchymal density was calculated on the high-resolution computed tomography scans. We found a strong correlation between the response to DI and baseline FEV1 %predicted ( r2 = 0.70, P < 0.0001) or baseline FEV1/forced vital capacity ( r2 = 0.57, P = 0.001). RV %predicted and functional residual capacity %predicted correlated inversely ( r2 = 0.33, P = 0.02 and r2 = 0.32, P = 0.03, respectively), and parenchymal density at RV correlated directly ( r2 = 0.30, P = 0.03), with the response to DI. Finally, the effect of DI correlated to the change in large airway area from RV to total lung capacity ( r2 = 0.44, P = 0.01). We conclude that loss of the effects of DI is strongly associated with COPD severity and speculate that the reduction in the effectiveness of DI is due to the failure to expand the lungs because of the hyperinflated state and/or the parenchymal damage that prevents distension of the airways with lung inflation.

scholarly journals The similarities and differences between pleuroparenchymal fibroelastosis and idiopathic pulmonary fibrosis

2019 ◽  
Vol 16 ◽  
pp. 147997311986794 ◽  
Author(s):  
Hiroshi Ishii ◽  
Yoshiaki Kinoshita ◽  
Hisako Kushima ◽  
Nobuhiko Nagata ◽  
Kentaro Watanabe
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Vital Capacity ◽  
Marked Decrease ◽  
Clinical Course ◽  
Walking Distance ◽  
Thoracic Cage ◽  
Pleuroparenchymal Fibroelastosis ◽  
Current Classification

The idiopathic form of pleuroparenchymal fibroelastosis (PPFE) is categorized as a rare idiopathic interstitial pneumonia in the current classification. The majority of PPFE cases are idiopathic, but many predisposing factors or comorbidities have been reported. Although histological PPFE is predominantly located in the upper lobes, which are less often affected by fibrosis in patients with idiopathic pulmonary fibrosis (IPF), the clinical course of PPFE is seemingly similar to that of IPF. However, upper lobe fibroelastosis has various clinical and physiological characteristics that differ from those of IPF, including a flattened thoracic cage and a marked decrease in the forced vital capacity (FVC) but with a preserved residual volume. Compared with IPF, the decrease in the walking distance is mild despite the markedly decreased FVC in PPFE, and chest radiograph more frequently shows the elevation of bilateral hilar opacities with or without tracheal deviation. The prognosis may be related to the development of fibrosing interstitial pneumonia in the lower lobes with elevated levels of serum Krebs von den Lungen-6; however, there is marked variation in the pathogenesis and clinical features in PPFE. A proposal of the diagnostic criteria for idiopathic PPFE with and without surgical lung biopsy, which has recently been published, may be useful.

scholarly journals DSP rs2076295 variants influence nintedanib and pirfenidone outcomes in idiopathic pulmonary fibrosis: a pilot study

2021 ◽  
Vol 15 ◽  
pp. 175346662110425
Author(s):  
Martina Doubkova ◽  
Eva Kriegova ◽  
Simona Littnerova ◽  
Petra Schneiderova ◽  
Martina Sterclova ◽  
...  
Keyword(s):  
Pilot Study ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Cross Validation ◽  
Healthy Individuals ◽  
Lung Capacity ◽  
Diffuse Lung ◽  
Antifibrotic Drugs

Background: The antifibrotic drugs nintedanib and pirfenidone are used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the association of common profibrotic polymorphisms in MUC5B (mucin 5B, rs35705950) and DSP (desmoplakin, rs2076295) on antifibrotic treatment outcomes in IPF. Methods: MUC5B rs35705950 and DSP rs2076295 were assessed in IPF patients ( n = 210, 139 men/71 women) from the Czech EMPIRE registry and age- or sex-matched healthy individuals ( n = 205, 125 men/80 women). Genetic data were collated with overall survival (OS), acute exacerbation episodes, worsening lung function and antifibrotic treatment. Results: We confirmed overexpression of the MUC5B rs35705950*T allele (55.2% versus 20.9%, p < 0.001) and the DSP rs2076295*G allele (80.4% versus 68.3%, p < 0.001) in IPF compared with controls. On antifibrotic drugs, lower mortability was observed in IPF patients with DSP G* allele ( p = 0.016) and MUC5B T* allele ( p = 0.079). Carriers of the DSP rs2076295*G allele benefitted from nintedanib treatment compared with TT genotype by a longer OS [hazard ratio (HR) = 7.99; 95% confidence interval (CI) = 1.56–40.90; p = 0.013] and a slower decline in lung function (HR = 8.51; 95% CI = 1.68–43.14; p = 0.010). Patients with a TT genotype (rs2076295) benefitted from treatment with pirfenidone by prolonged OS ( p = 0.040; HR = 0.35; 95% CI = 0.13–0.95) compared with nintedanib treatment. Both associations were confirmed by cross-validation analysis. After stratifying by MUC5B rs35705950*T allele carriage, no difference in treatment outcome was observed for nintedanib or pirfenidone ( p = 0.784). In the multivariate model, smoking, age, forced vital capacity (FVC) and DLCO (diffuse lung capacity) at the IPF diagnosis were associated with survival. Conclusion: Our real-world study showed that IPF patients with MUC5B T* allele or DSP G* allele profit from antifibrotic treatment by lower mortability. Moreover, carriers of the DSP rs2076295*G allele benefit from treatment with nintedanib, and TT genotype from treatment with pirfenidone. MUC5B rs35705950 did not impact the outcome of treatment with either nintedanib or pirfenidone. Our single-registry pilot study should be confirmed with an independent patient cohort.

scholarly journals Does Technetium-99m Diethylenetriaminepentaacetate Clearance Predict the Clinical Course of Idiopathic Pulmonary Fibrosis?

2004 ◽  
Vol 11 (7) ◽  
pp. 477-479 ◽  
Author(s):  
Marco Mura ◽  
Gelorma Belmonte ◽  
Stefano Fanti ◽  
Angela Maria Pacilli ◽  
Luca Fasano ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Clinical Course ◽  
Pulmonary Function Tests ◽  
Functional Tests ◽  
High Resolution Computed Tomography ◽  
Technetium 99M ◽  
Potential Indicator

Clearance of inhaled technetium-99m diethylenetriaminepentaacetate (99mTc-DTPA) is a potential indicator of disease activity and progression in idiopathic pulmonary fibrosis (IPF). The objective of the present study was to evaluate the prognostic value of99mTc-DTPA scans in IPF. A total of 22 patients (18 males), aged 33 to 80 years with IPF were followed for six to 20 months (mean 13 months). At diagnosis, high resolution computed tomography (HRCT) scans showed a honeycomb pattern with bibasilar reticular opacities in all cases. At T0 (diagnosis) and T1 (follow-up), each patient had pulmonary function tests (forced vital capacity, diffusing capacity of the lung for carbon monoxide and partial arterial O2pressure), extension of fibrosis evaluated by HRCT visual score and99mTc-DTPA lung clearance. Results at T0 and T1 were compared, taking into account the whole population and patients with relatively fast and slow99mTc-DTPA wash-out.99mTc-DTPA clearance did not show any significant correlation with functional tests or HRCT score. These findings indicate that clearance of inhaled99mTc-DTPA is not of value in following the progress of IPF.

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

Pneumologie ◽  
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

scholarly journals Chemotherapy in idiopathic pulmonary fibrosis and small-cell lung cancer with poor lung function

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiyue Zhang ◽  
Wei Li ◽  
Chunyan Li ◽  
Jie Zhang ◽  
Zhenzhong Su
Keyword(s):  
Lung Cancer ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Small Cell Lung Cancer ◽  
Cell Lung Cancer ◽  
Small Cell ◽  
Small Cell Lung ◽  
High Resolution Computed Tomography ◽  
Chemotherapeutic Regimen

Abstract Background Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with unclear pathogenesis. IPF is considered as a risk factor for lung cancer. Compared to other lung cancers, small-cell lung cancer (SCLC) has a lower incidence, but has a more aggressive course. Patients with IPF and SCLC have a lower survival rate, more difficult treatment, and poorer prognosis. Case presentation Case 1 was of a 66-year-old man with IPF for 5 years, who was admitted to our hospital for dyspnea. Case 2 was of a 68-year-old woman, who presented with chest pains, cough, and dyspnea. Both patients had extremely poor lung function. High-resolution computed tomography and pathology revealed that both patients had IPF and SCLC. Chemotherapy comprising nedaplatin (80 mg/m2) and etoposide (100 mg for 5 days) was initiated for both patients. Antifibrotic agents were continued during the chemotherapeutic regimen. Both patients showed improvement in their condition after treatment. Conclusion The favorable outcomes in these 2 cases suggests that chemotherapy is worth considering in the management of patients having SCLC and IPF with poor lung function.

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