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American Journal of Respiratory and... Jun 2024
PubMed: 38941619
DOI: 10.1164/rccm.202406-1123LE -
American Journal of Respiratory and... Jun 2024
PubMed: 38941617
DOI: 10.1164/rccm.202405-1008LE -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Any test that provides sufficient prognostic information to guide treatment decisions in idiopathic pulmonary fibrosis (IPF) is not available. The aim of our study was...
BACKGROUND AND AIM
Any test that provides sufficient prognostic information to guide treatment decisions in idiopathic pulmonary fibrosis (IPF) is not available. The aim of our study was to determine the predictive factors of mortality in patients with IPF treated with antifibrotics.
METHODS
Patients with diagnosis of IPF who were treated with antifibrotics between 2016 - 2021 were included in the study. Demographic, clinical and laboratory characteristics of the patients was derived from hospital records retrospectively. Kaplan Meier and multivariate cox regression analysis were achieved for detection of mortality predictors.
RESULTS
Study population was composed of 119 IPF patients with a male predominance of 80.7% (n=96). Mean age of the patients was 67.9 ± 7.07 years. On univariate analysis, sex was not a significant predictor of mortality (HR 1.79; 95% CI: 0.87 - 3.69, p =0.11). BMI ≤ 26,6 m2/kg, DLCO ≤ 3.11 ml/mmHg/min, age over 62 years, 6DWT ≤ 382 meters, NLR ≤ 2.67 and PDW ≤ 16.7% were found to be significant for predicting mortality. On multivariate cox regression analysis four parameters remained significant for prediction of mortality: RDW > 14%, NLR ≤ 2.67, BMI ≤ 26,6 m2/kg and DLCO ≤ 3.11 ml/mmHg/min (respectively, HR: 2.0. 95% CI: 1.02 - 3.91, p=0.44; HR: 2.68. 95% CI: 1.48 - 4.85, p=0.001, HR: 2.07. 95% CI: 1.14 - 3.76, p=0.02, HR: 3.46. 95% CI: 1.85 - 6.47, p<0.001). A scoring system with these parameters discriminated patients with worse prognosis with a sensitivity of 89.1 % and a specificity of 65.8 % when total point was over 2 (AUC0.83, p<0.001). Conclusions In this study, DLCO, BMI, RDW and NLR levels significantly predicted mortality in IPF patients. Along with GAP index, scoring system with these simple parameters may give information about the prognosis of an IPF patient treated with antifibrotics.
PubMed: 38940720
DOI: 10.36141/svdld.v41i2.13779 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial lung disease (ILD) characterized by subpleural parenchymal fibrosis and elastosis mainly in the...
BACKGROUND
Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial lung disease (ILD) characterized by subpleural parenchymal fibrosis and elastosis mainly in the upper lobes. PPFE occurs in a secondary form that overlaps with underlying medical conditions or complications. This study evaluated the clinical impact of coexisting factors on the survival of patients with PPFE.
METHODS
Fifty-five PPFE patients were retrospectively evaluated. The patients' diagnoses were categorized as "idiopathic PPFE" with no known cause or "secondary PPFE" with underlying medical conditions or complications. The clinical characteristics and survival rates of these groups were compared.
RESULTS
Twenty-eight patients (50.9%) were diagnosed with idiopathic PPFE and 27 (49.1%) with secondary PPFE, including cases of occupational dust exposure, connective tissue disease (CTD), post-hematopoietic stem cell transplantation (HSCT), and a family history of ILD. The idiopathic and secondary PPFE groups had similar clinical features, laboratory tests, and pulmonary function profiles, including a low body mass index, normal Krebs von den Lungen-6, high surfactant protein-D, and high residual volume/total lung capacity. In the secondary PPFE group, post-HSCT was associated with a worse prognosis, and CTD was associated with better prognosis. A multivariate analysis demonstrated that post-HSCT and a reduced forced vital capacity were significantly associated with a worsened survival in patients with PPFE.
CONCLUSIONS
The prognosis of PPFE is highly influenced by underlying medical conditions or complications. Patients with post-HSCT PPFE should be monitored particularly closely, as they are at higher risk of a poor prognosis than others.
PubMed: 38940719
DOI: 10.36141/svdld.v41i2.13845 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Adequate respiratory muscle strength is required to meet the increased ventilatory demand during physical activities. However, it is not well known whether respiratory...
BACKGROUND
Adequate respiratory muscle strength is required to meet the increased ventilatory demand during physical activities. However, it is not well known whether respiratory muscle strength is impaired in patients with idiopathic pulmonary fibrosis (IPF).
OBJECTIVES
This study aimed to investigate the relationship between respiratory muscle strength and exercise capacity, quality of life, physical activity level, and fatigue in IPF patients.
METHODS
The study comprised 30 individuals with idiopathic pulmonary fibrosis (IPF) and 30 healthy controls. Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) were measured to assess respiratory muscle strength. The International Physical Activity Questionnaire-Short Form, 6-minute walk test distance (6MWD), St George Respiratory Questionnaire (SGRQ), and Fatigue Severity Scale (FSS) were employed to evaluate physical activity level, exercise capacity, quality of life, and fatigue severity, respectively.
RESULTS
MIP (81±29 vs.73±20 cmH2O) and MEP (93±31 vs. 93±34 cmH2O) did not differ significantly between IPF patients and controls (p>0.05). In patients with IPF, MIP was significantly correlated with 6MWD (r=0.533), SGRQ (r=-0.428), and FSS (r=-0.433). Multivariate models including MIP, MEP, FEV1, FVC, and PA level explained 74% of the variance in the 6MWD (p<0.001), and MIP, FEV1, and PA level were independent predictors of the 6MWD, with FEV1 being the strongest predictor (β=0.659). Multivariate models predicting SGRQ revealed none of MIP, FEV1 or PA level was directly influencing the SGRQ score.
CONCLUSIONS
This study suggests that patients with IPF do not have respiratory muscle weakness. Inspiratory muscle strength has a direct influence on exercise capacity but an indirect effect on quality of life, probably by influencing exercise capacity.
PubMed: 38940715
DOI: 10.36141/svdld.v41i2.14884 -
Sarcoidosis, Vasculitis, and Diffuse... Jun 2024Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease of unknown cause with a poor prognosis. The aim of our study is to determine the role of...
BACKGROUND AND AIM
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease of unknown cause with a poor prognosis. The aim of our study is to determine the role of Krebs von den Lungen-6(KL-6),Matrix metalloproteinase (MMP)-7, Surfactant protein A (SP-A), Surfactant protein D(SP-D), vascular endothelial growth factor (VEGF) and periostin in the diagnosis of IPF and in the response monitoring of patients treated.
METHOD
47 IPF patients, 27 non-IPF interstitial lung disease (ILD) patients and 21 healthy individuals were included in the study. Demographic data, pulmonary function test- Diffusing capacity of the lung for carbon monoxide (PFT-DLCO) measurements, High-resolution computed tomography (HRCT) findings of the patients were recorded, and serum samples were taken.
RESULTS
While periostin and SP-A levels were not significantly different between IPF and non-IPF ILD, they were significantly higher in both IPF and non-IPF ILD compared to healthy control group (p=0.002,p=0.006 for periostin and p=0.002,p<0.001 for SP-A, respectively).By receiver operating characteristic (ROC) analysis, the cut-off point for periostin to distinguish IPF is >594.5 pg/ml (sensitivity 72%, specificity 76%), while the cut-off point for SP-A is found >6.62 ng/ml (sensitivity 87.2%,specificity 57.1%). In the combined ROC analysis based on SP-A=6.62 ng/ml and periostin >634.6 pg/ml values, sensitivity was found to be 85% and specificity was 57%.Considering the correlation of forced expiratory volume in the first second (FEV1)(%), forced vital capacity (FVC)(%), restriction and diffusion severities with biomarker levels in the 6th month of IPF patients treated, a correlation was detected between MMP-7 levels and restriction severities (p=0.020), between KL-6 levels and restriction and diffusion severities (p=0.002), and between SP-A levels and FVC(%)(p=0.006).
CONCLUSION
It is thought that biomarkers SP-A and periostin may contribute significantly to the diagnosis of patients with IPF, and SP-A, MMP-7 and KL-6 levels may contribute significantly to treatment follow-up.
PubMed: 38940711
DOI: 10.36141/svdld.v41i2.15454 -
Pediatric Pulmonology Jun 2024Newborn screening (NBS) for cystic fibrosis (CF) is universal in the United States. Protocols vary but include an immunoreactive trypsinogen (IRT) level and CFTR variant...
BACKGROUND
Newborn screening (NBS) for cystic fibrosis (CF) is universal in the United States. Protocols vary but include an immunoreactive trypsinogen (IRT) level and CFTR variant panel. California CF NBS has a 3-step screening: IRT level, variant panel, and CFTR sequencing if only one variant identified on panel.
METHODS
This was a cohort study of infants with CF born in California (2007-2021) to examine racial and ethnic differences in having a false-negative NBS result for CF and at which step the false-negative occurred. We examined how different CFTR variant panels would improve detection of variants by race and ethnicity: original 39-variant panel, current 75-variant panel, and all 402 disease-causing CFTR variants in the CFTR2 database.
RESULTS
Of the 912 infants born in California with CF, 84 had a false-negative result: 38 due to low IRT level and 46 with a high IRT value (but incomplete variant detection). Asian (OR 6.3) and Black infants (OR 2.5) were more likely to have a false-negative screening result than non-Hispanic white infants. The majority of false-negative screening (but CF diagnosis) cases among American Indian/Native Alaskan and non-Hispanic White infants were due to low IRT levels. The majority of Asian and Hispanic infants with false-negative screening had no variants detected. Detection of two CFTR variants was improved with the 75-variant panel in Black, Hispanic, and non-Hispanic White infants and with the 402-variant panel in Black, Hispanic, non-Hispanic White, and other race infants.
CONCLUSIONS
Larger CFTR panels in NBS improved the detection of CF in all races and ethnicities.
PubMed: 38940324
DOI: 10.1002/ppul.27155 -
Journal of Medical Economics Jun 2024AimsWe aimed to describe the clinical, economic, and societal burdens of cystic fibrosis (CF) and impact of CF transmembrane conductance regulator modulator (CFTRm)...
AimsWe aimed to describe the clinical, economic, and societal burdens of cystic fibrosis (CF) and impact of CF transmembrane conductance regulator modulator (CFTRm) treatment on people with CF, caregivers, and healthcare systems.Material and MethodsThis retrospective study used linked real-world data from Swedish national population-based registries and the Swedish CF Quality Registry to assess clinical, economic, and societal burden and CFTR impact in CF. Records from people with CF and a ten-fold control population without CF matched by sex, birth year, and location were compared during 2019. Outcomes for a subset aged >6 years initiating lumacaftor/ivacaftor (LUM/IVA) in 2018 were compared 12 months pre- and post-treatment initiation.ResultsPeople with CF (n = 743) had >10 times more inpatient and outpatient specialist visits annually vs controls (n = 7406). Those aged >18 had an additional 77·7 (95% CI: 70·3, 85·1) days of work absence, at a societal cost of €11,563 (95% CI: 10,463, 12,662), while caregivers of those aged <18 missed an additional 6.1 (5.0, 7.2) workdays. With LUM/IVA treatment, people with CF (n = 100) had significantly increased lung function (mean change in ppFEV [3·8 points; 95% CI: 1·1, 6·6]), on average 0·5 (95% CI: -0·8, -0·2) fewer pulmonary exacerbations and 45·2 (95% CI: 13·3, 77·2) fewer days of antibiotics. Days of work lost by caregivers of people with CF aged <18 decreased by 5·4 days (95% CI: 2·9, 7·9).ConclusionCF is associated with a high clinical economic and societal burden in Sweden. Improvements in clinical status observed in people with CF treated with LUM/IVA were reflected in reduced caregiver and societal burden.
PubMed: 38939921
DOI: 10.1080/13696998.2024.2373000 -
Cureus May 2024Introduction The coronavirus disease 2019 (COVID-19) pandemic has impacted the lives of thousands of patients worldwide with many patients having residual symptoms...
Introduction The coronavirus disease 2019 (COVID-19) pandemic has impacted the lives of thousands of patients worldwide with many patients having residual symptoms months after the acute infection. The severity of lung involvement ranges from mild asymptomatic to severe acute respiratory distress syndrome (ARDS), which may lead to pulmonary fibrosis. Pulmonary fibrosis increases the long-term morbidity of post-COVID-19 patients in the form of restrictive lung disease. The six-minute walk test (6MWT), Borg scale, and spirometry are simple and low-cost tests used to evaluate a patient's exercise capacity and functional status. This study was conducted to assess the residual symptoms and functional status using spirometry and 6MWT in COVID-19 patients of moderate to severe category after three months of discharge. Methods This was an observational, prospective, and cross-sectional study conducted at a tertiary care center in North India, aiming to enroll a minimum of 50 patients who recovered from COVID-19 pneumonia. These patients were previously hospitalized with moderate to severe disease severity as defined by the Indian Council of Medical Research (ICMR) criteria, and the assessment occurred at least three months after their discharge. Individuals who were under 18 years of age or pregnant or had any respiratory or cardiac illness in the past were excluded from the study. Results A total of 50 patients were included in the study for final analysis. After a three-month follow-up, 40 (80%) patients were still symptomatic. The most commonly reported symptom was exertional dyspnea in 21 (42%), dyspnea at rest in 16 (32%), and fatigue in three (6%) patients. Of the total patients, 37 (74%) covered a distance less than expected in the six-minute walk test. The mean distance covered by patients was 426.1 ± 115.01 m, in contrast to the expected mean distance of 537.22 ± 37.61 m according to standard equations for Indian males and females. A fall in oxygen saturation by more than or equal to 3% was observed in approximately 24 (48%) patients after the six-minute walk test. The mean value of fatigue and dyspnea score was 3.2 ± 1.7 (moderate score). Among patients with moderate disease during their hospital stay, a higher proportion exhibited a normal pattern on pulmonary function tests (PFT) compared to those severely affected, 23 (69.70%) versus two (11.76%), respectively. Conclusion The persistence of symptoms and functional limitation of activities should be anticipated in patients with COVID-19. Spirometry and 6MWT can be a valuable tool in determining the prevalence of functional limitation in recovered patients of COVID-19. It can potentially help in determining and further planning the rehabilitative measures in the management of COVID-19 survivors. It can also be concluded that it is important to have a long-term follow-up in patients with moderate to severe COVID-19.
PubMed: 38939290
DOI: 10.7759/cureus.61221 -
Journal of Extracellular Biology Jul 2023High-resolution computed tomography (HRCT) imaging is critical for diagnostic evaluation of Idiopathic Pulmonary Fibrosis (IPF). However, several other interstitial lung...
High-resolution computed tomography (HRCT) imaging is critical for diagnostic evaluation of Idiopathic Pulmonary Fibrosis (IPF). However, several other interstitial lung diseases (ILDs) often exhibit radiologic pattern similar to IPF on HRCT making the diagnosis of the disease difficult. Therefore, biomarkers that distinguish IPF from other ILDs can be a valuable aid in diagnosis. Using mass spectrometry, we performed proteomic analysis of plasma extracellular vesicles (EVs) in patients diagnosed with IPF, chronic hypersensitivity pneumonitis, nonspecific interstitial pneumonitis, and healthy subjects. A five-protein signature was identified by lasso regression and was validated in an independent cohort using ELISA. The five-protein signature derived from mass spectrometry data showed an area under the receiver operating characteristic curve of 0.915 (95%CI: 0.819-1.011) and 0.958 (95%CI: 0.882-1.034) for differentiating IPF from other ILDs and from healthy subjects, respectively. Stepwise backwards elimination yielded a model with 3 and 2 proteins for discriminating IPF from other ILDs and healthy subjects, respectively, without compromising diagnostic accuracy. In summary, we discovered and validated EV protein biomarkers for differential diagnosis of IPF in independent cohorts. Interestingly, the biomarker panel could also distinguish IPF and healthy subjects with high accuracy. The biomarkers need to be evaluated in large prospective cohorts to establish their clinical utility.
PubMed: 38939072
DOI: 10.1002/jex2.98