Deterioration In Forced Vital Capacity Research Articles

Disease progression in patients with usual interstitial pneumonia and probable UIP patterns on computed tomography with various underlying etiologies: a retrospective cohort study.

Usual interstitial pneumonia (UIP) is a pattern of interstitial pneumonia that is caused by different etiologies. This study aimed to investigate the transplant-free survival (TFS) and the decline in forced vital capacity (FVC) of the patients with UIP and probable UIP patterns on CT caused by various underlying conditions. A retrospective cohort study was conducted, enrolling patients with interstitial lung disease exhibiting a CT pattern consistent with UIP or probable UIP. Clinical and prognostic data of patients categorized by the etiology were compared. A total of 591 patients were included and classified into the following groups: idiopathic pulmonary fibrosis (IPF) (n = 320), connective tissue disease (CTD)-UIP (n = 229), asbestosis-UIP (n = 28), and hypersensitivity pneumonitis (HP)-UIP (n = 14). Advanced age, elevated levels of serum cytokeratin fraction 21-1 and percentage of neutrophils in bronchoalveolar lavage were observed in all groups. IPF patients showed a more rapid decline in FVC (133.9 mL/year) compared to CTD-UIP (24.5 mL/year, p = 0.001) and asbestosis-UIP (61.0 mL/year, p = 0.008) respectively. Sub-analysis of CTD-UIP revealed that patients with rheumatoid arthritis (RA)-UIP (88.1 mL/year) or antineutrophil cytoplasmic antibody-associated vasculitis (AAV)-UIP (72.9 mL/year) experienced a faster deterioration in FVC compared to those with primary Sjögren's syndrome (pSS)-UIP (25.9 mL/year, p < 0.05). Kaplan-Meier curves showed that IPF had the poorest TFS (median 55.9 months), followed by HP-UIP (57.5 months), CTD-UIP (66.7 months), and asbestosis-UIP (TFS not reached). RA-UIP or AAV-UIP did not exhibit any prognostic advantages compared to IPF, while asbestosis-UIP and pSS-UIP showed better survival rates. Patients with UIP caused by different underlying conditions share certain common features, but the trajectories of disease progression and survival outcomes differ.

Long-term follow-up of respiratory function in facioscapulohumeral muscular dystrophy

ObjectiveTo evaluate the 5-year change in respiratory function in patients with facioscapulohumeral muscular dystrophy (FSHD).MethodsGenetically confirmed patients with FSHD aged ≥ 18 years were examined twice over five years. Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) were measured using hand-held spirometry with a face mask. Several clinical outcome measures were correlated to respiratory function.ResultsNinety-two patients were included (57% male, age 18–75 years). At baseline, the spirometry outcomes of 41 patients showed a restrictive ventilatory pattern (FVC < 80% and FEV1/FVC ≥ 70% of predicted) and of 48 patients at follow-up. The mean FVC decreased from baseline to follow-up from 79.0 to 76.7% predicted (p = 0.021). This decrease was driven by a subgroup of 15 patients who had a deterioration of FVC of > 10% predicted. The subgroup of 15 patients was more severely affected at baseline (p = 0.002 for FSHD clinical score and 0.007 for Ricci score). They developed more frequently spinal and thorax deformities (p < 0.001 for kyphoscoliosis and 0.012 for pectus excavatum) and had a larger decline in axial muscle function (p = 0.020). Only weak correlations were found between the change in FVC% predicted and the change in clinical scores between baseline and follow-up.InterpretationRespiratory function remained stable in most patients with FSHD, but a subgroup of patients showed a pronounced deterioration. They showed more severe muscle weakness including the leg muscles at baseline (Ricci score ≥ 6), had spinal and thorax deformities and a relatively fast decline in axial muscle function at follow-up.

Unbiased, comprehensive analysis of Japanese health checkup data reveals a protective effect of light to moderate alcohol consumption on lung function

The overall effect of lifestyle habits, such as alcohol consumption, on general health remains controversial and it is important to clarify how such habits affect aging-related health impairments. To discover novel impacts of lifestyle on general health, we employed a mathematical approach to perform a comprehensive, unbiased, cross-sectional analysis of data from 6036 subjects who participated in a Japanese health checkup. Notably, we found that moderate alcohol consumption was positively correlated with lung function, muscle mass, and strength. Health checkup data were collected periodically from the same subjects. These people were light to moderate drinkers who had high health awareness and were basically free of major underlying diseases. We next analyzed 5 years of data from 1765 of these subjects. We found that higher baseline alcohol consumption, as well as increased alcohol intake over 5 years attenuated time-related deterioration of forced vital capacity without affecting total lung volume. This effect was independent of smoking. Our study suggests a possible protective effect of moderate amounts of alcohol on lung function, due to increased muscle mass/strength and forced vital capacity.

Clinical characteristics of non-idiopathic pulmonary fibrosis, progressive fibrosing interstitial lung diseases: A single-center retrospective study.

Progressive fibrosing interstitial lung disease (PF-ILD) is a progressive phenotype of fibrosing ILDs with varying definitions and elusive clinical characteristics. We aimed to clarify the clinical features and prognosis of PF-ILD cases based on the deterioration of pulmonary function.Altogether, 91 consecutive ILD patients who underwent at least 2 pulmonary function tests (PFTs) with an interval of at least 24 months, as the screening period, between January 2009 and December 2015 were retrospectively reviewed. The deterioration of forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLco) was calculated based on PFT data and screening period. The definition of PF-ILD was1.relative decline of 10% or more in FVC per 24 months or2.relative decline in FVC of 5% or more with decline in DLco of 15% or more per 24 months.Medical records of 34 patients with idiopathic pulmonary fibrosis (IPF), 11 patients with non-IPF, PF-ILD, and 46 patients with non-IPF, non-PF-ILD were retrospectively analyzed. Patient characteristics, pharmacologic or non-pharmacologic treatment status, and prognosis were compared between the IPF and non-IPF groups and between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups.Eleven patients (19.3%) showed a progressive phenotype in the non-IPF group. The pulmonary function data at the first PFT were worse in non-IPF, PF-ILD patients than in non-IPF, non-PF-ILD patients. There were no differences in the proportion of patients who were observed without pharmacologic treatment or of those receiving pharmacologic treatment between the non-IPF, PF-ILD and non-IPF, non-PF-ILD groups. Low %FVC at the first PFT and the usual interstitial pneumonia-like fibrotic pattern on high-resolution computed tomography were risk factors for PF-ILD in the non-IPF group. The mortality in the non-IPF, PF-ILD group was significantly worse than that of the non-IPF, non-PF-ILD group and was as poor as that of the IPF group. Multivariate logistic analysis showed that aging and low %DLco at the first PFT were risk factors for mortality within the non-IPF group.The prognosis of non-IPF, PF-ILD patients was as poor as that of IPF patients. Non-IPF, PF-ILD patients require more intensive treatment before disease progression.

Forced vital capacity less than 1: A mark for high-risk patients.

Rib fractures (RFx) continue to be a source of morbidity and mortality. A RFx care pathway has been used based on forced vital capacity (FVC). The objective of this study was to test the hypothesis that deterioration of FVC to less than 1 after admission is a marker for high-risk patients and affects outcomes. A retrospective study of patients enrolled in an RFx care pathway at a Level 1 trauma center from 2009 to 2014. All patients had an admission FVC greater than 1. 2 groups were analyzed: patients with a lowest inpatient FVC less than 1 (Group A) compared to patients with lowest inpatient FVC of 1 or greater (Group B). Complications [pneumonia, upgrade to the intensive care unit, readmission, and intubation] and demographics were examined. Patients without documented admission FVCs were excluded. p < 0.05 was considered significant. A total of 1,106 patients were analyzed (Group A, 187; Group B, 919). Patients whose FVC dropped less than 1 (Group A) had a higher complication rate [15% (Group A) vs 3.2% (Group B); p < 0.001]. Rates of pneumonia, readmission, unplanned upgrade, and intubation were all significantly higher in Group A [pneumonia: 9% (Group A) vs 1.4% (Group B), p < 0.001; readmission: 4% (Group A) vs 1.7% (Group B), p = 0.04; upgrade; 3.7% (Group A) vs 0.2% (Group B), p < 0.001; intubation: 1.6% (Group A) vs 0.1% (Group B), p = 0.02]. Hospital length of stay was longer in Group A [10 days (Group A) vs 4 days (Group B), p < 0.001]. Forced vital capacity predicts complications in patients with RFx. Patients whose FVC falls less than 1 during admission are at high risk for pulmonary complications. Daily FVC testing for patients admitted with RFx can predict outcomes. Forced vital capacity less than 1 should be considered as a marker for complications. Once FVC drops less than 1, patients should be considered for increased interventions. Even if the patient has not yet clinically deteriorated, consideration for higher level of care is warranted. Prognostic study, level III.

EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis.

Prognostic factors of idiopathic pulmonary fibrosis (IPF) currently recognized include changes in vital capacity and radiologic findings. However, most of the prognostic studies in IPF are based on clinical studies with preselected IPF populations. Therefore, we decided to analyze the factors influencing IPF prognosis based on the real-practice data from our IPF registry. Data of 514 subjects consecutively entered since 2012 into Czech EMPIRE IPF registry were analyzed. Median age of our patient cohort was 67 years (50-82). Median overall survival (OS) of the cohort was 63.1 months. The clinical course of IPF according to FVC (forced vital capacity) changes was stabilized in 32.8% of patients (29.7% according to DLCO [diffuse lung capacity] changes), slowly progressive in 39.5% (45%), rapidly progressive in 23.5% (20.7%); and 1.7% patients had at least one acute exacerbation during follow-up. Deterioration in FVC of ≥10% at month 12 and in DLCO of ≥15% at months 12, 18, and 24 influenced the OS significantly. The fast progressors defined by the DLCO decline rate had higher risk of death compared to those defined by the FVC change over time. In multivariate analysis, age ≥70 years, interstitial HRCT scores ≥3, and change in DLCO of ≥15% at month 12 were confirmed as factors negatively influencing OS. DLCO changes over time were shown as a better predictor of mortality compared with FVC changes in our study. In our opinion it is necessary to implement the DLCO analysis into clinical trials and routine practice.

Isolated decrease of the lung diffusing capacity in patients with systemic sclerosis without pulmonary arterial hypertension: a long-term prospective study

The objective of this 5year prospective study was to investigate a clinical role of isolated decrease of DLCO in patients with systemic sclerosis (SS) without pulmonary arterial hypertension (PAH).Methods. We selected 48 out of 142 patients with SS: 35 (73%) with limited SS and 13 (27%) with diffuse SS. The average length of the disease was 12.9 ± 7.9 years. Inclusion criteria were DLCO &lt; 80% pred., forced vital capacity (FVC) ≥ 80% pred. and systolic pulmonary artery pressure (PAP) ≤ 35 mm Hg according to echocardiographic examination (echoCG). High resolution computed tomography (HRCT), spirometry, DLCO measurement, and echoCG were obtained at baseline and after 4.7 ± 1 year of follow up. All patients were treated with standard therapy.Results. CT signs of interstitial lung disease (ILD) were found in 43 (89.6%) patients at baseline and newly developed in 3 other patients during the followup. During the followup, lung CT improved in 5 (10.4%) patients and progressed in 15 (31.3%) patients. Over 5 years, FVC did not change significantly (97.6 ± 10.7% and 100.8 ± 18.9%; р = 0.15), while DLCO significantly decreased both in limited and diffuse SS groups (59.8 ± 13.5% and 56.3 ± 12%; р = 0.006). Mean PAP values remained within normal range in majority of patients. Clinically significant FVC reduction (≥ 10%) was found in 5 patients; of them, CT signs of ILD at baseline were seen in 3 patients and newly developed during the followup in 2 others. Clinically significant DLCO reduction (≥ 10%) was documented in 11 (23%) patients, all had CT signs of ILD at baseline, although CT progression during the followup was noted only in six of them. Contemporary deterioration in FVC, DLCO and CT was found in 3 patients.Conclusion. Clinical course of ILD in SS patients with isolated DLCO reduction and without PAH was relatively benign, with respiratory volumes being preserved within normal range for long time. Comparison of radiological and functional changes in a prospective study has suggested that DLCO is a more sensitive tool to determine ILD progression compared to HRCT. Regular DLCO measurements could be used as a reliable tool for monitoring of ILD associated with SS.

Role of the cardio-pulmonary exercise test and six-minute walking test in the evaluation of exercise performance in patients with late-onset Pompe disease

In patients with late-onset Pompe disease, we explored the role of the Cardiopulmonary Exercise Test (CPET) and the Six-Minute Walking Test (6MWT) in the assessment of exercise capacity and in the evaluation of the effects of enzyme replacement therapy (ERT). Eight patients affected by late-onset Pompe disease, followed up at the Centre for Neuromuscular Diseases and treated with ERT, underwent a baseline evaluation with a spirometry, a CPET and a 6MWT. Four of them were restudied after 36 months of treatment. Three patients showed a reduction in exercise capacity as evaluated by peak oxygen uptake (VO2) measured at the CPET and Distance Walked (DW) measured at the 6MWT (median % predicted: 67.1 [range 54.3–99.6] and 67.3 [56.6–82.6], respectively). Cardiac and respiratory limitations revealed by the CPET were correlated to peak VO2, but not to the DW. Nevertheless, percent of predicted values of peak VO2 and DW were strongly correlated (rho = 0.85, p = 0.006), and close to identity. In the longitudinal evaluation forced vital capacity decreased, while peak VO2 and DW showed a trend to a parallel improvement. We concluded that although only the CPET revealed causes of exercise limitation, which partially differed among patients, CPET and 6MWT showed a similar overall degree of exercise impairment. That held true in the longitudinal assessment during ERT, where both tests demonstrated similar small improvements, occurring despite deterioration in forced vital capacity.

English

BACKGROUND: Occupation exposes an individual to certain hazards which are known as occupational diseases or pneumoconiosis. Carpet weavers are constantly exposed to dust in their workplace environment, posing a threat to their health. Spirometry is a readily available tool to measure pulmonary functions in the high risk group at an early stage which helps to take necessary measures to prevent further damage. AIMS: To study and compare the effects of long term dust exposure on pulmonary functions of carpet weavers with those of healthy subjects unexposed to such dust. MATERIALS AND METHODS: 50 adult female workers from carpet making industry were chosen for our study. 50 age and sex matched healthy subjects who were not exposed to excessive dust, enrolled as the controls. Forced expiratory spirograms were recorded by RMS Medspiror. Parameters such as forced vital capacity (FVC), forced expiratory volume in 1st second (FEV1), the ratio of FEV1/FVC, forced expiratory flow in the middle half of FVC (FEF25-75%), peak expiratory flow rate (PEFR) were assessed in both cases and controls. STATISTICAL ANALYSIS: The results were analyzed by using the student's unpaired t-test. RESULTS: Carpet weavers showed deterioration in FVC, FEV1, FEF25-75%, PEFR and FEV1/FVC ratio which was statistically highly significant (P< 0.001), suggestive of obstructive respiratory disorder. CONCLUSION: Weavers are at risk of developing occupational lung disease which can be prevented by taking meticulous measures and

AB0647 Effects of Cyclophosphamide Pulse Therapy on Parameters of Lung Function in Patients with Systemic Sclerosis

ObjectivesTo analyze the effects of cyclophosphamide (CYC) pulse therapy on parameters of lung function in patients (pts) with systemic sclerosis (SSc).MethodsThirty pts with SSc (25 women and 5 men, aged...

Inhaled Budesonide and Oral Dexamethasone Prevent Acute Mountain Sickness

BackgroundThis double-blind, randomized controlled trial aimed to investigate inhaled budesonide and oral dexamethasone compared with placebo for their prophylactic efficacy against acute mountain sickness after acute high-altitude exposure. MethodsThere were 138 healthy young male lowland residents recruited and randomly assigned to receive inhaled budesonide (200 μg, twice a day [bid]), oral dexamethasone (4 mg, bid), or placebo (46 in each group). They traveled to 3900 m altitude from 400 m by car. Medication started 1 day before high-altitude exposure and continued until the third day of exposure. Primary outcome measure was the incidence of acute mountain sickness after exposure. ResultsOne hundred twenty-four subjects completed the study (42, 39, and 43 in the budesonide, dexamethasone, and placebo groups, respectively). Demographic characteristics were comparable among the 3 groups. After high-altitude exposure, significantly fewer participants in the budesonide (23.81%) and dexamethasone (30.77%) groups developed acute mountain sickness compared with participants receiving placebo (60.46%) (P = .0006 and P = .0071, respectively). Both the budesonide and dexamethasone groups had lower heart rate and higher pulse oxygen saturation (SpO2) than the placebo group at altitude. Only the budesonide group demonstrated less deterioration in forced vital capacity and sleep quality than the placebo group. Four subjects in the dexamethasone group reported adverse reactions. ConclusionsBoth inhaled budesonide (200 μg, bid) and oral dexamethasone (4 mg, bid) were effective for the prevention of acute mountain sickness, especially its severe form, compared with placebo. Budesonide caused fewer adverse reactions than dexamethasone.

Functional Outcomes in Duchenne Muscular Dystrophy Scoliosis

While most studies of Duchenne muscular dystrophy scoliosis focus on technical and radiographic indices, functional status is a more important factor to consider in the management of Duchenne muscular dystrophy. The objectives of the current study were to compare the pulmonary function, radiographic outcome, and functional recovery, with use of validated questionnaires, in surgically and nonsurgically treated patients with Duchenne muscular dystrophy who have scoliosis.Sixty-six patients (forty treated surgically and twenty-six treated nonsurgically) with a minimum follow-up of two years were included in this study. Forced vital capacity, radiographic parameters (the Cobb angle, lordosis, and pelvic obliquity), and functional status, according to the modified Rancho scale and manual muscle test, were measured preoperatively and at the time of the final follow-up. The Muscular Dystrophy Spine Questionnaire (MDSQ) was completed at the final follow-up evaluation.Pulmonary function, functional scores (manual muscle test and modified Rancho scale), and radiographic measurements, except for lordosis, were similar for both groups at the time of the initial consultation (p > 0.05). At the time of the final follow-up, all radiographic parameters were significantly improved in the surgical group compared with the nonsurgical group. The mean score (and standard deviation) on the manual muscle test was not significantly different between the surgical and nonsurgical groups (23.2 ± 8.3 versus 22.8 ± 6.3; p = 0.828). The mean score on the modified Rancho scale also showed similar results in the groups (3.9 ± 0.3 and 4.04 ± 0.3, respectively; p = 0.088). The surgical group had higher mean MDSQ scores than the nonsurgical group (35.1 ± 14.7 and 26.9 ± 9.9, respectively; p = 0.008). Both groups showed a decrease in forced vital capacity at the time of the final follow-up, but the deterioration of forced vital capacity was significantly slower (p = 0.035) in the surgical group (268 ± 361 mL) than in the nonsurgical group (536 ± 323 mL).Surgery in patients who had Duchenne muscular dystrophy with scoliosis improved function and decreased the rate of deterioration of forced vital capacity compared with patients treated conservatively. However, the muscle power and forced vital capacity decreased in both groups.

Cyclophosphamide for scleroderma lung disease: a systematic review and meta-analysis

To assess the effectiveness of cyclophosphamide in the management of scleroderma-related interstitial lung disease (ILD). In this systematic review study, the primary outcome measures were change in forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (D(L)CO) of the patients after 6 and 12 months. To assess the effect of cyclophosphamide on early stage of ILD, alveolitis, in SSc patients, we selected the studies that used the BAL findings or HRCT or recent deterioration of PFT with minimal chest X-ray finding in early stage of disease as diagnosis of alveolitis. A sensitive systematic search strategy was used to find all relevant studies. Finally, 17 trials were included in the analysis that was performed using STATA. (Version 8) and Review Manager (version 4.1; MetaView version 4.1) softwares. Results from 10 studies were pooled for the outcome variable of FVC after 12 months. The summary WMD (random effects) was 2.45 (95% CI, 0.760-4.149 P = 0.005), which means that cyclophosphamide was able to prevent deterioration of FVC after 12 months. In pooled data of 13 studies, about DLCO after 12 months WMD (random effects) was 2.003 2.96 (95% CI, -0.228 to 6.159 P = 0.069), which means that cyclophosphamide was not able to prevent deterioration of D(L)CO after 12 months. If we considered clinically sensible improvement as absolute value ≥10% in DLCO and VC, then result of treatment with cyclophosphamide treatment in scleroderma patients with ILD was not significant.

Respiratory function deterioration is not time-linked with upper-limb onset in amyotrophic lateral sclerosis.

In amyotrophic lateral sclerosis (ALS), symptoms apparently spread following regional rules, and depending on the site of onset. We examined if respiratory function deterioration appears earlier or is more severe in patients with upper-limb onset. We compared the results of various pulmonary function tests (PFT) obtained at diagnosis depending on the site of onset in 49 ALS patients. In a longitudinal study, we compared the deterioration of forced vital capacity (FVC) in relation to the site of onset, and analyzed the time elapsed to reach values below 80% of predicted according to site of onset, and we compared the survival depending on the site of onset. No significant differences in PFT were found in the upper-limb onset group in any of the analysis performed. No differences in survival were detected in any disease onset group. Pulmonary function deterioration does not appear to be time-linked to arm onset in ALS.