CD8+ T lymphocytes in bronchoalveolar lavage in idiopathic pulmonary fibrosis

<p>Abstract</p> <p>Background</p> <p>Recently it was shown that in Idiopathic Pulmonary Fibrosis (IPF) tissue infiltrating CD₈₊T lymphocytes (TLs) are associated with breathlessness and physiological indices of disease severity, as well as that CD₈₊TLs recovered by bronchoalveolar lavage (BAL) relate to those infiltrating lung tissue. Since BAL is a far less invasive technique than tissue biopsy to study mechanisms in IPF we further investigated the usefulness offered by this means by studying the relationship between BAL macrophages, neutrophils, eosinophils, CD₃₊, CD₄₊, CD₈₊, CD_8+/38+TLs and CD₄₊/CD₈₊ratio with breathlessness and physiological indices.</p> <p>Patients and methods</p> <p>27 IPF patients, 63 ± 9 years of age were examined. Cell counts were expressed as percentages of total cells and TLs were evaluated by flow cytometry. FEV₁, FVC, TLC, RV, <it>D</it>LCO, PaO₂, and PaCO₂were measured in all. Breathlessness was assessed by the Medical Research Council (MRC) chronic dyspnoea scale.</p> <p>Results</p> <p>CD₈₊TLs correlated positively (r_s= 0.46, p = 0.02), while CD₄₊/CD₈₊ratio negatively (r_s= -0.54, p = 0.006) with the MRC grade. CD₈₊TLs correlated negatively with RV (r_s= -0.50, p = 0.017). CD_8+/38+TLs were negatively related to the FEV₁and FVC (r_s= -0.53, p = 0.03 and r_s= -0.59, p = 0.02, respectively). Neutrophils correlated positively with the MRC grade (r_s= 0.42, p = 0.03), and negatively with the <it>D</it>LCO (r_s= -0.54, p = 0.005), PaO₂(r_s= -0.44, p = 0.03), and PaCO₂(r_s= -0.52, p = 0.01).</p> <p>Conclusion</p> <p>BAL CD₈₊TLs associations with physiological and clinical indices seem to indicate their implication in IPF pathogenesis, confirming our previous tissue study.</p

Heterozygous Alterations of TNFRSF13B/TACI in Tonsillar Hypertrophy and Sarcoidosis

TNFRSF13B/TACI defects have been associated with CVID pathogenesis and/or phenotype, especially the development of benign lymphoproliferation and autoimmunity. Our purpose was to investigate the role of TNFRSF13B/TACI defects in the pathogenesis of two common lymphoproliferative disorders, namely, sarcoidosis and tonsillar hypertrophy (TH). 105 patients (71 with sarcoidosis and 34 with TH, including 19 without infectious causative and 15 due to Haemophilus influenzae) were analyzed for TNFRSF13B/TACI defects. Two out of 19 TH patients without infectious cause (10.5%) and 2 patients with sarcoidosis (2.8%) displayed rare TNFRSF13B/TACI defects (I87N, L69TfsX12, E36L, and R202H, resp.). Both mutations identified in TH patients have been assessed as deleterious for protein function, while the patient with the R202H mutation and sarcoidosis exhibited also sIgG4D. Our study further supports the notion that TNFRSF13B/TACI defects alone do not result in CVID but may be also found frequently in distinct clinical phenotypes, including benign lymphoproliferation and IgG subclass deficiencies

Vibration Response Imaging: evaluation of rater agreement in healthy subjects and subjects with pneumonia

<p>Abstract</p> <p>Background</p> <p>We evaluated pulmonologists variability in the interpretation of Vibration response imaging (VRI) obtained from healthy subjects and patients hospitalized for community acquired pneumonia.</p> <p>Methods</p> <p>The present is a prospective study conducted in a tertiary university hospital. Twenty healthy subjects and twenty three pneumonia cases were included in this study. Six pulmonologists blindly analyzed images of normal subjects and pneumonia cases and evaluated different aspects of VRI images related to the quality of data aquisition, synchronization of the progression of breath sound distribution and agreement between the maximal energy frame (MEF) of VRI (which is the maximal geographical area of lung vibrations produced at maximal inspiration) and chest radiography. For qualitative assessment of VRI images, the raters' evaluations were analyzed by degree of consistency and agreement.</p> <p>Results</p> <p>The average value for overall identical evaluations of twelve features of the VRI image evaluation, ranged from 87% to 95% per rater (94% to 97% in control cases and from 79% to 93% per rater in pneumonia cases). Inter-rater median (IQR) agreement was 91% (82-96). The level of agreement according to VRI feature evaluated was in most cases over 80%; intra-class correlation (ICC) obtained by using a model of subject/rater for the averaged features was overall 0.86 (0.92 in normal and 0.73 in pneumonia cases).</p> <p>Conclusions</p> <p>Our findings suggest good agreement in the interpretation of VRI data between different raters. In this respect, VRI might be helpful as a radiation free diagnostic tool for the management of pneumonia.</p

Epidemiology of ILDs and their progressive-fibrosing behaviour in six European countries

The PERSEIDS study aimed to estimate incidence/prevalence of interstitial lung diseases (ILDs), fibrosing Interstitial lung diseases (F-ILDs), idiopathic pulmonary fibrosis (IPF), systemic sclerosis-associated ILD (SSc-ILD), other non-IPF F-ILDs and their progressive-fibrosing (PF) forms in six European countries, as current data are scarce.This retrospective, two-phase study used aggregate data (2014–2018). In Phase 1, incident/prevalent cases of ILDs above were identified from clinical databases through an algorithm based on codes/keywords, and incidence/prevalence was estimated. For non-IPF F–ILDs, the relative percentage of subtypes was also determined. In Phase 2, a subset of non-IPF F-ILD cases was manually reviewed to determine the percentage of PF behaviour and usual interstitial pneumonia-like (UIP-like) pattern. A weighted mean percentage of progression was calculated for each country and used to extrapolate incidence/prevalence of progressive-fibrosing ILDs (PF–ILDs).In 2018, incidence/105 person-years ranged between 9.4–83.6(ILDs), 7.7–76.2(F-ILDs), 0.4–10.3(IPF), 6.6–71.7(non-IPF F-ILDs) and 0.3–1.5(SSc-ILD); and prevalence/105 persons ranged between 33.6–247.4(ILDs), 26.7–236.8(F-ILDs), 2.8–31.0(IPF), 22.3–205.8(non-IPF F-ILDs) and 1.4–10.1(SSc-ILD). Among non-IPF F-ILDs, sarcoidosis was the most frequent subtype. PF behaviour and UIP-like pattern were present in a third of non-IPF F-ILD cases each and hypersensitivity pneumonitis showed the highest percentage of progressive behaviour. Incidence of PF-ILDs ranged between 2.1–14.5/105 person-years, and prevalence between 6.9–78.0/105 persons.To our knowledge, PERSEIDS is the first study assessing incidence, prevalence and rate of progression of ILDs across several European countries. Still below the threshold for orphan diseases, the estimates obtained were higher and more variable than reported in previous studies, but differences in study design/population must be considered.</p

Serum VEGF levels are related to the presence of pulmonary arterial hypertension in systemic sclerosis

<p>Abstract</p> <p>Background</p> <p>The association between systemic sclerosis and pulmonary arterial hypertension (PAH) is well recognized. Vascular endothelial growth factor (VEGF) has been reported to play an important role in pulmonary hypertension. The aim of the present study was to examine the relationship between systolic pulmonary artery pressure, clinical and functional manifestations of the disease and serum VEGF levels in systemic sclerosis.</p> <p>Methods</p> <p>Serum VEGF levels were measured in 40 patients with systemic sclerosis and 13 control subjects. All patients underwent clinical examination, pulmonary function tests and echocardiography.</p> <p>Results</p> <p>Serum VEGF levels were higher in systemic sclerosis patients with sPAP ≥ 35 mmHg than in those with sPAP < 35 mmHg (352 (266, 462 pg/ml)) vs (240 (201, 275 pg/ml)) (p < 0.01), while they did not differ between systemic sclerosis patients with sPAP < 35 mmHg and controls. Serum VEGF levels correlated to systolic pulmonary artery pressure, to diffusing capacity for carbon monoxide and to MRC dyspnea score. In multiple linear regression analysis, serum VEGF levels, MRC dyspnea score, and D_LCOwere independent predictors of systolic pulmonary artery pressure.</p> <p>Conclusion</p> <p>Serum VEGF levels are increased in systemic sclerosis patients with sPAP ≥ 35 mmHg. The correlation between VEGF levels and systolic pulmonary artery pressure may suggest a possible role of VEGF in the pathogenesis of PAH in systemic sclerosis.</p

A Qualitative Content Analysis of Spirituality and Religiosity amongst Greek COPD Patients

Chronic Obstructive Pulmonary Disease (COPD) is a chronic and common disease throughout the world. Spiritual/religious beliefs are often central to patients with serious illnesses and could serve as a resource for coping with such illnesses. A qualitative methodology was chosen in order to gain a thorough understanding of 75 (male n = 69, 92%, female n = 6, 8%) Greek COPD patients’ perceptions of spirituality and religiosity and explore the importance of practicing their beliefs while 25 patients refused to participate in the study. The fewer female participant patients in the study are related to the lower percentage of women suffering from COPD in Greece, which can be further linked to the low smoking habit of women. A total of four patients (5.3%) stated that they had no religious or spiritual understanding of their life; 41 (54.7%) reported a religious belief; eight (10.7%) told of a spiritual belief; and 22 (29.3%) of the participants reported both a religious and a spiritual belief. Faith in the will of God seems to be a particularly strong feature of patients’ beliefs. Praying and church attendance were more likely to confirm the importance of practicing their beliefs in their daily lives but COPD seems to prevent patients from regular churchgoing. Religion and spirituality were found to be helpful while patients did not seem to have unrealistic expectations from God when dealing with illness. When asked, participants defined spirituality as “happiness”, “love”, “our God”, “the spirit”, and as an act of altruism. Adopting a more holistic perspective for COPD patients in the clinical setting, spirituality and religiosity can offer suggestions for interventions related to their health issues

Physical Activity and Quality of Sleep in Patients with End-Stage Renal Disease on Hemodialysis: A Preliminary Report

Chronic kidney disease significantly impairs patients’ daily lives and worsens their quality of life. The aim of this study was to investigate the physical activity and quality of sleep, during three days (previous day of dialysis, on the day of dialysis and after day of dialysis), in patients with end-stage renal on hemodialysis. 12 hemodialysis patients were included in our study, answered the Pittsburgh Sleep Quality Index (PSQI) questionnaire, and for each patient were used a smart bracelet for three days (day-pre- and posthemodialysis and day at hemodialysis) to record daily physical activity (steps, distance) and estimate the quality of sleep. Results showed differences between three days average of steps and distance and PSQI parameters “…engaging in social activity?” (steps, p=0.006, distance, p=0.006) and “…enthusiasm to get things done?” (steps, p=0.029, distance, p=0.030). Our study suggests interrelationship between sleep quality and physical activity