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Background: Air pollution affects idiopathic pulmonary fibrosis (IPF) patients and is a risk factor for IPF-Acute-Exacerbation (IPF-AE). Pathogenetic mechanisms could implicate pathways of tissue regeneration and repair following injury. Aim and objectives: To examine the association of long-term air pollution exposure with inflammatory mediators involved in lung repair, in IPF and AE-IPF. Methods: Consecutive IPF patients referred to our Department from October 2013 to June 2019 were included. Diagnoses were based on international guidelines. Long-term personal air pollution exposures were assigned to each patient retrospectively, for O3, NO2, PM2.5, PM10 based on geo-coded residential addresses. Inflammatory mediators (IL-1a, IL-1b, IL-4, IL-5, IL-6, IL-8, IL-10, IL-13, active TGF-β, TNFa, CCL2, CCL3, CCL-18, MMP-1, MMP-7, MMP-9, osteopontin) were measured in the peripheral blood by ELISA. Linear regression models were used adjusting for confounders. Results: 118 IPF patients (mean age 72±8.3 years) were included. In AE-IPF, a 10μg/m3 increase in O3 was significantly positively associated with 40.4% change of IL-4 (p=0.011) and marginally with 40% a 62.2% changes of IL-13 (p=0.052) and osteopontin (p=0.097). PM2.5, PM10 and NO2 were inversely associated with 50.6, 50.8 a 21.7% changes of IL-4 (p=0.003, p=0.003, p=0.022, respectively) and 73.2, 74.6 a 32.4% changes of osteopontin (p=0.024, p=0.022, p=0.097, respectively). In stable IPF patients, PM2.5 and PM10 were positively associated with an increase in the 76% changes of IL-13 (p=0.059, p=0.058, respectively). Conclusion: Our findings support an association of IL-4, IL-13 and osteopontin with IPF-AE related to air pollution long-term exposure. Discrepancies in the impact of different pollutants need further investigation.

Background: Telomere-related genes (TRG) mutations are detected in 30% of familial pulmonary fibrosis (FPF) and are also associated with personal/familial extra-pulmonary disease suggestive of short telomere syndrome (STS). Aim: to evaluate the prevalence of TRG mutations in a national cohort of interstitial lung disease (ILD) with suspected genetic predisposition in Greece and to define patients’ characteristics and impact on outcome.Methods: Genetic diagnoses made between December 2014-September 2019 in patients with FPF, pulmonary fibrosis at young age or personal and/or family extra-pulmonary disease suggestive of STS were analyzed. Results: 150 ILD patients, 73% male, age-at-diagnosis 67 years old, 33% non-smokers, 75% with definite/probable UIP or CPFE, 93% with dyspnea/cough, FVC% pred 75%, DLCO% pred 49% were tested; FPF was 65%, 19% young age, 21% personal and 17% family extra-pulmonary disease suggestive of STS; 21% were tested for more than 1 indication. TRG pathogenic variations were detected in 19 (13%): 13 in a familial, 3 in young age and 3 in extra-pulmonary disease suggestive of STS context as follows: TERT, TERC, RTEL1, PARN, NOP10, NHP2 in 8,5,2,2,1 and 1 respectively. No difference was detected in epidemiological-clinical-radiologic-functional characteristics between patients without and with mutations apart from age-at-diagnosis [69 vs 62 years, p

A 73-year-old Caucasian male, ex-smoker with a smoking history of 50 pack-years, presented to our department complaining of chronic cough, gradually progressive shortness of breath on exertion and fatigue during recent months. He did not report any chest pain or fever. The patient’s medical history only included arterial hypertension, while in the past he worked in the stone crushing industry., Can you diagnose this man presenting with chronic cough and progressive shortness of breath on exertion and fatigue developing over several months? http://ow.ly/ekZd30k8VcM

Introduction: Short telomeres are a risk factor for idiopathic pulmonary fibrosis (IPF). Few data exist for telomere length (TL) in acute exacerbation of IPF (AE-IPF) as well as in other fibrotic interstitial lung diseases (ILD). Aim: To measure TL in sporadic fibrotic ILDs including IPF, AE-IPF, Collagen-Vascular-Disease (CVD), Combined pulmonary fibrosis-emphysema (CPFE) and compare them with age-matched healthy controls in the Greek population. Methods: Consecutive ILD patients were enrolled. TL was measured from peripheral white blood cells using a multiplex quantitative polymerase chain reaction (qPCR). The relative TL was estimated from the ratio of the telomere repeat copy number to a single gene (human b-globin gene) copy number (telomere/single-copy gene [T/S] ratio). Results: 84 participants were studied [mean age (68.6±0.71) years], 24 stable IPF (69.7±1.2), 18 ΑE-IPF (70.0±1.6), 7 CTD-ILDs (68.8±3,4), 10 CPFE (69.7±1.4) and 25 age-matched healthy controls (66.0±1.2). TL in all ILD patients was significantly shortened compared to controls [mean T/S ratio (SE) (0.79±0.05) vs (2.11±0.29), p 0.72, 7.0±1.68 months vs 14.4±4.3), no significant association was observed in overall survival. Conclusion: Short TL was detected across all fibrotic groups of ILD. Further shortening of TL during AE-IPF was not found. “Grant of Hellenic Thoracic Society”

Acute exacerbation of Idiopathic Pulmonary Fibrosis (AE-IPF) represents an acute, clinically significant respiratory deterioration of unidentifiable cause with high mortality. The potential role of air pollution on this devastating event in the progress of IPF has not been yet elucidated. This study aims to determine the association between air pollution exposure and AE-IPF. All consecutive IPF patients referred to our department from October-2013 to October-2016 were studied. Demographic, functional data and AE-IPF events were recorded. Blood levels of IL-6 and IL-8 were measured. Air pollution exposures were assigned to each patient retrospectively, for ozone, nitrogen dioxide and particulate matter, based on geo-coded residential addresses. Logistic regression models and Spearman correlation were used to assess air pollutants’ association with AE-IPF and pro-inflammatory mediators. A total of 94 IPF (43 AE-IPF) patients were included in the analysis. AE-IPF patients presented significantly higher levels of IL-6 and IL-8 compared to stables. A positive, non-significant association, between AE-IPF and a 10 μg/m 3 increase in previous year mean level of NO 2 (OR=1.021, 95% CI 0.825-1.265) and O 3 (OR=1.002, 95% CI 0.769-1.305) as well as short-term exposure to NO 2 (OR Lag0-1 =3.024, 95% CI 0.631-14.498; OR antecedent month =3.586, 95% CI 0.565-22.741; OR antecedent 6weeks =3.554, 95% CI 0.470-26.860) was found. A significant positive association was observed between IL-8 and NO 2 whilst a negative one with O 3 for all patients. There is some indication that short as well as long term exposure to increased concentrations of air pollutants is associated to AE-IPF. Studies with more patients to achieve appropriate statistical power are needed.

To date no nationwide epidemiological study of asthma in the general population has been performed in Greece. The present study evaluated the prevalence of asthma and asthma related symptoms (ARS) in a randomly selected sample representative of the adult general population in Greece. The EΜEΝΟ survey (National Morbidity and Risk Factors Survey) supported by the European Social Fund and national funds, is currently performed in Greece, aiming to include 6,000 adults using a multi-stage stratified random sampling method, involving localities throughout the country. Information on asthma and ARS during the last 12 months was collected through questionnaires administered by trained interviewers. An interim analysis of participants recruited from 03/2014 to 12/2015 was performed. Prevalence of asthma and ARS was determined taking into account the design of the study and additional adjustments for age and sex. A total of 5,137 participants were included. The prevalence of asthma was 6.3% (CI 95%: 5,6%-7,0%) and it was higher in women (7.2% vs 5.3% in men, p=0.011). We also observed higher prevalences of ARS in women except wheezing. Prevalences of ARS (overall) are presented below Asthma and asthma related symptoms prevalence in the general adult population in Greece are higher than the global ones estimated by the World Health Survey (To et al, BMC Public Health 2012; 12: 204).

The precise pathogenetic mechanisms and the potential role of uncontrolled fibroblastic proliferation or occult infection in IPF acute exacerbations need clarification. The aim of the study was the assessment of profibrotic and proinflammatory profile in both stable and excacerbated patients and their association to outcome. Stable or exacerbated patients referred to our department from October 2013 until November 2014 were included. IL-6, IL-8 and TGF-b were measured. All patients were followed up for 12 months. Tobit and logistic regression analysis were applied. Forty-one patients were included in the study [18 exacerbated, median age (IQR) 68.5 (67.0, 78.0) and 23 with stable IPF, median age (IQR) 71.0 (69.0, 74.0)]. Exacerbated patients presented higher plasma levels of IL-6 and IL-8 compared to those with stable IPF [median (IQR) 6.2 (3.2, 26.5) vs 2.1 (1.0, 4.4) pg/ml, p=0.002; median (IQR) 16.5 (12.9, 44.8) vs 10.1 (7.9, 13.2) pg/ml, p=0.046, respectively]. Most of the differences were independent to age, smoking status, procalcitonin, CRP, corticosteroid and oxygen treatment. No significant differences were observed in the profibrotic cytokine TGF-b between the two groups. An increase of IL-6 or IL-8 by 1 pg/ml increased the odds of death by 5,6% (p=0.021) and 6,7% (p=0.013) respectively in all patients and by 3,8% (p=0.093) and 4,3% (p=0.078) in exacerbated patients. The increased plasma levels of IL-6 and IL-8 found in exacerbated patients compared to stable suggest the presence of inflammation that may also relate to occult infection. Furthermore, our results indicate that plasma levels of IL-6 and IL-8 are associated with increased risk of death in all.

To date no nationwide epidemiological study of asthma in the general population has been performed in Greece. The present study examines the association of asthma and asthma-related symptoms (ARS) with obstructive pattern (OP) in the adult general population in Greece. The EΜEΝΟ survey (National Morbidity and Risk Factors Survey) supported by the European Social Fund and national funds is currently performed in Greece, aiming to include 6,000 adults using a multi-stage stratified random sampling method throughout the country. Information on asthma and ARS was collected through questionnaires administered by interviewers. Participants were also submitted to spirometry. An interim analysis of participants recruited from 03/2014 to 12/2015 was performed. A total of 1980 participants who had technically acceptable spirometry were included in the study. Individuals reporting asthma or ARS had significantly higher prevalence of OP in spirometry than those who did not report. High percentages of OP were also observed among individuals with known asthma and ARS. Our results indicate a high proportion of obstructive pattern among individuals with known asthma and ARS in the population of Greece. Partially controlled asthma may be an explanation. Further studies are needed to elucidate potential factors such as socioeconomic, under treatment, smoking, persistent environmental exposures that may be implicated.