These data suggest that sarcoidosis and idiopathic pulmonary fibrosis are the most frequent ILDs in our population. In comparison with the few previous reports, interesting dissimilarities have been observed.

Background and objective The acute effects of e‐cigarettes have not been scientifically demonstrated yet. The aim of this study was to assess the acute changes in pulmonary function and airway inflammation in patients with asthma after vaping one e‐cigarette. Methods Twenty‐five smokers suffering from stable moderate asthma according to GINA guidelines with no other comorbidities and 25 healthy smokers matched with the baseline characteristics of the asthmatic patients were recruited. PFT, IOS, FeNO and EBC were performed before and after vaping one e‐cigarette with nicotine. pH and concentrations of IL‐1β, IL‐4, IL‐5, IL‐6, IL‐8, IL‐10, IL‐13, IL‐17A, TNF‐α, ISO8 and LTB4 were measured in EBC. Results FFEV1/FVC ratio and PEF were reduced in asthmatic patients after e‐cigarette. Z5Hz and R5Hz, R10Hz and R20Hz increased in both groups. FeNO and EBC pH increased by 3.60 ppb (P = 0.001) and 0.15 (P = 0.014) in asthmatic patients after e‐cigarette, whereas they decreased in control group by 3.28 ppb (P < 0.001) and 0.12 (P = 0.064), respectively. The concentrations of IL‐10, TNF‐α and ISO8 in EBC increased in asthmatic patients after e‐cigarette and the changes in concentrations of IL‐1β and IL‐4 differed significantly between the two groups. Conclusion E‐cigarette vaping resulted in acute alteration of both pulmonary function and airway inflammation in stable moderate asthmatic patients.

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<b><i>Background:</i></b> In patients with idiopathic pulmonary fibrosis (IPF) with isolated exertional desaturation, there are limited data regarding the effectiveness of oxygen supplementation during exercise training; the underlying mechanisms that contribute to these responses are unknown. <b><i>Objectives:</i></b> To examine in these IPF patients the effects of oxygen supplementation during submaximal exercise (vs. medical air) on cerebral/skeletal muscle oxygenation and systemic hemodynamics. <b><i>Methods:</i></b> In this randomized, cross-over, placebo-controlled trial, IPF patients (<i>n</i> = 13; 63.4 ± 9.6 years) without resting hypoxemia but a significant desaturation during maximal cardiopulmonary exercise testing underwent 2 steady-state exercise trials (65% peak-work-load), breathing either oxygen-enriched or medical air. Cerebral/skeletal muscle oxygenation (near-infrared spectroscopy) and beat-by-beat hemodynamics (photoplethysmography) were monitored. <b><i>Results:</i></b> In the air protocol, from the initial minutes of submaximal exercise, patients exhibited a marked decline in cerebral oxygenated hemoglobin (O₂Hb) and an abrupt rise in deoxygenated hemoglobin (HHb). Oxygen supplementation alleviated desaturation, lessened dyspnea, and prolonged exercise duration (<i>p</i> &#x3c; 0.01). Oxygen supplementation during exercise (i) attenuated cerebral deoxygenation (cerebral-HHb: 0.7 ± 1.9 vs. 2.5 ± 1.5 μmol/L, O₂ and air protocol; <i>p</i> = 0.009) and prevented cerebral-Hb_difference decline (2.1 ± 2.7 vs. −1.7 ± 2.0 μmol/L; <i>p</i> = 0.001), (ii) lessened the decline in muscle O₂-saturation index, and (iii) at isotime exercise, it resulted in lower muscle-HHb (<i>p</i> = 0.05) and less leg fatigue (<i>p</i> &#x3c; 0.05). No differences between protocols were observed in exercise cardiac output and vascular resistance. <b><i>Conclusions:</i></b> IPF patients with isolated exertional hypoxemia exhibit an inability to increase/maintain cerebral oxygenation during submaximal exercise. Correcting desaturation with O₂ supplementation prevented the decline in brain oxygenation, improved muscle oxygenation, and lessened dyspnea, suggesting an efficacy of acute oxygen supplementation during exercise training in protecting brain hypoxia in these IPF patients.

<b><i>Background:</i></b> Monogenic and polygenic inheritances are evidenced for idiopathic pulmonary fibrosis (IPF). Pathogenic variations in surfactant protein-related genes, telomere-related genes (TRGs), and a single-nucleotide polymorphism in the promoter of <i>MUC5B</i> gene encoding mucin 5B (rs35705950 T risk allele) are reported. This French-Greek collaborative study, Gen-Phen-Re-GreekS in inheritable IPF (iIPF), aimed to investigate genetic components and patients’ characteristics in the Greek national IPF cohort with suspected heritability. <b><i>Patients and Methods:</i></b> 150 patients with familial PF, personal-family extrapulmonary disease suggesting short telomere syndrome, and/or young age IPF were analyzed. <b><i>Results:</i></b> <i>MUC5B</i> rs35705950 T risk allele was detected in 103 patients (90 heterozygous, 13 homozygous, allelic frequency of 39%), monoallelic TRG pathogenic variations in 19 patients (8 <i>TERT</i>, 5 <i>TERC</i>, 2 <i>RTEL1</i>, 2 <i>PARN</i>, 1 <i>NOP10</i>, and 1 <i>NHP2</i>), and biallelic <i>ABCA3</i> pathogenic variations in 3. Overlapping <i>MUC5B rs35705950</i> T risk allele and TRG pathogenic variations were shown in 11 patients (5 <i>TERT</i>, 3 <i>TERC</i>, 1 <i>PARN</i>, 1 <i>NOP10</i>, and 1 <i>NHP2</i>), <i>MUC5B</i> rs35705950 T risk allele, and biallelic <i>ABCA3</i> pathogenic variations in 2. In 38 patients, neither <i>MUC5B</i> rs35705950 T risk allele nor TRG pathogenic variations were detectable. Kaplan-Meier curves showed differences in time-to-death (<i>p</i> = 0.025) where patients with <i>MUC5B</i> rs35705950 T risk allele alone or in combination with TRG pathogenic variations presented better prognosis. <b><i>Conclusion:</i></b> The Gen-Phen-Re-GreekS in iIPF identified multiple and overlapping genetic components including the rarest, underlying disease’s genetic “richesse,” complexity and heterogeneity. Time-to-death differences may relate to diverse IPF pathogenetic mechanisms implicating “personalized” medical care driven by genotypes in the near future.