Long-Term Lung Sequelae in Survivors of Severe/Critical COVID-19 Pneumonia: The "Non-Steroid", "Non-Interventional" Approach.

: Long-term lung sequelae in severe COVID-19 survivors, as well as their treatment, are poorly described in the current literature. : To investigate lung fibrotic sequelae in survivors of severe/critical COVID-19 pneumonia and their fate according to a "non-interventional" approach. : Prospective study of the above COVID-19 survivors after hospital discharge from March 2020 to October 2022.

Clinical remission in patients with severe eosinophilic asthma treated with mepolizumab: A analysis of RELIght study.

Remission of asthma can occur as part of the natural history of the disease; however, the use of biologics can result in disease remission in some patients. In this post hoc analysis of the RELIght study, we aimed to evaluate clinical remission in real life among patients treated with mepolizumab, to detect possible differences between "remitters" and "nonremitters," and to evaluate possible predictors of remission. Clinical remission was defined as the absence of asthma exacerbations, discontinuation of oral corticosteroids (OCS), achievement of asthma control (Asthma Control Test [ACT] ≥ 20), and stable or improved lung function.

Childhood interstitial lung disease survivors in adulthood: a European collaborative study.

Background: Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood.

Aim: To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres.

Investigating a middle‑step COVID‑19 unit in Greece.

During the coronavirus disease-19 (COVID-19) pandemic, there was an unprecedented requirement for hospital bed availability. The present study aimed to examine the characteristics and outcomes of patients hospitalized in a COVID-19 unit that operated as a novel middle-step unit in Greece. The present study aimed to determine whether the middle-step unit supported the central general hospitals; thus, highlighting the potential of these models in future pandemics.

Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults.

Background And Objective: Variants in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults, with few studies in adults.

Methods: We conducted a multicentre retrospective study of all consecutive adult patients diagnosed with ILD associated with variants in SFTPC or ABCA3 in the French rare pulmonary diseases network, OrphaLung. Variants and chest computed tomography (CT) features were centrally reviewed.

mutation-related hereditary pulmonary alveolar proteinosis: the "long and winding road" into adulthood.

https://bit.ly/3QcsYwM.

Lung transplantation outcome in adult surfactant-related interstitial lung disease: first evidence to move on.

https://bit.ly/3QxG73Z.

Effects of short-term air pollution exposure on symptoms development in the course of idiopathic pulmonary fibrosis.

Background: Lately a potential detrimental effect of air pollution to idiopathic pulmonary fibrosis emerged. We aimed to assess the effects of short-term air pollution exposure to the clinical course of IPF.

Research Design And Methods: IPF patients were followed intensively for four nonconsecutive study periods between 13 July 2020 and 5 September 2021.

From Karl Wurm and Guy Scadding's staging to F-FDG PET/CT scan phenotyping and far beyond: perspective in the evading history of phenotyping in sarcoidosis.

Sarcoidosis is an inflammatory granulomatous disease of unknown etiology involving any organ or tissue along with any combination of active sites, even the most silent ones clinically. The unpredictable nature of the sites involved in sarcoidosis dictates the highly variable natural history of the disease and the necessity to cluster cases at diagnosis based on clinical and/or imaging common characteristics in an attempt to classify patients based on their more homogeneous phenotypes, possibly with similar clinical behavior, prognosis, outcome, and therefore with therapeutic requirements. In the course of the disease's history, this attempt relates to the availability of a means of detection of the sites involved, from the Karl Wurm and Guy Scadding's chest x-ray staging through the ACCESS, the WASOG Sarcoidosis Organ Assessment Instruments, and the GenPhenReSa study to the F-FDG PET/CT scan phenotyping and far beyond to new technologies and/or the current "omics.

Diffuse panniculitis in a teenage male with ZZ α-antitrypsin deficiency.

https://bit.ly/3EDmCzT.

COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study.

https://bit.ly/3M0wKnZ.

Genetics in Idiopathic Pulmonary Fibrosis: A Clinical Perspective.

Background: Unraveling the genetic background in a significant proportion of patients with both sporadic and familial IPF provided new insights into the pathogenic pathways of pulmonary fibrosis.

Aim: The aim of the present study is to overview the clinical significance of genetics in IPF.

Perspective: It is fascinating to realize the so-far underestimated but dynamically increasing impact that genetics has on aspects related to the pathophysiology, accurate and early diagnosis, and treatment and prevention of this devastating disease.

Autoimmune pulmonary alveolar proteinosis in children.

In childhood, a multitude of causes lead to pulmonary alveolar proteinosis (PAP), an excessive surfactant accumulation in the alveolar space, limiting gas exchange. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) causing autoimmune PAP, the principal aetiology in adults, are rare. In this first case series on autoimmune PAP, we detail the presentation and management issues of four children.

Long-term personal air pollution exposure and risk for acute exacerbation of idiopathic pulmonary fibrosis.

Background: Urban air pollution is involved in the progress of idiopathic pulmonary fibrosis (IPF). Its potential role on the devastating event of Acute Exacerbation of IPF (AE-IPF) needs to be clarified. This study examined the association between long-term personal air pollution exposure and AE- IPF risk taking into consideration inflammatory mediators and telomere length (TL).

MUC5B promoter variant rs35705950 and rheumatoid arthritis associated interstitial lung disease survival and progression.

Background: The major risk factor for idiopathic pulmonary fibrosis (IPF), MUC5B rs35705950, was found to be associated with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Whilst the MUC5B rs35705950 T risk allele has been associated with better survival in IPF, its impact on RA-ILD prognosis remains to be determined. Our objective was to explore the influence of MUC5B rs35705950 on survival and progression in RA-ILD.

Vedolizumab-induced acute interstitial lung injury in a 39-year-old male with ulcerative colitis.

Vedolizumab, an anti-integrin antibody, is effective for moderate to severe ulcerative colitis and Crohn's disease treatment with a good safety profile due to its gut selective mechanism of action. Upper respiratory tract vedolizumab adverse events are common; however, they are mild and do not require treatment withdrawal. Herein, we present a 39-year-old patient under vedolizumab treatment for ulcerative colitis who presented acute severe interstitial lung injury that necessitated vedolizumab withdrawal and systemic steroids administration.

Inhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis.

Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by progressive surfactant accumulation and hypoxemia. It is caused by disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling, which pulmonary alveolar macrophages require to clear surfactant. Recently, inhaled GM-CSF was shown to improve the partial pressure of arterial oxygen in patients with aPAP.