Severe coronavirus disease (COVID-19) is characterized by an excessive proinflammatory cytokine storm, resulting in acute lung injury and development of acute respiratory distress syndrome (ARDS). The role of corticosteroids is controversial in severe COVID-19 pneumonia and associated hyper-inflammatory syndrome. We reported a case series of six consecutive COVID-19 patients with severe pneumonia, ARDS and laboratory indices of hyper-inflammatory syndrome. All patients were treated early with a short course of corticosteroids, and clinical outcomes were compared before and after corticosteroids administration. All patients evaded intubation and intensive care admission, ARDS resolved within 11.8 days (median), viral clearance was achieved in four patients within 17.2 days (median), and all patients were discharged from the hospital in 16.8 days (median). Early administration of short course corticosteroids improves clinical outcome of patients with severe COVID-19 pneumonia and evidence of immune hyperreactivity.

Although studies show that control of asthma can be achieved in the majority of patients, surveys repeatedly show that this is not the case in real life. Important measures to implement in order to achieve asthma control are trained healthcare professionals, a good patient-doctor relationship, patient education, avoidance of exposure to triggers, personalised management and adherence to treatment. These measures help the majority of asthma patients but have not yet been widely implemented and there should be a concerted action for their implementation. Moreover, further and focused research is needed in severe/refractory asthma. @ERSpublications Achieving asthma control requires implementation of evidence-based guidelines and further research into severe asthma http://ow.ly/KzrOp

Background and Objective There remains a paucity of large databases for patients with idiopathic pulmonary fibrosis (IPF) and lung cancer. We aimed to create a European registry. Methods This was a multicentre, retrospective study across seven European countries between 1 January 2010 and 18 May 2021. Results We identified 324 patients with lung cancer among 3178 patients with IPF (prevalence = 10.2%). By the end of the 10 year‐period following IPF diagnosis, 26.6% of alive patients with IPF had been diagnosed with lung cancer. Patients with IPF and lung cancer experienced increased risk of all‐cause mortality than IPF patients without lung cancer (HR: 1.51, [95% CI: 1.22–1.86], p < 0.0001). All‐cause mortality was significantly lower for patients with IPF and lung cancer with a monocyte count of either <0.60 or 0.60–<0.95 K/μl than patients with monocyte count ≥0.95 K/μl (HR [<0.60 vs. ≥0.95 K/μl]: 0.35, [95% CI: 0.17–0.72], HR [0.60–<0.95 vs. ≥0.95 K/μl]: 0.42, [95% CI: 0.21–0.82], p = 0.003). Patients with IPF and lung cancer that received antifibrotics presented with decreased all cause‐mortality compared to those who did not receive antifibrotics (HR: 0.61, [95% CI: 0.42–0.87], p = 0.006). In the adjusted model, a significantly lower proportion of surgically treated patients with IPF and otherwise technically operable lung cancer experienced all‐cause mortality compared to non‐surgically treated patients (HR: 0.30 [95% CI: 0.11–0.86], p = 0.02). Conclusion Lung cancer exerts a dramatic impact on patients with IPF. A consensus statement for the management of patients with IPF and lung cancer is sorely needed.

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Germline telomere-related gene mutations are associated with familial pulmonary fibrosis and lead to short telomere syndrome [1,2]. Most of the short telomere syndrome-related genes, such as TERT, TERC, DKC1, TINF2, NHP2 and NOP10, were identified in dyskeratosis congenita patients with early onset mucocutaneous manifestations and/or bone marrow failure [3]. The telomerase complex includes TERT, the TERC RNA and DKC1. NOP10, along with DKC1, NHP2 and GAR1, is essential for TERC stability and telomere maintenance [4]. Homozygous NOP10 mutation (c.100C>T, p.Arg34Trp) has been reported only once in a consanguineous family with autosomic recessive dyskeratosis congenita without pulmonary fibrosis [5]. Moreover, the p.Arg34Trp NOP10 mutation caused telomere shortening in homozygous and heterozygous carriers [5]. Here we provide evidence that a heterozygous NOP10 mutation (c.17A>G,p. Tyr6Cys) identified in a large family co-segregates with adult-onset familial pulmonary fibrosis.The proband (II.1), a 68-year-old non-smoker female, was diagnosed with pulmonary fibrosis at the age of 66 years (figure 1a and b). Two deceased, a brother (II.2) and sister (II.6), and one alive sister (II.7) were also diagnosed with pulmonary fibrosis (figure 1c). Another brother (II.4) and his son (III.5) died from leukaemia while one of the sons of the proband (III.2) had long-term leukopenia. The deceased father of the proband (I.1) was diagnosed with non-alcoholic liver cirrhosis (figure 1a). Mucocutaneous manifestations were not observed upon examination in this family.We identified by whole exome sequencing in the proband (II.1) a heterozygous pathogenic missense mutation in NOP10 (NM_ 018648) c.17A>G,p.Tyr6Cys according to international recommendations (figure 1a) [6]. No other rare variants predicted to be deleterious were found in whole exome data from the proband in the other genes linked to short telomere syndrome (TERT, TERC, DKC1, TINF2, RTEL1, PARN, NAF1, ACD, NHP2, WRAP53, SHQ1 and ZCCHC8) [7-9]. The variation is absent in 140 000 individuals from the gnomAD database. The mutated adenine is conserved at a genomic level (GERP=5). The in silico tools Polyphen 2 and CADD predicted a deleterious impact of the amino acid change p. Tyr6Cys with scores of 1 and 24, respectively, reflecting high conservation at a protein level. Flow cytometry and FISH (flowFISH) revealed that telomere length of the proband were shorter than the first percentile [6]. It was not possible to organise flowFISH analysis for all individuals of this family. Thus, telomere length for the individuals II.1, II.7, III.1 and III.2 was also measured through telomeric restriction fragment assay [9] at 7.4 kb, 6.3 kb, 8.5 kb and 8.6 kb, respectively. Telomere length for the individuals III.1 and III.2 were found to be shorter relative to the mother's telomere length [2]. The fact that non-carrier (III.1) and carrier (III.2) brother were found to have the same range of telomere length could be attributed to the "heritability" of telomere length and evocates epigeneti...