Pirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF). We present our real-world experience in terms of Pirfenidone’s effect on mortality and adverse events profile outside the restrictions of a clinical trial. This is a retrospective observational intention to treat study of 82 consecutive IPF patients (UHH cohort). We observed a high 3-years survival rate of 73% without excluding patients who discontinued treatment for different reasons. The survival was compared to the survival of an IPF cohort from a tertiary referral center (RBH cohort). After exclusion of severe cases (DLco< 30%), in unadjusted analysis, the survival in the UHH cohort was better than in the RBH cohort (HR:0.32, 95% CI: 0.19–0.53, p < 0.0001). After adjustment for age, gender and FVC, the survival remained higher in the UHH cohort (HR:0.28, 95% CI: 0.16–0.48, p < 0.0001). We observed a similar safety profile compared to previously published data and a lower rate of drug discontinuation due to photosensitivity reactions. Conclusion: Pirfenidone provides a survival benefit in a real-life IPF cohort compared to previously used medications. Counselling patients and proactively managing possible adverse effects can reduce the necessity to discontinue pirfenidone.

This review summarises some of the key features of interstitial lung diseases (ILDs) from a translational science point of view and brings insights into potential therapeutic options. Genetic predisposition and environmental factors like smoking, pollution and infections significantly impact the onset, progression and treatment response in ILDs, highlighting the need for personalised management. Fibroblasts are central to ILD pathology, influencing the tissue microenvironment, immune cell interactions and extracellular matrix (ECM) production, making them critical therapeutic targets. Monocyte-derived M2 macrophages drive fibrosis in idiopathic pulmonary fibrosis by secreting cytokines and remodelling the ECM. Understanding macrophage subtypes and their dynamics offers new therapeutic possibilities. Chronic type 2 immunity contributes to fibrosis, emphasising the need to enhance protective markers in order to even out the balance shift of pathological immune responses in ILD treatments. Serum biomarkers like Krebs von den Lungen-6 (KL-6), surfactant protein (SFTP) D, matrix metalloproteinase-7 (MMP-7), and C-C motif chemokine ligand (CCL)-18 are valuable for diagnosing and predicting ILD progression, although more research is needed for clinical application. Animal models, especially bleomycin-based models, offer insights into ILD pathology, but challenges like lung hyperinflation highlight the need for careful model selection and translational research to bridge preclinical and clinical findings.

Hypersensitivity pneumonitis (HP) is a complex interstitial lung disease caused by chronic inhalation of a wide variety of antigens in susceptible and sensitized individuals, commonly associated with an occupational exposure. An impressive number of inciting antigens causing hypersensitivity pneumonitis have been found to cover a wide range of occupations. As working practices have changed over time, especially in industrialized countries, new names for occupational HP have emerged. This review emphasizes the main diagnostic issues arising from the high variability of clinical presentation and the broad spectrum of causal antigens. Furthermore, it provides an overview of current methods to unveil possible causes of hypersensitivity pneumonitis, highlights HP’s current diagnostic and treatment challenges and the remaining areas of uncertainty, and presents prevention strategies.

Double-origin right bronchial artery is a rare anatomical variation with varying presentation. Patients can be asymptomatic, but in case they present with hemoptysis, such anatomical variations may lead to clinical dilemmas. In this case report, we describe a young patient who presented with...

Changes in mitochondrial quality control are crucial in lung diseases, and in pulmonary fibrosis susceptibility. Diminished mitochondrial quality control results in mitochondrial dysfunction and increased ROS production. Recently, we showed that mitochondria in BAL AMs from IPF patients have a significant increase in mitochondrial ROS (mtROS) associated with reduced expression of mitochondria-encoded OXPHOS genes (1).The distinction between IPF and non-IPF ILDs is important given the worse prognosis in IPF, and the different therapeutic approaches. Chronic Hypersensitivity pneumonitis (CHP), which is considered antigen-driven, can have comparable outcome to that of IPF, although associated with airway inflammation and chronic airway injury. In this study we evaluated the levels of mtROS in ILD patients, in fresh BAL cells at the time of diagnosis. The levels of mtROS were measured by flow cytometry. Healthy subjects were also included. Patients’ characteristics were analysed, including pulmonary function tests. An increase in mtROS levels was demonstrated in IPF (p<0.05) relative to controls , as well as in CHP patients relative to controls (p<0.05).There was also a trend of higher mitoSOX levels in CHP when compared with IPF. MitoSOX levels showed significant inverse correlation with diffusion capacity for carbon monoxide (DLco%). Furthermore, the levels of mtROS significantly correlated with patients’ age. Our results showed elevated levels of oxidised mitochondria in BAL macrophages in CHP and IPF. Impaired mitochondria homeostasis is associated with chronic inflammation and age-associated diseases and may provide a better understanding of AMs activation in f-ILDs. 1. Tsitoura E, Vasarmidi E. et al.Resp.Res. 2019