Introduction Fibrotic hypersensitivity pneumonitis (fHP) is an immune-mediated interstitial lung disease caused by sensitization to chronic allergen inhalation. This study aimed to determine prognostic indicators of progression and mortality in fHP. Methods This was a retrospective, multicenter, observational, cross-sectional cohort study of consecutive patients diagnosed with fHP from 1 January 2012 to 31 December 2021. Multivariate Cox regression analyses were used to calculate hazard ratios (HRs) with 95% confidence interval (CI) for predictors of progression and survival. Results A total of 403 patients were diagnosed with fHP: median (IQR) age 66.5 (14.0) years, 51.9% were women and 55.1% were never smokers. The cause of fHP was mainly fungal (39.7%) or avian (41.4%). Lung biopsy was performed in 269 cases (66.7%). In the whole cohort the variables that were related to mortality-lung transplant were older age (HR=1.08, p<0.001), FVC(% pred) (HR=0.96, p=0.001), lymphocytosis in BAL (HR 0.93,p=0.001), presence of acute exacerbation during follow-up (HR=3.04,p=0.001) and GAP (Gender, Age, Physiology) index (HR=1.96,p<0.01). It stands out in multivariate Cox regression analyses in the group of biopsied patients the presence of fibroblastic foci at biopsy (HR=8.39,p<0.001) as highly significant predictor for increased mortality-lung transplant. Also, GAP, lymphocytosis in BAL and age are predictors of progression (HR=1.26,p=0.009; HR=0.97,p=0.018, and HR=1.03,p=0.018, respectively). Conclusion the study identified several prognostic factors for progression and/or survival in fHP. The presence of fibroblastic foci at biopsy was consistent predictor for increased mortality and the presence of lymphocytosis in BAL was inversely related to mortality.

A low-cost custom-made pseudo-anthropomorphic lung phantom, offering a model for ultrasound-guided interventions, is presented. The phantom is a rectangular solidstructure fabricated with polyvinyl alcohol cryogel (PVA-C) and cellulose to mimic the healthy parenchyma. The pathologies of interest were embedded as inclusions containing gaseous, liquid, or solid materials. The ribs were 3D-printed using polyethylene terephthalate, and the pleura was made of a bidimensional reticle based on PVA-C. The healthy and pathological tissues were mimicked to display acoustic and echoic properties similar to that of soft tissues. Theflexible fabrication process facilitated the modification of the physical and acoustic properties of the phantom. The phantom’s manufacture offers flexibility regarding the number, shape, location, and composition of the inclusions and the insertion of ribs and pleura. In-plane and out-of-plane needle insertions, fine needle aspiration, and core needle biopsy were performed under ultrasound image guidance. The mimicked tissues displayed a resistance and recoil effect typically encountered in a real scenario for a pneumothorax, abscesses, and neoplasms. The presented phantom accurately replicated thoracic tissues (lung, ribs, and pleura) and associated pathologies providing a useful tool for training ultrasound-guided procedures.

Introduction and Aim: Pirfenidone and nintedanib are anti-fibrotic approved treatments for idiopathic pulmonary fibrosis (IPF). Its efficacy and safety in other fibrotic interstitial lung diseases (ILD) is under study in clinical trials. The aim was to analyse the effect of these drugs in non-IPF progressive fibrotic patients that couldn’t be included in these trials. Methods: Multicenter retrospective study of 34 progressive PF non-IPF patients treated with anti-fibrotic drugs. Demographic, clinical data and treatment-related variables were evaluated. The reason to start off-label anti-fibrotic was disease progression with previous therapy and exclusion from clinical trials. Results: Patients with non-IPF progressive PF were: 38% unclassifiable PF (UPF), 20% familial pulmonary fibrosis (FPF), 18% CTD-ILD, 15% chronic hypersensitivity pneumonitis (CHP) and 9% pneumoconiosis. Mean age was 67±12 years, 74% were male and 47% ex-smokers. 21% of cases had usual interstitial pneumonia HRCT pattern. Mean FVC was 66%±23 and DLCO 45%±12. Co-medications were: low-dose prednisone (20), mycophenolate (5) and sirolimus (1). Twenty-four subjects (71%) received pirfenidone and ten (29%) nintedanib. After one year, a FVC decrease of 5-10% was observed in only 3 patients (1 CHP, 1 pneumoconiosis, 1 UPF), while others showed stability or improvement. Main reported adverse effects were: 20% hypertransaminasemia, 27% diarrhea and 15% weight loss, requiring 5 patients to stop therapy. Conclusion: After one year of anti-fibrotic therapy, most patients with non-IPF progressive PF showed functional stability. Clinical trials are mandatory to evaluate their potential efficacy, but these drugs are a safe option in progressive PF patients.

Introduction: Connective tissue disease-related interstitial lung disease (CTD-ILD) are a collection of systemic autoimmune disorders associated with lung abnormalities. Understanding CTD-ILD onset and progression is essential to improve patient’s survival by tailoring personalize treatments avoiding adverse events. The aim of this work was to analyse the progression of these patients treated with rituximab (RTX). Methods: Retrospective study of a cohort of 29 CTD-ILD patients treated with RTX for clinical-functional impairment (2015-2022). Comorbidities, radiological pattern, functional evolution and treatments were analysed. Results: Mean age was 62±11 years, 66% were women. One third had ≥3 points in Charlson index. Most prevalent CTD-ILD were: 38% rheumatoid arthritis,15% interstitial pneumonia with autoimmune features and 15% Overlap Sjögren9s syndrome-Scleroderma. The 31% at diagnosis had a radiological pattern of UIP. 97% received steroid and 18% antifibrotic drugs; among the immunosuppressants used prior to RTX, the most used were methotrexate (45%) and mycophenolate (38%). Mean number of RTX doses was 3.31±2.11 and only 38%(11) had adverse effects, most of which were mild, with only 1 exacerbation and 5 admissions for infections. At 12 months, there was an improvement in mean FVC (+121 ml/4%), stabilisation of DLCO and better exercise tolerance (+44 metres in WT6M). Currently, 11 patients have home oxygen, 1 is on waiting list for lung transplant and 10 have died. Conclusion: RTX treatment in patients with CTD-ILD contributes to functional stabilisation with mild adverse effects, favouring improvement in quality of life and survival.

Background The COVID-19 pandemic is one of the greatest public health problems. Our aims were to describe epidemiological characteristics, know the amount of protective antibodies and their permanence after a COVID-19 primary infection in patients with risk of pulmonary fibrosis. Methods Descriptive epidemiological and follow-up study of the humoral response in patients at risk of pulmonary fibrosis Post-Covid-19 hospitalized, between March and October 2020, and who were followed up for a one year after hospital discharge. Results 72 patients participated in the study, 52 showed pre-existing chronic comorbidities. COVID-19 clinical severity was rated in 6% mild, 58% as moderate and 36% as severe. After a year of follow-up, the forty percent had pulmonary sequelae, the most frequent (20%) was mild pulmonary fibrosis. Any case of reinfection was detected. All patients presented RBD IgG antibodies and 88% presented IgA antibodies after 8-9 months. The amount of RBD IgG was similar at 4-5 and 8-9 months post-Covid. There was no difference when level of RBD IgG according to the severity of the COVID-19 (p=0.441, p=0.594). Conclusions Mild pulmonary fibrosis sequelae is exceptional but was detected in a high percentage. The amount of RBD IgG is maintained throughout the convalescent phase and seems to protect against new reinfections despite of emerging viral variants. However, seems not predict the developed or not of pulmonary fibrosis.

Introduction and Aim: Ultrasound (US) provides valuable information in pathologies related to the lung parenchyma that are in direct contact with the pleura.The aim of this work was to design custom-made low-cost phantoms, whose characteristics mimic the lung parenchyma, to aid professionals in thoracic US imaging. Such phantoms would allow them to gain diagnostic and therapeutic capabilities in a clinical setting, in which infectious and neoplastic etiology diseases present a high prevalence. Methods: Water-based phantoms were fabricated using a 10% concentration of Polyvinyl Alcohol Cryogel (PVA-C) as gelling agent. Cellulose (20 mm) was employed as acoustic scatter at 1% concentration.In order to mimic the pathologies of interest, such as neoplasia or pleural effusion, different spherical inclusions, filled with solid or liquid, were placed at variable depths. Results: US characterization of each fabricated phantom was performed using a standard clinical US system (Z6 Mindray).Acquired images confirmed the similarity of the fabricated phantoms with the normal lung parenchyma.Furthermore, the measured speed of sound was within the limits of soft tissues reported in the literature. In addition, ultrasound images of the inclusions were validated. Interobserver variability did not affect the characteristics of the phantoms. Conclusion: The availability of these phantoms simulating the lung parenchyma provides tools to gain capabilities in the use of US imaging. Therefore, earlier and improved diagnosis of several pathologies could be achieved as well as faster and more precise interventions. Thus, the number of invasive surgical procedures and their associated costs would be reduced.