Guadalupe Bermudo Peloche

Interstitial lung diseases (ILDs) are associated with late diagnosis and a poor prognosis. Early diagnosis could be crucial. Aims: evaluate the feasibility and benefits of a rapid diagnostic circuit for ILD. Methods: A training program for ILD identification and a direct referral diagnostic circuit to the ILD Unit of University Hospital of Bellvitge were established in primary care centers. ILD patients were diagnosed and followed-up until study completion, death or lung transplantation. Diagnostic, therapeutic and prognostic outcomes were compared to patients referred through the common circuit during the same period of time. Results: Of 123 patients referred directly from the primary care, 112 had ILD. The most common diagnosis were IPF and fibrotic HP. The main reasons for suspecting ILD were radiological features (74%) and abnormal lung auscultation (67%). Compared with patients referred through the common circuit, there was a statistically significant reduction in the time from symptom onset to diagnosis (6 vs. 22.1 months, p<0.01) and in the percentage of fibrosing ILD (55.9 vs 63.9%, p 0.36). Although patients from rapid circuit were older, they had better FVC and DLCO at diagnosis (p 0.04) and lung biopsies were performed more frequently. More patients were elegible for lung transplant. Conclusions: Identifying potential patients with fibrotic ILD through rapid circuit working with primary care physicians is feasible and useful. erj;68/suppl_70/PA3046/F1 F1 F1

Background and Objectives: Nintedanib is indicated for progressive pulmonary fibrosis (PPF) based on clinical trial results. The primary objectives of this study were to evaluate the effectiveness of nintedanib on forced vital capacity (FVC) and diffusing lung capacity for CO (DLCO) after one year of treatment, and to compare the annual rate of decline (“slope”) for FVC, DLCO and 6 minute walking distance (6MWD) with the year prior to treatment. The secondary objectives were antifibrotic safety, tolerability, adverse events, immunosuppressant use, dyspnoea and survival. Materials and Methods: This study was a single-centre, retrospective, observational cohort study that included consecutive patients with PPF treated with nintedanib. Results: Fifty-five patients with non-IPF fibrotic ILD initiated nintedanib due to fibrosis progression. Most patients (63.4%) stabilised/improved FVC after 1 year of treatment, and 82.5% stabilised/improved DLCO. The “slope” of FVC and DLCO was reduced after 1 year of treatment compared to the year before initiation, although the difference was not statistically significant: FVC slope was +0.61% in the year after initiation vs. −2.3% in the year prior (mean change: 2.94%, 95%CI [−4.74, 10.62]); DLCO slope was −3.8% after treatment vs. −7% before initiation (mean change: 3.24%, 95%CI [−7.43, 13.92]). Dyspnoea improved in 23.2% of patients. A reduction in immunosuppressant use was observed after nintedanib initiation. Forty-one patients (74.5%) experienced at least one side effect: diarrhoea (60%), hepatotoxicity (23.6%), or asthenia (12.7%). Fifteen patients required permanent or temporary treatment discontinuation. Conclusions: In our real-world PPF cohort, most patients showed FVC and/or DLCO stabilisation or improvement after one year of nintedanib treatment. A non-significant reduction in the rate of FVC decline after one year of treatment was also observed, as was a reduction in symptom severity in our real-life PPF cohort.

Chronic interstitial lung changes are potential long-term sequelae of severe COVID-19. This study aims to evaluate the prevalence and type of interstitial changes over time and identify serum biomarkers associated with persistent lung abnormalities. Prospective, multicenter, observational cohort study of patients with severe COVID-19 pneumonia between March 2020 and June 2021. Clinical evaluation, pulmonary function tests (PFTs), and chest high-resolution computed tomography (HRCT) were performed at baseline and up to 24 months. Serum biomarkers were assessed at baseline and at 12 months. Of the 290 patients enrolled, 247 completed the follow-up. Mild dyspnea (mMRC1) was the most common residual symptom. PFTs showed significant improvement: mean FVC increased from 88.8% to 99.5%, and DLCO from 71.6% to 82%. Among patients with baseline DLCO < 80% (n = 67), only 38.8% achieved normalization. Radiological improvement was observed in all patients, though 32% of the 118 patients assessed at 24 months still had residual changes, primarily mild reticulation, ground glass opacities, and lower lobes traction bronchiectasis. Pulmonary functional improvement correlated with radiological resolution, especially during the first year. Elevated baseline levels of MMP-7 and KL6 were associated with persistent interstitial abnormalities at 12 months. Severe COVID-19 survivors exhibit progressive functional and radiological improvement, particularly within the first year. However, a subset of patients shows long-term interstitial changes. Elevated MMP-7 and KL-6 levels may help identify individuals at higher risk for persistent interstitial changes.

Interstitial lung diseases (ILDs) are complex rare diseases that associate a delay in diagnosis and eventually poor prognosis. Early and accurate diagnosis could be crucial. This study aimed to evaluate the feasibility and benefits of a rapid diagnostic circuit for ILD. A training program for ILD identification and a direct referral diagnostic circuit to the ILD Unit of University Hospital of Bellvitge were established in primary care centers in the southern metropolitan area of Barcelona (Spain). ILD patients were diagnosed and followed-up until study completion, death or lung transplantation. Diagnostic, therapeutic and prognostic outcomes were compared to patients referred through the common circuit during the same period of time. Of 123 patients referred directly from the primary care, 112 had ILD. The most common diagnosis were idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis. The main reasons for suspecting ILD were interstitial radiological features (74%) and abnormal lung auscultation (67%). Eighteen patients were asymptomatic. Compared with patients referred through the common circuit, there was a statistically significant reduction in the time from symptom onset to diagnosis (6 vs. 22.1 months, p < 0.01) and in the percentage of fibrosing ILD (55.9 vs 63.9%, p 0.36). Although patients from rapid circuit were older, they had better forced vital capacity and diffusing capacity for carbon monoxide at diagnosis (p 0.04) and lung biopsies were performed more frequently. More patients were elegible for lung transplant. Identifying potential patients with fibrotic ILD through rapid circuit working with primary care physicians is feasible and useful.

Early and accurate diagnosis of idiopathic pulmonary fibrosis and other progressive fibrotic ILDs is relevant for prognosis and therapeutic approach. The present study aims to evaluate the feasibility and usefulness of a rapid ILD-circuit involving primary care centres and ILD Unit of a tertiary center. Methods: training program for clinical suspicion and identification of ILD patients was performed in the primary care centers of the area and online interaction was maintained. ILD patients diagnosed through this circuit were recruited and followed-up for at least 3 years. The results were compared to patients referred through the common diagnostic circuit during the same period. Results: A total of 706 new referred patients were included; 103 patients were directly referred from the primary care to the ILD Unit and 603 patients arrived through the common ILD circuit. The main differences are as follows (table 1) there was statistically significant reduction in the time from onset of symptoms to diagnosis and in the number of complementary tests performed compared to patients in the common circuit (p=0.001 and p=0.04 respectively). Although the higher proportion of fibrotic ILD patients thought the normal diagnostic circuit, the percentage of patients receiving anti-fibrotic treatment was higher in the rapid diagnostic group. Patients diagnosed using the rapid circuit arrived more frequently to lung transplantation and showed a better 7-year survival rate. Conclusions: Identification of potential fibrotic ILD patients by using a rapid circuit working with primary care physicians is feasible and useful. Furthermore, the rapid circuit circuit reduces the ILD patient journey and the number of complementary tests required.

The treatment of post-covid pulmonary fibrotic abnormalities remain a challenge in severe COVID19 survivors. Aims and Objectives: To evaluate the efficacy and safety profile of pirfenidone in subjects who present clinical-radiological signs of pulmonary fibrosis after severe SARS-COV2 pneumonia. Methods: Randomized multicenter double-blind phase-II placebo-controlled clinical trial that included patients with post-covid19 pulmonary fibrotic-like changes after at least one-month of hospital discharge. Patients received treatment for 6 months. The main endpoint was the rate of patients that improved (%FVC-change and high-resolution computed tomography (HRCT) fibrotic score). Results: 113 patienets were randomized. Most were male (74%), mean age 63.7 years and 50% had required tracheal intubation and mechanical ventilation during hospital stay. Mean HRCT-score was 20%(+/-14.8), mean FVC% 78(+/-19) and DLCO% 58.5(+/-19) at randomization, 49% required oxygen at home and 95% were receiving oral glucocorticoids. Although no statistically significant differences were found in the rate of patients that improved between groups, the mean percentage of FVC improvement was +12.74%(10.6) with pirfenidone and +4.35%(22) with placebo (p=0.06). Fibrotic-score reduction was -5.3% with pirfenidone and -2.5% with placebo. Clinically significant K-BILD score improvement was observed in 55% of pirfenidone patients and 39% of placebo (p=0.19). The presence of adverse events was not statistically different. Conclusions: Most patients with fibrotic-like changes after severe covid19 improve or stabilize in both arms, but the grade of improvement with pirfenidone over 24 weeks tends to be higher.

Introduction: The pandemic COVID-19 viral infection is a global threat with a high mortality, mainly due to acute respiratory distress syndrome and respiratory failure (ARDS). Some of these patients develop radiological pulmonary fibrotic signs after just few weeks of hospitalization. Objectives: To evaluate those ARDS-COVID19 patients at intensive care unit (ICU) that present low-compliance, ventilatory difficulties and fibrotic signs at the chest HRCT, including safety profile of anti-fibrotic use. Methods: observational retrospective study of hospitalized patients that required intensive care and were registered as ARDS-COVID19 complicated with pulmonary fibrotic radiological and physiological signs from March to July 2020. Results: From a total of388patients that required ventilatory support since ARDS-COVID19, 28 were included. The mean age was 56.32 ± 11.23 years. All patients presented severe respiratory failure with a median SaFi (Sa02/Fi02) 282.9% (IQR 284.47%). 27 patients required intubation and mechanical ventilation. Fibrotic signs on chest HRCT were identified at 39.6 days (SD 19.72) from admission; and all received corticosteroid treatment. 11 patients received pirfenidone. Mortality rate was 21.43% and was similar between groups, although patients that were treated with pirfenidone showed lower SaFi at hospitalization, more days of mechanical ventilation and tracheostomy. No severe adverse events associated to pirfenidone medication were observed. Conclusions: Pulmonary fibrotic changes after ARDS-Covid-19 at ICU present a high in-hospital mortality. Anti-fibrotic medication present a safety profile in these patients.

Background and objective: The relationship between IPF development and environmental factors has not been completely elucidated. Analysing geographic regions of idiopathic pulmonary fibrosis (IPF) cases could help identify those areas with higher aggregation and investigate potential triggers. We hypothesize that cross‐analysing location of IPF cases and areas of consistently high air pollution concentration could lead to recognition of environmental risk factors for IPF development.Methods: This retrospective study analysed epidemiological and clinical data from 503 patients registered in the Observatory IPF.cat from January 2017 to June 2019. Incident and prevalent IPF cases from the Catalan region of Spain were graphed based on their postal address. We generated maps of the most relevant air pollutant PM2.5 from the last 10 years using data from the CALIOPE air quality forecast system and observational data.Results: In 2018, the prevalence of IPF differed across provinces; from 8.1 cases per 100 000 habitants in Barcelona to 2.0 cases per 100 000 in Girona. The ratio of IPF was higher in some areas. Mapping PM2.5 levels illustrated that certain areas with more industry, traffic and shipping maintained markedly higher PM2.5 concentrations. Most of these locations correlated with higher aggregation of IPF cases. Compared with other risk factors, PM2.5 exposure was the most frequent.Conclusion: In this retrospective study, prevalence of IPF is higher in areas of elevated PM2.5 concentration. Prospective studies with targeted pollution mapping need to be done in specific geographies to compile a broader profile of environmental factors involved in the development of pulmonary fibrosis.