Introduction Fibrotic interstitial lung diseases (ILD) are a heterogeneous group of conditions. Progression criteria include clinical, imaging, and/or lung functional decline. Currently, the 6-min walk test (6MWT) is considered the gold standard for assessing exercise-induced desaturation. The 1-min sit-to-stand test (1STST) has the advantage of being easy to perform, making it feasible for use during clinical consultations. The aim of this study was to compare the 1STST with the 6MWT in patients with fibrotic ILD to detect exercise-induced desaturation. Methods A multicenter cross-sectional involved patients with fibrotic ILDs from two centers. The 6MWT and 1STST were performed with a 30-min rest period between the tests. The modified Borg scale was used to assess dyspnea and lower limb fatigue and continuous SpO2 and heart rate were monitored. Desaturation was defined as SpO2 < 90 % or decline >3 %. Results Ninety patients were included. The median walking distance in the 6MWT was 432 m and the median number of repetitions in the 1STST was 22, with a significant correlation (p < 0.001, ρ = 0.383). In addition, the minimum SpO2 and the desaturation levels recorded during the tests exhibited a moderate significant correlation (p < 0.001, ρ = 0.502 and p < 0.001, ρ = 0.537, respectively). There was a strong association between the increase in Borg score for dyspnea and a moderate association between the increase in Borg score for lower limb fatigue after both tests (p < 0.001, ρ = 0.706 and p < 0.001, ρ = 0.656, respectively). Conclusions This study demonstrates a significant correlation between the 6MWT and the 1STST in detecting exercise-induced desaturation.

Familial interstitial pneumonia (FIP) is defined as idiopathic interstitial lung disease (ILD) in two or more relatives. Genetic studies on familial ILD discovered variants in several genes or associations with genetic polymorphisms. The aim of this study was to describe the clinical features of patients with suspected FIP and to analyze the genetic variants detected through next-generation sequencing (NGS) genetic testing. A retrospective analysis was conducted in patients followed in an ILD outpatient clinic who had ILD and a family history of ILD in at least one first- or second-degree relative and who underwent NGS between 2017 and 2021. Only patients with at least one genetic variant were included. Genetic testing was performed on 20 patients; of these, 13 patients had a variant in at least one gene with a known association with familial ILD. Variants in genes implicated in telomere and surfactant homeostasis and MUC5B variants were detected. Most variants were classified with uncertain clinical significance. Probable usual interstitial pneumonia radiological and histological patterns were the most frequently identified. The most prevalent phenotype was idiopathic pulmonary fibrosis. Pulmonologists should be aware of familial forms of ILD and genetic diagnosis.

The PERSEIDS study aimed to estimate incidence/prevalence of interstitial lung diseases (ILDs), fibrosing interstitial lung diseases (F-ILDs), idiopathic pulmonary fibrosis (IPF), systemic sclerosis-associated ILD (SSc-ILD), other non-IPF F-ILDs and their progressive-fibrosing (PF) forms in six European countries, as current data are scarce. This retrospective, two-phase study used aggregate data (2014-2018). In Phase 1, incident/prevalent cases of ILDs above were identified from clinical databases through an algorithm based on codes/keywords, and incidence/prevalence was estimated. For non-IPF F-ILDs, the relative percentage of subtypes was also determined. In Phase 2, a subset of non-IPF F-ILD cases was manually reviewed to determine the percentage of PF behaviour and usual interstitial pneumonia-like (UIP-like) pattern. A weighted mean percentage of progression was calculated for each country and used to extrapolate incidence/prevalence of progressive-fibrosing ILDs (PF-ILDs). In 2018, incidence/105 person-years ranged between 9.4 and 83.6 (ILDs), 7.7 and 76.2 (F-ILDs), 0.4 and 10.3 (IPF), 6.6 and 71.7 (non-IPF F-ILDs), and 0.3 and 1.5 (SSc-ILD); and prevalence/105 persons ranged between 33.6 and 247.4 (ILDs), 26.7 and 236.8 (F-ILDs), 2.8 and 31.0 (IPF), 22.3 and 205.8 (non-IPF F-ILDs), and 1.4 and 10.1 (SSc-ILD). Among non-IPF F-ILDs, sarcoidosis was the most frequent subtype. PF behaviour and UIP-like pattern were present in a third of non-IPF F-ILD cases each and hypersensitivity pneumonitis showed the highest percentage of progressive behaviour. Incidence of PF-ILDs ranged between 2.1 and 14.5/105 person-years, and prevalence between 6.9 and 78.0/105 persons. To our knowledge, PERSEIDS is the first study assessing incidence, prevalence and rate of progression of ILDs across several European countries. Still below the threshold for orphan diseases, the estimates obtained were higher and more variable than reported in previous studies, but differences in study design/population must be considered.

Introduction: The natural course of idiopathic pulmonary fibrosis (IPF) changed since the introduction of antifibrotic therapy (AT). This therapy has been used in other progressive fibrotic interstitial lung diseases (PF-ILD) and its role has been evaluated in different clinical trials. Methods and Materials: Review of non-IPF patients with PF-ILD under AT followed at an ILD outpatient clinic that completed, at least, 6 months of treatment. Description of demographic characteristics, lung function evolution and pulmonary fibrosis extension analysis before and after 6-12 months of treatment. Results: Sixty patients with non-IPF PF-ILD started AT but only 27 met the inclusion criteria. A decline in FVC≥10% was the main reason for starting AT. T(-12M) - 12 months before antifibrotic therapy; T0 - beggining of AT; T(6-12) - 6-12 months after iniciating AT The predominant pattern on HRCT was usual interstitial pneumonia pattern (55,6%) and the extension of pulmonary fibrosis was higher at T(6-12M) compared to T0 (38.7 ±12.6% vs 34.63 ±14,1%; p-value<0.001). Conclusion: No significant difference was found on lung function decline and the extension of pulmonary fibrosis significantly worsened.

Endobronchial ultrasound-guided transbronchial fine-needle aspiration (EBUS-TBNA) is a new technique that facilitates cytologic sampling of mediastinal lymph nodes. We describe our initial experience with this method, including adequacy assessment, impact on cytopathologists' work, and diagnostic pitfalls. There were 229 EBUS-TBNA samples obtained from 100 patients; a mean of 22 minutes was spent with an average of 3 passes performed and 6 slides prepared per site. Of 193 aspirates, 5 were categorized as atypical, 54 as positive, and 134 as negative for malignancy; 36 (15.7%) aspirated were nondiagnostic. We found EBUS-TBNA to have a high specificity (100%) and good sensitivity (86%) in our institution, in which a cytopathologist is available on-site to ensure sample adequacy. Most true-negative samples had moderate to abundant lymphocytes, confirming lymphocyte numbers as a marker of adequacy. For pathologists, this was a relatively time-consuming procedure. Recognizing bronchial contamination, especially with metaplastic or dysplastic cells, is important for avoiding diagnostic pitfalls.

Poster: ECR 2021 / C-10782 / Smoking-related Interstitial Lung Diseases: (high)lighting fibrotic changes and their impact on management by: F. L. C. Marques dos Santos , I. Marques, C. Senra, A. Sanches, C. Nogueira, S. Campainha, S. Neves; Vila Nova de Gaia/PT

A 66-year-old man ingested sodium hydroxide in a suicide attempt in June 2018. In emergency room, he presented burning of the tongue and uvula and supraglottic and vocal cords edema. Because of the risk of airway obstruction, the patient underwent endotracheal intubation and started invasive mechanical ventilation. Five days later, a surgical tracheostomy was performed due to a large laryngeal necrosis including extensive epligottis involvement. After prolonged mechanical ventilation through tracheostomy, the patient presented a favorable clinical course allowing ventilator weaning and decannulation 2 months after initial presentation. In February 2019, cervical Computed Tomography (CT) revealed a polypoid lesion occupying 25% of the tracheal lumen, with contrast uptake and suggestive of malignant etiology (Figure 1A-B). The patient was asymptomatic. Rigid bronchoscopy showed that the pseudotumoral tracheal lesion corresponded to granulation tissue around suture thread from the previous tracheostomy, which were successfully removed during the procedure (Figure 1C-E). Pathological examination showed granulation tissue with extensive inflammatory infiltrate and foreign-body giant cell reaction.

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Introduction: We hypothesise that management of tracheal stenosis (TS) following intubation due to cardiogenic shock is particularly difficult. Methods: Retrospective study of patients with TS and history of intubation submitted to bronchoscopic procedures in a Bronchoscopy Unit, from 2014 to 2018. Patients were divided in 2 groups: I – intubation due to cardiogenic shock; II – intubation due to other causes. Clinical data was reviewed. Results: From a total of 55 patients with benign TS, 48 (87.3%) had history of intubation. Three were excluded due to missing cause of intubation. Out of 45 included patients (27 men, median age 58 years, 18 with smoking history), 13 (28.9%) matched group I and 32 (71.1%) group II. Patients’ characteristics and procedures are shown in Table 1. Group I was submitted to significantly more rigid bronchoscopies (6 vs 2, p 0.031) and mechanical dilations (5 vs 2, p 0.031) compared to group II. Discussion: Almost 30% of patients with TS were intubated due to cardiogenic shock. These patients required a higher number of endoscopic procedures, reflecting a more complex management. It would be important to identify risk factors and implement preventive strategies of tracheal injury in these at risk patients. Data: median (IQR), no. of patients (%) or no. of procedures

Introduction: Interstitial lung diseases (ILD) diagnosis requires a multidisciplinary workup, but sometimes it is impossible to achieve a confident diagnosis. Aim: To find predictive factors of mortality in patients with unclassifiable ILD (UILD). Methods: Retrospective analysis (Jan-2015 to Jan-2018) of patients diagnosed in our outpatient ILD clinic with UILD. Radiologic assessment was made by 2 independent radiologists and interobserver agreement was analysed. A cox regression analysis was performed. Results: From 379 patients, 37 (9.8%) were diagnosed with UILD. The most common reason of UILD was high surgical risk (21 patients, 55.3%). 14 patients (36.8%) died. The independent predictors of mortality were older age (HR 7.04 95% CI 1.05-47.31; p=0.045), lower FVC (HR 21.13, 95% CI 2.51-178.11; p=0.005) and LTOT (HR 11.24, 95% CI 1.89-66.81; p=0.008). Conclusions: UILD is a heterogeneous disease. Older age, lower FVC and LTOT seem to be independent predictors of mortality and interobserver agreement between radiologists ranged from good to very good.

Introducao: O diverticulo de Meckel e a anomalia gastrointestinal congenita mais comum e resulta da obliteracao incompleta do ducto vitelino. A hernia de Littre define-se pela presenca de um diverticulo de Meckel no saco herniario. O estrangulamento do diverticulo de Meckel numa hernia femoral e uma entidade extremamente rara que requer correcao cirurgica imediata. Caso Clinico: Reportamos o caso clinico de uma mulher de 18 anos que recorreu ao servico de urgencia por uma hernia femoral dolorosa e encarcerada a esquerda, sem sinais ou sintomas de obstrucao intestinal associados. Durante a intervencao cirurgica, aquando da abertura do saco herniario, foi identificado um diverticulo de Meckel, procedendo-se entao a diverticulectomia. A ansa de intestino delgado nao se encontrava herniada nem tinha sinais de isquemia, pelo que nao foi necessario realizar uma enterectomia. O defeito herniario foi subsequentemente corrigido atraves da tecnica de Lichtenstein modificada. Discussao: A sintomatologia de um diverticulo de Meckel complicado e inespecifica e dependente do tipo de complicacao. O diagnostico de hernia de Littre e exigente e dificil de estabelecer durante o pre-operatorio, uma vez que a sua apresentacao clinica e ambigua e a imagiologia tem um papel limitado. Quando a obstrucao do intestino delgado e provocada por um diverticulo de Meckel complicado, a intervencao cirurgica tem como objetivo principal remover o diverticulo e corrigir a patologia associada, independentemente da abordagem utilizada ser laparoscopica ou cirurgia aberta. Conclusao: No caso de uma hernia de Littre estrangulada o tratamento e baseado na condicao clinica do doente e na experiencia do cirurgiao. Nao obstante, o tratamento requer, pelo menos, uma resseccao completa do diverticulo para evitar complicacoes futuras.

Transbronchial lung cryobiopsy (TBLC) is a recent technique of obtaining lung tissue, however optimal protocol for this procedure is not well defined. To evaluate the diagnostic yield and complications of TBLC from one vs two lobes. Retrospective comparative analysis (Group 1: 1 lobe; Group 2: 2 lobes) of all TBLC performed for diagnosis of diffuse parenchymal lung diseases in our Hospital between May/2014 and January/2019. Number of procedures and demographics are shown in table. A specific pathological diagnosis was reached in 91% and 66% of patients from group 2 and 1, respectively (p=0.001). After multidisciplinary meeting, a definitive diagnosis ­­was possible in 88% of patients from group 2 vs 80% in group 1 (p=0.249). This trend was maintained even when only compared biopsies from two segments of the same lobe vs two lobes. The better pathological diagnosis and diagnostic yield was not associated with a significantly higher rate of complications. Our study showed that pathological diagnosis was superior in patients with biopsies from two lobes. However, this did not increase diagnostic yield. Studies with great number of patients are necessary.