The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Asthma is a chronic inflammatory disorder of the airways with the proven role of Th2 cells in its pathogenesis. The role and characteristic of different subsets of CD4⁺ cells is much less known. The aim of the study was to analyze the incidence of different subsets of CD4⁺ T cells, in particular different subsets of CD4⁺ cells with the co-expression of different cytokines. Twenty five stable asthmatic and twelve age-matched control subjects were recruited to the study. Bronchoscopy and bronchoalveolar lavage (BAL) were performed in all study subjects. CD4⁺ T cells were isolated from BAL fluid by positive magnetic selection. After stimulation simultaneous expression of TGF-β, FoxP3, CD25, IFN-γ, IL-4, TNF-α (set 1); IL-10, FoxP3, CD25, IFN-γ, IL-4, MIP-1β (set 2); IL-17A, IL-8, IFN-γ, IL-4, MIP-1β (set 3) were measured by flow cytometry. The percentage of CD4⁺ cells co-expressing Foxp3 and TGF-β (CD4⁺Foxp3⁺TGF-β⁺ cells) was significantly lower (P = 0.03), whereas the percentage of CD4⁺IL-17⁺ cells (P = 0.008), CD4⁺IL-17⁺ IFN-γ⁺ cells (P = 0.047) and CD4⁺IL-4⁺ cells (P = 0.01) were significantly increased in asthmatics compared with that seen in healthy subjects. A significantly higher percentage of CD4⁺Foxp3⁺ cells from asthma patients expressed IFN-γ (P = 0.01), IL-4 (P = 0.004) and CD25 (P = 0.04), whereas the percentage of CD4⁺IL-10⁺ cells expressing Foxp3 was significantly decreased in asthmatics (P = 0.03). FEV₁% predicted correlated negatively with the percentage of CD4⁺IL-17⁺ cells (r = -0.33; P = 0.046) and positively with CD4⁺Foxp3⁺TGF-β⁺ cells (r = 0.43; P = 0.01). Our results suggest that in the airways of chronic asthma patients there is an imbalance between increased numbers of CD4⁺IL-17⁺ cells and Th2 cells and decreased number of CD4⁺Foxp3⁺TGF-β⁺.

In this pilot study, we aim to determine differences in pathogenetic pathways between interstitial pneumonia with autoimmune features (IPAF), connective-tissue-disease-associated interstitial lung diseases (CTD-ILDs), and idiopathic interstitial pneumonias (IIPs). Forty participants were recruited: 9 with IPAF, 15 with CTD-ILDs, and 16 with IIPs. Concentration of transforming growth factor beta (TGF-β1), surfactant proteins A and D (SP-A, SP-D), interleukin 8 (IL-8), and chemokine 1 (CXCL1) were assessed with ELISA assay in bronchoalveolar lavage (BAL) fluid. We revealed that IL-8 and TGF-β1 concentrations were significantly lower in the IPAF group than in the CTD-ILD group (p = 0.008 and p = 0.019, respectively), but similar to the concentrations in the IIP group. There were significant correlations of IL-8 (rs = 0.46; p = 0.003) and CXCL1 (rs = 0.52; p = 0.001) and BAL total cell count (TCC). A multivariate regression model revealed that IL-8 (β = 0.32; p = 0.037) and CXCL1 (β = 0.45; p = 0.004) are significant predictors of BAL TCC. We revealed that IL-8 and TGF-β1 BAL concentrations vary in patients with different ILDs and found that IL-8 is a predictor of BAL TCC in IPAF. However, this needs to be confirmed in a multicenter cooperative study (ClinicalTrials.gov Identifier: NCT03870828).

Bronchofiberoscopy (FOB) is a procedure routinely performed for: lung cancer, obstruction, interstitial diseases, foreign bodies' removal, airway clearance, and hemoptysis. It causes acute airway narrowing leading to respiratory and cardiovascular stress. Due to increasing number of ill patients with respiratory failure (RF), conventional oxygen therapy (COT) is frequently insufficient to assure accurate oxygenation and prevent RF in patients requiring FOB. In this clinical scenario, patients may be intubated and supported with invasive mechanical ventilation (IMV) with the specific aim of allowing a safe FOB. However, this invasive strategy is associated with an increased risk of IMV-associated complications. Our study is a planned prospective multicenter three-arm randomized controlled trial (RCT). The target number of 300 patients was calculated based on the intubation risk in RF patients, which is 0.2-2%. The patients will be assigned to each arm based on Horowitz index. In each arm, the patients will be randomly assigned to one out of two dedicated respiratory support methods in each group i.e. COT/high flow nasal cannula (HFNC), HFNC/non-invasive ventilation (NIV) and NIV/IMV. In the manuscript the current state of art in the area of respiratory support is discussed. We have underlined knowledge gaps in medical evidence which we are planning to reveal with our results. The results of our study are clinically crucial, because they address current gaps concerning COT/HFNC/NIV/IMV. The expected findings of this study would allow for careful selection of respiratory support method to safely perform FOB in patients with hypoxemic RF.

Nintedanib is a disease-modifying agent licensed for the treatment of IPF. Data on Polish experience with nintedanib in IPF are lacking. The present study aimed to describe the safety and efficacy profiles of nintedanib in a large real-world cohort of Polish patients with IPF. This was a multicenter, retrospective, observational study of IPF patients treated with nintedanib between March 2018 and October 2021. Data collection included baseline clinical characteristics, results of pulmonary function tests (PFTs), and a six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), and treatment persistence were also retrieved. A total of 501 patients (70% male) with a median age of 70.9 years (IQR 65–75.7) were included in this study. Patients were followed on treatment for a median of 15 months (7–25.5). The majority of patients (66.7%) were treated with the full recommended dose of nintedanib and 33.3% of patients were treated with a reduced dose of a drug. Intermittent dose reductions or drug interruptions were needed in 20% of patients. Over up to 3 years of follow-up, pulmonary function remained largely stable with the minority experiencing disease progression. The most frequent ADRs included diarrhea (45.3%), decreased appetite (29.9%), abdominal discomfort (29.5%), weight loss (32.1%), nausea (20.8%), fatigue (19.2%), increased liver aminotransferases (15.4%), and vomiting (8.2%). A total of 203 patients (40.5%) discontinued nintedanib treatment due to diverse reasons including ADRs (10.2%), death (11.6%), disease progression (4.6%), patient’s request (6.6%), and neoplastic disease (2.2%). This real-world study of a large cohort of Polish patients with IPF demonstrates that nintedanib therapy is safe, and is associated with acceptable tolerance and disease stabilization. These data support the findings of previously conducted clinical trials and observational studies on the safety and efficacy profiles of nintedanib in IPF.