Inhaled pirfenidone solution (AP01) for IPF: a randomised, open-label, dose-response trial.

INTRODUCTION: Oral pirfenidone reduces lung function decline and mortality in patients with idiopathic pulmonary fibrosis (IPF). Systemic exposure can have significant side effects, including nausea, rash, photosensitivity, weight loss and fatigue. Reduced doses may be suboptimal in slowing disease progression. METHODS: This phase 1b, randomised, open-label, dose-response trial at 25 sites in six countries (Australian New Zealand Clinical Trials Registry (ANZCTR) registration number ACTRN12618001838202) assessed safety, tolerability and efficacy of inhaled pirfenidone (AP01) in IPF. Patients diagnosed within 5 years, with forced vital capacity (FVC) 40%-90% predicted, and intolerant, unwilling or ineligible for oral pirfenidone or nintedanib were randomly assigned 1:1 to nebulised AP01 50 mg once per day or 100 mg two times per day for up to 72 weeks. RESULTS: We present results for week 24, the primary endpoint and week 48 for comparability with published trials of antifibrotics. Week 72 data will be reported as a separate analysis pooled with the ongoing open-label extension study. Ninety-one patients (50 mg once per day: n=46, 100 mg two times per day: n=45) were enrolled from May 2019 to April 2020. The most common treatment-related adverse events (frequency, % of patients) were all mild or moderate and included cough (14, 15.4%), rash (11, 12.1%), nausea (8, 8.8%), throat irritation (5, 5.5%), fatigue (4, 4.4%) and taste disorder, dizziness and dyspnoea (three each, 3.3%). Changes in FVC % predicted over 24 and 48 weeks, respectively, were -2.5 (95% CI -5.3 to 0.4, -88 mL) and -4.9 (-7.5 to -2.3,-188 mL) in the 50 mg once per day and 0.6 (-2.2 to 3.4, 10 mL) and -0.4 (-3.2 to 2.3, -34 mL) in the 100 mg two times per day group. DISCUSSION: Side effects commonly associated with oral pirfenidone in other clinical trials were less frequent with AP01. Mean FVC % predicted remained stable in the 100 mg two times per day group. Further study of AP01 is warranted. TRIAL REGISTRATION NUMBER: ACTRN12618001838202 Australian New Zealand Clinical Trials Registry.

Are There Differences in Inflammatory and Fibrotic Pathways between IPAF, CTD-ILDs, and IIPs? A Single-Center Pilot Study.

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-ß1), 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-ß1 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 (ß = 0.32; p = 0.037) and CXCL1 (ß = 0.45; p = 0.004) are significant predictors of BAL TCC. We revealed that IL-8 and TGF-ß1 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).

PULMONARY ALVEOLAR MICROLITHIASIS. DISCREPANCIES BETWEEN RADIOLOGICAL FINDINGS AND CLINICAL PATTERN- CASE STUDY.

Pulmonary alveolar microlithiasis is a rare genetic disorder, inherited autosomally recessively, which is characterized by intra-alveolar deposition of microliths built mostly of calcium salts and phosphorus. This case study describing management of patient with pulmonary alveolar microlithiasis. A 49-year-old woman, diagnosed with pulmonary microlithiasis in 1979 was admitted to Pneumology Department due to increased dyspnea. On admission there were no clinical signs of active infection. The chest computer tomography scan confirmed the presence of advanced microlithiasis. Pulmonary function test revealed mild restriction with moderate diffusion impairment, due to severe hypoxemia present on 6-minute walking test patient was sent for specific assessment to local lung transplant team in Zabrze for consideration for lung transplantation. According to International Society for Heart & Lung Transplantation guidelines the patient was observed in 6 months intervals to reveal whether further disease progression will be observed. Clinical condition of our patient does not correlate with radiological scans, severe respiratory symptoms and cardiological complications. Computer tomography scan should not be the only indication for lung transplant.

Noninvasive Ventilation-Facilitated Bronchofiberoscopy in Patients with Respiratory Failure.

Respiratory failure is one of the most important risk factors for diagnostic bronchofiberoscopy (BF), whereas therapeutic bronchoscopies are typically performed in intubated patients. Only a few published studies analyzed the outcomes of noninvasive mechanical ventilation (NIV)-facilitated BF. In this case series, we present our experiences with NIV-facilitated diagnostic and therapeutic BF performed in patients with respiratory failure that was associated with acute interstitial pulmonary disease, chronic obstructive pulmonary disease, cystic fibrosis exacerbation, foreign body aspiration, tracheal stenosis, pneumonia, and in a patient with a neuromuscular disease. All of the patients were initially hypoxic and some had PaO2/FiO2 < 200, which corresponded to moderate-to-acute respiratory distress syndrome (ARDS). NIV-facilitated BF were performed for the diagnostic or therapeutic purposes. The former consisted of bronchoalveolar lavage and bacterial sampling in a patient with impaired cough reflex, airway assessment in otherwise unexplained respiratory failure and hemoptysis, and the latter of mucous plugs resolution, foreign body removal, and assistance in weaning from mechanical ventilation. All procedures were carried out using NIV in the spontaneous timed (ST) or average volume assured pressure support (AVAPS) mode with oxygen supplementation. There were no procedure-related complications noticed during NIV-facilitated BF. We conclude that NIV is a useful and safe tool that facilitates the performance of BF in severe pulmonary diseases. Prospective studies are required to set the recommendations for the procedure and to define the optimum ventilatory modes to be used.

Chronic Obstructive Pulmonary Disease and Platelet Count.

Recently, it has been shown in the murine model that platelet maturation takes place, to some extent, in the lungs. The extrapolation of these findings to humans leads to the possibility that chronic lung diseases could affect platelet maturation and, consequently, the platelet count. The aim of this study was to investigate whether there are changes in the platelet count in patients with chronic obstructive disease (COPD). The study included 44 patients, aged 66.5 ± 5.5 years, in stage II-IV COPD. The control group consisted of 48 age- and gender-matched patients without any respiratory diseases. We failed to find a significant difference in the platelet count between the two groups: 231 ± 80 vs. 223 ± 63 x 103/µL, respectively (p = 0.61). However, the number of platelets in the COPD patients was inversely associated with hemoglobin content (r = -0.57; p < 0.001), hematocrit (r = -0.40; p = 0.006), and the red cell count (r = -0.51; p < 0.001); the blood morphology indices that are typically increased in severe COPD. Such associations were absent in the control non-COPD group. We conclude that COPD has no influence on the platelet count in humans.

Influence of noninvasive ventilation on clinical status, dyspnea sensation and subsequent copd diagnosis.

Chronic obstructive lung disease (COPD) is a common life-threatening disease characterized by exposure to tobacco smoke, dyspnea and persistent lower airway obstruction coexistence of COPD and chronic heart failure (HF) may present a considerable challenge during both diagnostic and therapeutic processes. Herein, we report an elderly, obese male, an ex-smoker, suffering from both COPD and HF, and treated according to the applied guidelines for 15 years. On admission to hospital, the patient was diagnosed and treated for severe type 2 respiratory failure. The patient's COPD diagnosis was questioned at first, but then reconsidered after treatment described below. Noninvasive ventilation (NIV) improved the patient's clinical condition and reduced his dyspnea sensation. As a consequence, during check-ups, spirometry maneuvers could have been performed properly, revealing the underlying bronchial obstruction, which had been beforehand concealed by debilitation of respiratory muscles and decreased lung tissue compliance in a patient with chronic HF. Conclusion: NIV application in a patient with type 2 respiratory failure may significantly improve one's clinical condition, reduce dyspnea sensation and help establish an accurate diagnosis.

Phenotypes of COPD patients with a smoking history in Central and Eastern Europe: the POPE Study.

Chronic obstructive pulmonary disease (COPD) represents a major health problem in Central and Eastern European (CEE) countries; however, there are no data regarding clinical phenotypes of these patients in this region.Participation in the Phenotypes of COPD in Central and Eastern Europe (POPE) study was offered to stable patients with COPD in a real-life setting. The primary aim of this study was to assess the prevalence of phenotypes according to predefined criteria. Secondary aims included analysis of differences in symptom load, comorbidities and pharmacological treatment.3362 patients with COPD were recruited in 10 CEE countries. 63% of the population were nonexacerbators, 20.4% frequent exacerbators with chronic bronchitis, 9.5% frequent exacerbators without chronic bronchitis and 6.9% were classified as asthma-COPD overlap. Differences in the distribution of phenotypes between countries were observed, with the highest heterogeneity observed in the nonexacerbator cohort and the lowest heterogeneity observed in the asthma-COPD cohort. There were statistically significant differences in symptom load, lung function, comorbidities and treatment between these phenotypes.The majority of patients with stable COPD in CEE are nonexacerbators; however, there are distinct differences in surrogates of disease severity and therapy between predefined COPD phenotypes.

Depressiveness, symptoms of anxiety and cognitive dysfunctions in patients with asthma and chronic obstructive pulmonary disease (COPD): possible associations with inflammation markers: a pilot study.

Psychiatric symptoms of anxiety, depression and cognitive dysfunction often occur in patients suffering from somatic conditions such as asthma and chronic obstructive pulmonary disease (COPD) which constitute a major and growing public health problem. In the present study we therefore aimed at analyzing depressive symptoms as well as symptoms of anxiety and cognitive problems in patients with mild to moderate asthma and COPD. 59 participants-17 with asthma, 24 with COPD and 18 healthy controls were enrolled. Depressiveness was assessed with the beck depression inventory (BDI); anxiety symptoms were measured with the State-Trait Anxiety Inventory Part 1 and 2, and cognitive function levels were estimated with the Trail Making Test Part A and B. A score above the threshold indicative for depression was found by 33 % (n = 8) of COPD patients, 29 % (n = 5) of asthma patients compared to 0.05 % (n = 1) of the control group. Clinically relevant anxiety levels were found in 42 % (n = 10) of the COPD group, 41 % (n = 7) of the asthma patients and 17 % (n = 3) of the controls. Patients with COPD performed significantly worse on the TMT than other groups. Psychoemotional state and cognitive functions were found to be correlated with exposure to tobacco smoke (measured in pack-years) and airway obstruction (measured with FEV1). In conclusion, patients with mild to moderate asthma and COPD exhibit significantly higher levels of depressive and anxiety symptoms as well as cognitive dysfunctions than controls. The prevalence of these symptoms is related to the amount of exposure to tobacco smoke and the severity of airflow obstruction.

Innate immunity but not NLRP3 inflammasome activation correlates with severity of stable COPD.

BACKGROUND: In models of COPD, environmental stressors induce innate immune responses, inflammasome activation and inflammation. However, the interaction between these responses and their role in driving pulmonary inflammation in stable COPD is unknown. OBJECTIVES: To investigate the activation of innate immunity and inflammasome pathways in the bronchial mucosa and bronchoalveolar lavage (BAL) of patients with stable COPD of different severity and control healthy smokers and non-smokers. METHODS: Innate immune mediators (interleukin (IL)-6, IL-7, IL-10, IL-27, IL-37, thymic stromal lymphopoietin (TSLP), interferon γ and their receptors, STAT1 and pSTAT1) and inflammasome components (NLRP3, NALP7, caspase 1, IL-1ß and its receptors, IL-18, IL-33, ST2) were measured in the bronchial mucosa using immunohistochemistry. IL-6, soluble IL-6R, sgp130, IL-7, IL-27, HMGB1, IL-33, IL-37 and soluble ST2 were measured in BAL using ELISA. RESULTS: In bronchial biopsies IL-27+ and pSTAT1+ cells are increased in patients with severe COPD compared with control healthy smokers. IL-7+ cells are increased in patients with COPD and control smokers compared with control non-smokers. In severe stable COPD IL-7R+, IL-27R+ and TSLPR+ cells are increased in comparison with both control groups. The NALP3 inflammasome is not activated in patients with stable COPD compared with control subjects. The inflammasome inhibitory molecules NALP7 and IL-37 are increased in patients with COPD compared with control smokers. IL-6 levels are increased in BAL from patients with stable COPD compared with control smokers with normal lung function whereas IL-1ß and IL-18 were similar across all groups. CONCLUSIONS: Increased expression of IL-27, IL-37 and NALP7 in the bronchial mucosa may be involved in progression of stable COPD.

Advances in Respiratory Medicine-Aims and Scopes Update.

Advances in Respiratory Medicine, which has been published by MDPI since 2022, serves as a platform for hosting pneumological studies [...].

Analysis of psychoemotional state and intellectual abilities in patients with asthma and chronic obstructive pulmonary disease--preliminary results.

BACKGROUND: In the literature we can find examples of comorbidity of the diseases of the respiratory tract and mental disorders. Among them a particularly significant position is occupied by chronic obstructive pulmonary disease (COPD) and asthma, which may be accompanied by anxiety, depressive and cognitive symptoms. The present research project was aimed to establish a connection between psycho-intellectual functioning and suffering from the aforementioned diseases. SUBJECTS AND METHODS: The patients were divided into 3 groups. In the asthma group there were 11 people, mean age 54, who met the GINA criteria for asthma. The group of patients with COPD was formed by 12 people, mean age 67. The control group included 13 people, mean age 48. Patients from all the groups underwent spirometry, sputum induction and the following tests: Mini-Mental State Examination (MSSE), Trail Making Test (TMT A and B), Beck Depression Inventory - BDI (Beck et al. 1961) and State-Trait Anxiety Inventory for Adults - STAI 1 and 2. RESULTS: In the TMT tests results were the following: We can presume some deficiency when the time required by a patient to complete the task is longer than 78 seconds for Part A and 273 seconds for Part B. In our research the best mean time was obtained in control group (Part A - 30.04 s, Part B - 67.37 s), then in the asthma group (Part A - 35.54 s, Part B - 98.81 s) and in the COPD group (Part A - 42.80 s, Part B - 107.79 s). In our research study the lowest score for the Beck Depression Inventory was obtained in the control group (mean 6.15), then in asthma (mean 9.63) and in COPD (mean 13.61). Results for State-Train Anxiety Inventory were distributed as follows: mean score in the asthma group was 36.48 in Part 1 and 43.27 in Part 2, in the COPD group 36.41 in Part 1 and 42.66 in Part 2 and in the control group 32.61 in Part 1 and 36.75 in Part 2. CONCLUSIONS: In our research the level of anxiety and depression measured by self-assessment questionnaires was higher in the study groups than in the control group. Also cognitive functions were worse than in the healthy controls, especially among COPD patients.

Talc slurry pleurodesis via chest tube in department of pulmonology--a 24-case study.

INTRODUCTION: Chemical pleurodesis is an accepted palliative therapy for patients with recurrent and symptomatic pleural effusion. The aim of the study is to present our own experiences with a less invasive variant of this procedure performed with talc slurry administered via a chest tube under local anaesthesia. Available medical literature in Polish does not contain information about this type of pleurodesis. MATERIAL AND METHODS: During 2005-2011 in the Pulmonology and Respiratory Rehabilitation Department we hospitalized and diagnosed 162 patients with pleural fluid. Pleurodesis was performed in 24 patients (14.8%) with persistent pleural fluid. In this article we present retrospective analysis of safety, efficacy of treatment and patients' survival time. We also provide detailed information about this type of pleurodesis: clinical theory, indications, contraindications, patient's preparation, description of procedure with our modifications and use of chest X-ray and transthoracic ultrasound. RESULTS: The procedure was effective in 20 cases, partially effective in 3 cases and ineffective in one case. In-hospital mortality was 4.2% (one case). We frequently observed mild fever and local pain. Median hospitalization was 9 days. Median survival time was 32 days, whereas in the group of still living patients it was 96 days. CONCLUSIONS: Talc slurry pleurodesis with adequate patient preselection is a relatively effective and safe procedure. The procedure can be performed in a non-surgical pulmonology unit.

Characteristics of Interstitial Pneumonia With Autoimmune Features (IPAF): Protocol for a Multicenter Prospective Study.

BACKGROUND: "Interstitial lung disease" (ILD) is a broad term encompassing diseases of different backgrounds. "Interstitial pneumonia with autoimmune features" (IPAF) is a recent term that implies the presence of autoimmunity. OBJECTIVE: This study aims to determine the characteristics of Polish patients with IPAF, compare them with patients with other interstitial pneumonias, and search for the prognostic and diagnostic biomarkers of IPAF in serum and bronchoalveolar lavage fluid (BALF). METHODS: This multicenter prospective study plans to recruit 240 participants divided into 1 study group and 2 control groups. Biological fluid samples will be collected according to Polish Respiratory Society management guidelines and stored at -80°C for further tests. Prospective 5-year observations of 60 newly diagnosed individuals are planned. The study will be divided into subsections. First, we plan to characterize Polish patients with IPAF (study group) against their peers with other ILDs (2 control groups). Control group 1 will comprise patients with idiopathic ILDs, including mainly idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Control group 2 will comprise patients with connective tissue disease-associated interstitial lung diseases, such as rheumatoid arthritis, systemic sclerosis, polymyositis, dermatomyositis, Sjögren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus. Radiological and functional parameters will be analyzed. Patients will be compared in terms of high-resolution computed tomography results, the 6-minute walking test performance, and pulmonary function test parameters. The diagnosis of IPAF will be reassessed on a regular basis through multidisciplinary discussion in order to determine its clinical stability. In the laboratory arm, inflammation and fibrosis pathways will be assessed. Cytokine levels (interleukin 8, transforming growth factor beta 1, chemokine C-C motif ligand [CXCL]18, CXCL1, surfactant protein [SP]-A, SP-D, Krebs von den Lungen-6 protein, and chitinase 1) will be measured in serum and BALF. A comparative analysis of serum and BALF cytokine levels will be performed in order to establish potential differences between systemic and local inflammatory pathways. In the quality of life (QoL) arm of the study, dyspnea and cough and their impact on various aspects of the QoL will be assessed. Depression and anxiety will be measured with the Hospital Anxiety and Depression Modified Scale and the 9-item Patient Health Questionnaire, and potential correlations with symptom prevalence will be assessed. RESULTS: This study will start recruiting patients to phase 1 in October 2023. The final results will be available in 2028. We plan to publish preliminary results after 2-3 years from the start of phase 1. CONCLUSIONS: This study will be a step toward a better understanding of IPAF etiopathogenesis and outcomes. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): PRR1-10.2196/44802.

The use of non-invasive respiratory assistance to facilitate bronchofiberoscopy performance in patients with hypoxemic (type one) respiratory failure - Study protocol.

PURPOSE: 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. MATERIALS AND METHODS: 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. RESULTS: The results of our study are clinically crucial, because they address current gaps concerning COT/HFNC/NIV/IMV. CONCLUSION: The expected findings of this study would allow for careful selection of respiratory support method to safely perform FOB in patients with hypoxemic RF.

May noninvasive mechanical ventilation and/ or continuous positive airway pressure increase the bronchoalveolar lavage salvage in patients with pulmonary diseases? Randomized clinical trial - Study protocol.

PURPOSE: Bronchoalveolar lavage (BAL) procedure is a useful tool in the diagnosis of patients with interstitial lung disease (ILD) and is helpful in clinical research of chronic obstructive pulmonary disease (COPD) patients. Still little is known about predictors of poor BAL salvage. The trial aims to find the most efficient way to improve BAL recovery. MATERIAL AND METHODS: Our study is a prospective, multicenter, international, two-arm randomized controlled trial. We aim to obtain BAL samples from a total number of 300 patients: 150 with ILD and 150 with COPD to achieve a statistical power of 80 â€‹%. Patients with initial BAL salvage <60 â€‹% will be randomized into the non-invasive ventilation (NIV) or continuous positive airway pressure (CPAP) arm. The NIV and CPAP will be set according to the study protocol. The influence on BAL salvage will be assessed in terms of BAL volume and content. Multivariable analysis of the additional test results to determine predictors for low BAL recovery will be conducted. In a study subgroup of approximately 20 patients per specific disease, a metabolomic assessment of exhaled air condensate will be performed. All procedures will be assessed in terms of the patient's safety. The trial was registered on clinicaltrials.gov (ID# NCT05631132). Interested experienced centers are invited to join the research group by writing to the corresponding author. CONCLUSION: The results of our prospective study will address the currently unsolved problem of how to increase BAL salvage in patients with pulmonary diseases without increasing the risk of respiratory failure exacerbation.

Efficacy of oral amantadine among patients hospitalised with COVID-19: A randomised, double-blind, placebo-controlled, multicentre study.

BACKGROUND: Amantadine has been proposed as a treatment for COVID-19 because it shows anti-SARS-CoV-2 activity in vitro. However, to date, no controlled study has assessed the safety and efficacy of amantadine in COVID-19. RESEARCH QUESTION: Whether amantadine is effective and safe among patients with different COVID-19 severity classifications. STUDY DESIGN: and Methods: This was multi-centre, randomised, placebo-controlled study.Patients with oxygen saturation ≤94% and no need for high-flow oxygen or ventilatory support were randomly allocated to receive oral amantadine or placebo (1:1) for 10 days in addition to standard care. The primary endpoint was time to recovery assessed over 28 days since randomisation, defined as discharge from hospital or no need for supplemental oxygen. RESULTS: The study was terminated early due to a lack of efficacy after an interim analysis. Final data from 95 patients who received amantadine (mean age, 60.2 years; 65% male; 66% with comorbidities) and 91 patients who received placebo (mean age, 55.8 years; 60% male; 68% with comorbidities) were obtained. The median (95% CI) time to recovery was 10 days both in the amantadine (9-11) and placebo arms (8-11; subhazard ratio = 0.94 [95%CI 0.7-1.3]). The percentage of deaths and percentage of patients who required intensive care at 14 and 28 days did not significantly differ between the amantadine and placebo groups. INTERPRETATION: Adding amantadine to standard care in patients hospitalised with COVID-19 did not increase the likelihood of recovery. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT04952519; www. CLINICALTRIALS: gov.

A Real-World Multicenter Retrospective Observational Study on Polish Experience with Nintedanib Therapy in Patients with Idiopathic Pulmonary Fibrosis: The PolExNIB Study.

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.

[Diagnostic and treatment of chronic obstructive pulmonary disease based on GOLD statement 2011]. / Diagnostyka i leczenie stabilnej postaci przewleklej obturacyjnej choroby pluc w swietle najnowszej aktualizacji GOLD 2011.

Chronic obstructive pulmonary disease (COPD) is one of the most important diseases because of high and constantly increasing prevalence, morbidity and mortality. An update of the Global strategy for the diagnosis, management, and prevention of COPD - GOLD report was published in the last days of 2011. In the paper the most important information concerning diagnosis, treatment and prevention of COPD based on GOLD statement 2011 were presented. The most interesting new information concerning diagnosis of COPD are following: post-bronchodilator ratio of FEV1/FVC < 0.7 still confirms the presence of airflow limitation in COPD; assessment of COPD symptoms is based on CAT and mMRC tests; acute bronchodilator reversibility test is no longer recommended for making COPD diagnosis; introduction of a new division of COPD patients into 4 groups (A, B, C or D) based on the level of airflow limitation, risk of exacerbations and severity of symptoms. The last change has fundamental impact on treatment guidelines, as in the past it was based only on spirometric classification, whereas currently it is guided by A-D groups selection.

Advances in Respiratory Medicine (ARM)-Past, Present, Future.

Advances in Respiratory Medicine (ARM) is the journal of the Polish Respiratory Society [...].