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.

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.

Therapeutic decisions in a cohort of patients with idiopathic pulmonary fibrosis: a multicenter, prospective survey from Poland.

Background: Pirfenidone and nintedanib are considered as the standard of care in idiopathic pulmonary fibrosis (IPF), but there is no consensus as to which of these two agents should be regarded as first-line treatment. Objective: To provide real-world data on therapeutic decisions of pulmonary specialists, particularly the choice of the antifibrotic drug in patients with IPF. Methods: This was a multicenter, prospective survey collecting clinical data of patients with IPF considered as candidates for antifibrotic treatment between September 2019 and December 2020. Clinical characteristics and information on the therapeutic approach were retrieved. Statistical evaluation included multiple logistic regression analysis with stepwise model selection. Results: Data on 188 patients [74.5% male, median age 73 (interquartile range, 68-78) years] considered for antifibrotic therapy were collected. Treatment was initiated in 138 patients, while 50 patients did not receive an antifibrotic, mainly due to the lack of consent for treatment and IPF severity. Seventy-two patients received pirfenidone and 66 received nintedanib. Dosing protocol (p < 0.01) and patient preference (p = 0.049) were more frequently associated with the choice of nintedanib, while comorbidity profile (p = 0.0003) and concomitant medication use (p = 0.03) were more frequently associated with the choice of pirfenidone. Age (p = 0.002), lung transfer factor for carbon monoxide (TLCO) (p = 0.001), and gastrointestinal bleeding (p = 0.03) were significantly associated with the qualification for the antifibrotic treatment. Conclusion: This real-world prospective study showed that dose protocol and patient preference were more frequently associated with the choice of nintedanib, while the comorbidity profile and concomitant medication use were more frequently associated with the choice of pirfenidone. Age, TLCO, and history of gastrointestinal bleeding were significant factors influencing the decision to initiate antifibrotic therapy.

Risk factors of complications during noninvasive mechanical ventilation -assisted flexible bronchoscopy.

PURPOSE: Flexible bronchoscopy (FB) causes airway narrowing and may cause respiratory failure (RF). Noninvasive mechanical ventilation (NIV) is used to treat RF. Until recently, little was known about noninvasive mechanical ventilation assisted flexible bronchoscopy (NIV-FB) risk and complications. MATERIALS AND METHODS: A retrospective analysis of NIV-FB performed in 20 consecutive months (July 1, 2018-February 29, 2020) was performed. Indications for: FB and NIV, as well as impact of comorbidities, blood gas results, pulmonary function test results and sedation depth, were analyzed to reveal NIV-FB risk. Out of a total of 713 FBs, NIV-FB was performed in 50 patients with multiple comorbidities, acute or chronic RF, substantial tracheal narrowing, or after previously unsuccessful FB attempt. RESULTS: In three cases, reversible complications were observed. Additionally, due to the severity of underlining disease, two patients were transferred to the ICU where they passed away after >48h. In a single variable analysis, PaO2 69 â€‹± â€‹18.5 and 49 â€‹± â€‹9.0 [mmHg] (p â€‹< â€‹0.05) and white blood count (WBC) 10.0 â€‹± â€‹4.81 and 14.4 â€‹± â€‹3.10 (p â€‹< â€‹0.05) were found predictive for complications. Left heart disease indicated unfavorable NIV-FB outcome (p â€‹= â€‹0.046). CONCLUSIONS: NIV-FB is safe in severely ill patients, however procedure-related risk should be further defined and verified in prospective studies.

Validation of the Polish-language version of the COPD Assessment Test.

INTRODUCTION: The COPD Assessment Test (CAT) is a standardized patient-completed tool dedicated to assessment of symptom severity. While the Polish CAT version has been used for a few years, it has not been validated so far. OBJECTIVES: The aim of the study was to validate the Polish­language version of the CAT questionnaire by assessing its reproducibility and reliability. PATIENTS AND METHODS: Validation of the Polish­language version of the CAT questionnaire was a substudy of the international multicenter observational cross­sectional POPE survey of patients with chronic obstructive pulmonary disease (COPD) in Central and Eastern European countries. The study was completed in 395 outpatients with stable disease at least 4 weeks before the survey. Validation was performed with the use of the Spearman correlation and Cronbach α coefficients, Cohen κ test, and the Bland-Altman procedure. RESULTS: The internal consistency assessed by the Cronbach α coefficient was 0.87 for the questionnaire and 0.84 to 0.86 for its separate items. The repeatability of the questionnaire was good to very good (Cohen κ, 0.76-0.85; P <0.01). The Spearman coefficient for the sum of scores of test-retest responses was 0.95 (P <0.01). The Bland-Altman analysis revealed very good test-retest and interrater reliability, with the mean difference between test I and test II results of -0.556 (95% CI, -0.345 to 0.767). CONCLUSIONS: The Polish version of the CAT questionnaire is a reproducible and reliable instrument for evaluation of patients with COPD and should be recommended for use in clinical practice.

Vitamin D status of severe COPD patients with chronic respiratory failure.

INTRODUCTION: The aim of the study was to measure the concentrations of vitamin D in serum of COPD patients with chronic respiratory failure in comparison to healthy control group. The correlation between the levels of vitamin D in serum and the selected clinical, spirometric and blood gas parameters was the additional aim of the study. MATERIAL AND METHODS: The study included 61 patients with diagnosed COPD in stadium of chronic respiratory failure (45 men and 16 women) and 37 healthy controls (19 men and 18 women). The following procedure were performed in all studied subjects: detailed history (especially: daily activity, diet, tobacco and alcohol use), post-bronchodilator spirometry, assessment of 25(OH)D in serum and for COPD group only blood gas analysis. Recruitment for the study was conducted from November to April. Statistical analysis was performed using the following statistical methods: t-Student test, Mann-Whitney U test, Spearman correlation test and Chi-kwadrat test. RESULTS: There was no significant differences between COPD and control group for the levels of 25(OH)D in serum. Median and lower; upper quartile were respectively following: 24,75 nmol/l (16,9; 36,4) vs. 24,06 nmol/l (16,3; 37,2), p=0,69. Vitamin D deficiency was present in 60 COPD patients (98,3% of all patients) and in 36 control group subject (97,3% of all healthy volunteers). The difference was not statistically significant. The levels of vitamin D in serum did not significantly correlated with any of studied parameters (spirometry, blood gas, age, the level of activity, BMI, tobacco smoke exposure and others). However, the level of activity in COPD group correlated positively with spirometry values and negatively with age and number of exacerbations. CONCLUSION: The results of the study showed that in autumn-winter time in Poland there are very frequent deficiency of vitamin D in serum not only in COPD patients in respiratory failure stage but also in elderly healthy persons. However, in contrary to expectations the deficiency of vitamin D in COPD patients with respiratory failure were similar to that seen in healthy persons.

Distribution and characteristics of COPD phenotypes - results from the Polish sub-cohort of the POPE study.

Background: This study aimed to examine the distribution of predefined phenotypes, demographic data, clinical outcomes, and treatment of patients who were included in the Polish cohort of the Phenotypes of COPD in Central and Eastern Europe (POPE) study. Patients and methods: This was a sub-analysis of the data from the Polish cohort of the POPE study, an international, multicenter, observational cross-sectional survey of COPD patients in Central and Eastern European countries. The study included patients aged >40 years, with a confirmed diagnosis of COPD, and absence of exacerbation for at least 4 weeks before study inclusion. A total of seven Polish centers participated in the study. Results: Among the 430 Polish COPD patients enrolled in the study, 61.6% were non-exacerbators (NON-AE), 25.3% were frequent exacerbators with chronic bronchitis (AE CB), 7.9% were frequent exacerbators without chronic bronchitis (AE NON-CB), and 5.1% met the definition of asthma-COPD overlap syndrome (ACOS). There were statistically significant differences among these phenotypes in terms of symptom load, lung function, comorbidities, and treatment. Patients with the AE CB phenotype were most symptomatic with worse lung function, and more frequently reported anxiety and depression. Patients with the ACOS phenotype were significantly younger and were diagnosed with COPD earlier than those with other COPD phenotypes; those with the ACOS phenotype were also more often atopic and obese. Conclusion: There is significant heterogeneity among COPD patients in the Polish population in terms of phenotype and clinical outcome. The non-exacerbator phenotype is observed most frequently in Poland, while the frequent exacerbator with chronic bronchitis phenotype is the most symptomatic.

[Zygomatic localization of Langerhans cell histiocytosis - a case report].

Langerhans cell histocytosis is a rare disease. LCH is reported mainly in children aged less than 15 years, however it may appear at any age. In this work, the case of 17-month boy is reported. The boy was admitted to the pediatric ophthalmology department in order to diagnose the swelling and bruising in the lower part of left orbit, which had lasted for one month before. The clinical status, various imaging techniques' results (MRI, TK and PET) and immunohistochemistry led to the final diagnosis of multisystem LCH (spread to masticator muscle and zygomatic bone). After the pharmacological therapy the total remission was achieved. Since four years the child is regularly controlled by oncologists and hematologists with no relapse reported.

Pulmonary function tests in the preoperative evaluation of lung cancer surgery candidates. A review of guidelines.

Before planned surgical treatment of lung cancer, the patient's respiratory system function should be evaluated. According to the current guidelines, the assessment should start with measurements of FEV1 (forced expiratory volume in 1 second) and DLco (carbon monoxide lung diffusion capacity). Pneumonectomy is possible when FEV1 and DLco are > 80% of the predicted value (p.v.). If either of these parameters is < 80%, an exercise test with VO2 max (oxygen consumption during maximal exercise) measurement should be performed. When VO2 max is < 35 % p.v. or < 10 ml/kg/min, resection is associated with high risk. If VO2 max is in the range of 35-75% p.v. or 10-20 ml/kg/min, the postoperative values of FEV1 and DLco (ppoFEV1, ppoDLco) should be determined. The exercise test with VO2 max measurement may be replaced with other tests such as the shuttle walk test and the stair climbing test. The distance covered during the shuttle walk test should be > 400 m. Patients considered for lobectomy should be able to climb 3 flights of stairs (12 m) and for pneumonectomy 5 flights of stairs (22 m).

Evaluation of COPD progression based on spirometry and exercise capacity.

INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is characterized by an airflow limitation that is usually progressive. The progression of COPD expressed as the rate of an annual decline in FEV 1 is very heterogeneous. Exercise capacity in COPD patients is often diminished and becomes worsened over the time. The purpose of the study was to examine how the change in FEV 1 and exercise capacity would deteriorate over long-term observation. MATERIAL AND METHODS: A total of 22 men with COPD were examined. At the beginning the average age was 59 ± 8.1 years and the mean post-bronchodilator FEV 1 was 52 ± 14.9% predicted. Pulmonary function testing was performed at entry and then each year for 10 years, and exercise testing on a cycle ergometer was performed at entry and after 10 years. RESULTS: FEV 1 and maximum oxygen uptake (VO2max), maximum mechanical work (W max ), maximum minute ventilation (V Emax ) and maximum tidal volume (V Tmax ) declined significantly over the observation time. The mean annual decline in FEV 1 was 42 ± 37 mL, and the mean decline for VO 2max was 30 ± 15 mL/min/yr and 0.44 ± 0.25 mL/min/kg/yr. Regression analysis revealed that the changes in FEV 1 do not predict changes in VO2max. We observed a correlation between the annual change in V Emax and annual change in VO2max (r = 0.51 p < 0.05). The baseline FEV 1 (expressed as a percentage of predicted and in absolute values) is the predictor of FEV 1 annual decline (r = 0.74 and 0.82; p < 0.05). CONCLUSIONS: We observed over time deterioration in exercise capacity in COPD patients which is independent of decline in airflow limitation. The long term follow-up of exercise capacity is important in monitoring of COPD patients in addition to pulmonary function.

[Hemoptysis and dyspnea in a woman treated with acenocoumarol due to venous thrombosis]. / Krwioplucie i dusznosc u kobiety leczonej acenokumarolem z powodu zakrzepicy zylnej.

A case report of young woman diagnosed as having microscopic polyangiitis (MPO) presenting with diffuse alveolar hemorrhage (DAH). DAH is a rare, but life-threatening disorder. The patients presented with dyspnea, cough, hemoptysis (not constant). The radiographic features are very characteristic and reveal the signs of diffuse, bilateral alveolar filling in chest HRCT especially in middle and lower zones. Anaemia with iron deficiency and hypoxic respiratory failure. Elevation of diffuse capacity (above 30% of predicted) is often recognized as a result of presence of blood in the alveoli. Broncho-alveolar lavage reveal haemosiderin laden macrophages. It may occurs most frequently as a secondary condition due to microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), Goodpasture syndrome. Among the many conditions it can accompany connective tissue disorders, antiphospholipid antibody syndrome, some medicines or toxic exposures.

[Role of c-c chemokines in the determination of pleural effusion etiology]. / Ocena przydatnosci naczyniowo-sródblonkowego czynnika wzrostu w diagnostyce plynu oplucnowego.

UNLABELLED: The purpose of the study was to evaluate predictive value of vascular endothelial growth factor (VEGF) in the differential diagnosis of pleural effusion. MATERIAL AND METHODS: By Light's criteria in 29 cases exudates and 10 transudates was recognized. We investigated 39 patients with pleural effusion (congestive heart failure--10, parapneumonic--11, tuberculous--6, malignant--12, transudate--10). RESULTS: The mean VEGF value were significantly higher (p = 0.002) in the effusion of lung cancer patient (3958.33 pg/ml) than in the serum (1030.33 pg/ml). The mean VEGF levels were significantly higher (p = 0.004) in patient with exudates (2711.10 pg/ml) than in transudates (683 pg/ml). Based on the receiver operating characteristic (ROC) curve analysis, cut off value in differentiation of exudates and transudates was accepted at the level of 560 pg/ml (sensitivity 80% and specificity 70%). The mean VEGF levels were significantly higher (p = 0.008) in the malignant effusion (3958.33 pg/ml) than in the tuberculous effusion (1308.90 pg/ml). Based on the ROC curve analysis, cut off value in differentiation of malignant and tuberculous effusion was accepted at the level of 2000 pg/ml (sensitivity 92% and specificity 83%). The positive correlation between pleural VEGF concentrations and pleural lactate dehydrogenase (LDH) level were observed (r = 0.67, p < 0.05). CONCLUSION: We conclude that pleural effusion VEGF level could be useful in the differentiation between exudates and transudate and also between malignant and tuberculous exudates.

[Role of C-C chemokines in the determination of pleural effusion etiology]. / Znaczenie C-C chemokin w ustalaniu etiologii plynu w oplucnej.

INTRODUCTION: Pleural effusion secondary to various diseases is associated with the presence of different inflammatory cells. The C-C chemokines (MCP-1 and MIP-1alpha), produced by pleural mesothelial cells, plays an important role in the recruitment of inflammatory cells to the pleural space. The purpose of the study was to evaluate predictive value of MCP-1 and MIP-1alpha in the differential diagnosis of pleural effusion. MATERIAL AND METHODS: Based on Light's criteria in 29 cases exudates and in 10 transudates were recognized. We investigated 39 patients with pleural effusion (congestive heart failure - 10, parapneumonic - 11, tuberculous - 6, malignant - 12). The C-C chemokines MCP-1 and MIP-1alpha levels in pleural effusion and serum were measured by ELISA. RESULTS: The MCP-1 was significantly higher (p = 0.009) in the patient with exudates than in patients with transudates (2436 pg/ml and 794 pg/ml respectively). ROC curve analysis revealed however that this parameter has limited value in the differentiation of exudates an transudates (MCP-1 cut off value 1060 pg/ml, sensitivity 48%, specificity 90%, PPV 93%, NPV 37%). The chemokine MIP-1alpha were significantly higher (p = 0.001) in tuberculous than in the malignant effusion (405 pg/ml and 30 pg/ml respectively). Based on the ROC curve analysis, as a cut off value in the differentiation of tuberculous and malignant pleural effusion a value 120 pg/ml was accepted. The sensitivity of this test was 66% and specificity 99%, PPV 80%, NPV 84%. CONCLUSIONS: The chemokine MCP-1 has a limited value in the differentiation between transudate and exudates; MIP-1alpha could be helpful in the differentiation between tuberculous and malignant pleural effusion.

[Prediction of exercise capacity in chronic obstructive pulmonary disease patients on the basis of maximum expiratory flow-volume curve]. / Mozliwosci wnioskowania o zdolnosci wysilkowej chorych na przewlekla obturacyjna chorobe pluc na podstawie krzywej maksymalnego przeplywu i objetosci.

INTRODUCTION: Patients with chronic obstructive pulmonary disease (COPD) often present reduced exercise tolerance. Limitation of exercise capacity in COPD is an essential feature, which can be accurately determined by incremental cycle ergometry. The relationship between resting lung function variables and exercise capacity has been studied extensively in COPD patients. A clear relationship between resting pulmonary function testing and exercise capacity has not been established. The present study was designed to assess the accuracy of new index such as area under the maximal expiratory flow-volume curve [A(ex)] in predicting exercise capacity (expressed by maximum oxygen uptake - V'O(2max) and maximal mechanical work - W(max)) in COPD patients. MATERIAL AND METHODS: The study was performed on 41 patients with mild - to severe COPD (FEV1, 57.2 +/- 15.3% predicted). Each subject underwent resting spirometry (area under the maximal expiratory flow-volume curve - A(ex) was calculated). Subsequently, they performed maximal incremental cycle ergometer exercise for determination of V'O(2max), W(max). RESULTS: Significant correlation was found between A(ex) and V'O(2max) (r = 0.57, p < 0.001). In the group of severe COPD significant correlation was found between A(ex) and W(max) (r = 0.50 p < 0.05). CONCLUSIONS: In chronic obstructive pulmonary disease, the index - area under the maximal expiratory flow-volume curve - A(ex), could be useful in assesment of COPD severity and in predicting exercise capacity.