Three Weeks of Pulmonary Rehabilitation Do Not Influence Oscillometry Parameters in Postoperative Lung Cancer Patients.

Background: Thoracic surgery is a recommended treatment option for non-small cell lung cancer patients. An important part of a patient's therapy, which helps to prevent postoperative complications and improve quality of life, is pulmonary rehabilitation (PR). The aim of this study was to assess whether the implementation of physical activity has an influence on forced oscillation technique (FOT) values in patients after thoracic surgery due to lung cancer. Methods: In this observational study, we enrolled 54 patients after thoracic surgery due to lung cancer, 49 patients with idiopathic interstitial fibrosis (IPF), and 54 patients with chronic obstructive pulmonary disease/asthma−COPD overlap (COPD/ACO). All patients were subjected to three weeks of in-hospital PR and assessed at the baseline as well as after completing PR by FOT, spirometry, grip strength measurement, and the 6-min walk test (6MWT). Results: We observed differences between FOT values under the influence of physical activity in studied groups, mostly between patients after thoracic surgery and COPD/ACO patients; however, no significant improvement after completing PR among FOT parameters was noticed in any group of patients. Improvements in the 6MWT distance, left hand strength, and right hand strength after PR were noticed (p < 0.001, 0.002, and 0.012, respectively). Conclusions: Three weeks of pulmonary rehabilitation had no impact on FOT values in patients after thoracic surgery due to lung cancer. Instead, we observed improvements in the 6MWT distance and the strength of both hands. Similarly, no FOT changes were observed in IPF and COPD/ACO patients after completing PR.

Prospective Evaluation of the NETest as a Liquid Biopsy for Gastroenteropancreatic and Bronchopulmonary Neuroendocrine Tumors: An ENETS Center of Excellence Experience.

BACKGROUND: There is a substantial unmet clinical need for an accurate and effective blood biomarker for neuroendocrine neoplasms (NEN). We therefore evaluated, under real-world conditions in an ENETS Center of Excellence (CoE), the clinical utility of the NETest as a liquid biopsy and compared its utility with chromogranin A (CgA) measurement. METHODS: The cohorts were: gastroenteropancreatic NEN (GEP-NEN; n = 253), bronchopulmonary NEN (BPNEN; n = 64), thymic NEN (n = 1), colon cancer (n = 37), non-small-cell lung cancer (NSCLC; n = 63), benign lung disease (n = 59), and controls (n = 86). In the GEPNEN group, 164 (65%) had image-positive disease (IPD, n = 135) or were image-negative but resection-margin/biopsy-positive (n = 29), and were graded as G1 (n = 106), G2 (n = 49), G3 (n = 7), or no data (n = 2). The remainder (n = 71) had no evidence of disease (NED). In the BPNEN group, 43/64 (67%) had IPD. Histology revealed typical carcinoids (TC, n = 14), atypical carcinoids (AC, n = 14), small-cell lung cancer (SCLC, n = 11), and large-cell neuroendocrine carcinoma (LCNEC, n = 4). Disease status (stable or progressive) was evaluated according to RECIST v1.1. Blood sampling involved NETest (n = 563) and NETest/CgA analysis matched samples (n = 178). NETest was performed by PCR (on a scale of 0-100), with a score ≥20 reflecting a disease-positive status and >40 reflecting progressive disease. CgA positivity was determined by ELISA. Samples were deidentified and measurements blinded. The Kruskal-Wallis, Mann-Whitney U, and McNemar tests, and the area under the curve (AUC) of the receiver-operating characteristics (ROC) were used in the statistical analysis. RESULTS: In the GEPNEN group, NETest was significantly higher (34.4 ± 1.8, p < 0.0001) in disease-positive patients than in patients with NED (10.5 ± 1, p < 0.0001), colon cancer patients (18 ± 4, p < 0.0004), and controls (7 ± 0.5, p < 0.0001). Sensitivity for detecting disease compared to controls was 89% and specificity was 94%. NETest levels were increased in G2 vs. G1 (39 ± 3 vs. 32 ± 2, p = 0.02) and correlated with stage (localized: 26 ± 2 vs. regional/distant: 40 ± 3, p = 0.0002) and progression (55 ± 5 vs. 34 ± 2 in stable disease, p = 0.0005). In the BPNEN group, diagnostic sensitivity was 100% and levels were significantly higher in patients with bronchopulmonary carcinoids (BPC; 30 ± 1.3) who had IPD than in controls (7 ± 0.5, p < 0.0001), patients with NED (24.1 ± 1.3, p < 0.005), and NSCLC patients (17 ± 3, p = 0.0001). NETest levels were higher in patients with poorly differentiated BPNEN (LCNEC + SCLC; 59 ± 7) than in those with BPC (30 ± 1.3, p = 0.0005) or progressive disease (57.8 ± 7), compared to those with stable disease (29.4 ± 1, p < 0.0001). The AUC for differentiating disease from controls was 0.87 in the GEPNEN group and 0.99 in BPC patients (p < 0.0001). Matched CgA analysis was performed in 178 patients. In the GEPNEN group (n = 135), NETest was significantly more accurate for detecting disease (99%) than CgA positivity (53%; McNemar test χ2 = 87, p < 0.0001). In the BPNEN group (n = 43), NETest was significantly more accurate for disease detection (100%) than CgA positivity (26%; McNemar's test χ2 = 30, p < 0.0001). CONCLUSIONS: The NETest is an accurate diagnostic for GEPNEN and BPNEN. It exhibits tumor biology correlation with grading, staging, and progression. CgA as a biomarker is significantly less accurate than NETest. The NETest has substantial clinical utility that can facilitate patient management.

A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study.

BACKGROUND: Pirfenidone is an antifibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF). The drug is available for Polish patients with IPF since 2017. The PolExPIR study aimed to describe the real-world data (RWD) on the Polish experience of pirfenidone therapy in IPF with respect to safety and efficacy profiles. METHODS: This was a multicentre, retrospective, observational study collecting clinical data of patients with IPF receiving pirfenidone from January 2017 to September 2019 across 10 specialized pulmonary centres in Poland. Data collection included baseline characteristics, pulmonary function tests (PFTs) results and six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), treatment persistence, and survival were also collected up to 24 months post-inclusion. RESULTS: A total of 307 patients receiving pirfenidone were identified for analysis. The mean age was 68.83 (8.13) years and 77% were males. The median time from the first symptoms to IPF diagnosis was 15.5 (9.75-30) months and from diagnosis to start of pirfenidone treatment was 6 (2-23) months. Patients were followed on treatment for a median of 17 (12-22.75) months. Seventy-four patients (24.1%) required dose adjustments and 35 (11.4%) were chronically treated with different than the full recommended dose. A total of 141 patients (45.92%) discontinued therapy due to different reasons including ADRs (16.61%), death (8.79%), disease progression (6.51%), patient's own request (5.54%), neoplastic disease (3.91%) and lung transplantation (0.33%). Over up to 24 months of follow-up, the pulmonary function remained largely stable. The median annual decline in forced vital capacity (FVC) during the first year of pirfenidone therapy was -20 ml (-200-100) and during the second year was -120 ml (-340-30). Over a study period, 33 patients (10.75%) died. CONCLUSIONS: The PolExPIR study is a source of longitudinal RWD on pirfenidone therapy in the Polish cohort of patients with IPF supporting its long-term acceptable safety and efficacy profiles and reinforce findings from the previous randomised clinical trials and observational studies.

NETest Liquid Biopsy Is Diagnostic of Lung Neuroendocrine Tumors and Identifies Progressive Disease.

BACKGROUND: There are no effective biomarkers for the management of bronchopulmonary carcinoids (BPC). We examined the utility of a neuroendocrine multigene transcript "liquid biopsy" (NETest) in BPC for diagnosis and monitoring of the disease status. AIM: To independently validate the utility of the NETest in diagnosis and management of BPC in a multicenter, multinational, blinded study. MATERIAL AND METHODS: The study cohorts assessed were BPC (n = 99), healthy controls (n = 102), other lung neoplasia (n = 101) including adenocarcinomas (ACC) (n = 41), squamous cell carcinomas (SCC) (n = 37), small-cell lung cancer (SCLC) (n = 16), large-cell neuroendocrine carcinoma (LCNEC) (n = 7), and idiopathic pulmonary fibrosis (IPF) (n = 50). BPC were histologically classified as typical (TC) (n = 62) and atypical carcinoids (AC) (n = 37). BPC disease status determination was based on imaging and RECIST 1.1. NETest diagnostic metrics and disease status accuracy were evaluated. The upper limit of normal (NETest) was 20. Twenty matched tissue-blood pairs were also evaluated. Data are means ± SD. RESULTS: NETest levels were significantly increased in BPC (45 ± 25) versus controls (9 ± 8; p < 0.0001). The area under the ROC curve was 0.96 ± 0.01. Accuracy, sensitivity, and specificity were: 92, 84, and 100%. NETest was also elevated in SCLC (42 ± 32) and LCNEC (28 ± 7). NETest accurately distinguished progressive (61 ± 26) from stable disease (35.5 ± 18; p < 0.0001). In BPC, NETest levels were elevated in metastatic disease irrespective of histology (AC: p < 0.02; TC: p = 0.0006). In nonendocrine lung cancers, ACC (18 ± 21) and SCC (12 ± 11) and benign disease (IPF) (18 ± 25) levels were significantly lower compared to BPC level (p < 0.001). Significant correlations were evident between paired tumor and blood samples for BPC (R: 0.83, p < 0.0001) and SCLC (R: 0.68) but not for SCC and ACC (R: 0.25-0.31). CONCLUSIONS: Elevated -NETest levels are indicative of lung neuroendocrine neoplasia. NETest levels correlate with tumor tissue and imaging and accurately define clinical progression.

Influence of Gaseous Pollutants on COPD Exacerbations in Patients with Cardiovascular Comorbidities.

Exacerbations of chronic obstructive pulmonary disease (COPD) are a serious public health issue. Ambient pollution and meteorological factors are considered among precipitating factors. There are few data concerning the impact of ambient pollutants other than particulates on COPD exacerbations. Among gaseous pollutants four main groups of substances are primarily monitored: nitrogen oxides (NOx), sulphur dioxide (SO2), carbon monoxide (CO), and ozone (O3). In this study, 12,889 hospitalizations in the years 2006-2014 due to exacerbations of COPD in patients having a co-existing cardiovascular pathology were retrospectively analyzed. Cardiovascular disease was ruled out as the underlying reason of hospitalization. Data concerning the then accompanying gaseous pollutants and weather conditions were collected. The findings were that the impact of SO2 content was significantly associated with the relative risk (RR) of COPD exacerbation when the exposure took place at least 30 days or longer before hospital admission (RR 1.04-1.05; p < 0.05). In contrast, risk of COPD exacerbation rose when a shortening of the time lag between exposure to NOx and hospital admission was considered (RR 1.02-1.04; p < 0.05). O3 exposure was associated with a lower risk irrespective of the length of exposure/exacerbation lag (RR 0.77-0.90; p < 0.05). There were insignificant associations observed for CO. In conclusion, the study demonstrates a salient influence of a co-existing cardiovascular malady on the appearance of COPD-related respiratory exacerbations when the pollutant SO2 and NOx contents rose. In contrast, higher O3 content was associated with a lower risk of COPD exacerbation.

The role of alert pathogens in bacterial diseases in patients hospitalized at the Department of Pulmonary Diseases and Tuberculosis Public Clinical Hospital No 3 in Zabrze in 2008-2012

BACKGROUND: The current public health problem is the increasing bacterial resistance to antibiotics. Microorganisms isolated from infections are more often non-susceptible to most available drugs. The microorganisms producing resistance mechanisms have been classified as so called alert pathogens. METHODS: We performed a total number of 3810 tests of bronchoalveolar lavage and sputum of patients hospitalized for respiratory diseases at the Department of Pulmonary Diseases and Tuberculosis at Public Clinical Hospital No 3 in Zabrze (Poland). The research was performed in the microbiological laboratory of the Department of Microbiology and Immunology in Zabrze, Medical University of Silesia in Katowice, Poland. The analysis included Gram-positive and Gram-negative alert species strains. RESULTS: In the period of five years, 144 strains of alert microorganisms have been isolated. The percentage of Gramnegative alert pathogens producing ESBL and KPC increased. MRSA, Steptococcus pneumoniae and Streptococcus pyogenes have been found to be the most often present among Gram-positive alert microorganisms. The lowest value of cultured alert pathogens (3.9%) was noted in 2008, whereas the highest (16.5%) in 2011. Gram-positive alert microorganisms showed resistance to macrolides and lincosamides, however, Gram-negative alert microorganisms showed the highest percentage of resistance to penicillins, penicillins with inhibitors and cephalosporins. CONCLUSIONS: Our work has shown that over the period 2008­2012 an increased percentage of Gramnegative and Gram-positive alert microorganisms was observed.

Aspergilloma in atypical localisation in severe asthma patient - case report.

Pulmonary aspergillosis is a condition caused by the fungi Aspergillus. The form of disease depends on the immunological condition of the host organism and other concomitant illnesses that influence the pulmonary tissue. Asthmatic patients, in particular with the severe form of disease, who require the use of systemic glucocorticoids, are predisposed to develop allergic bronchopulmonary aspergillosis. Development of aspergilloma in the lung is preceded by the formation of pathological cavity in the course of another illness. The study reports a case of a severe asthma patient who developed aspergilloma in atypical localisation, without the presence of predisposing anatomical changes and illnesses.

Selected CC and CXC chemokines in children with atopic asthma.

INTRODUCTION: There are only limited data on CC and CXC chemokines regulation in children with asthma. AIM: We compared the serum profile of selected CC and CXC chemokines in patients with atopic asthma and healthy children. MATERIAL AND METHODS: Serum concentration of CC chemokines RANTES, MCP-1, and CXC chemokines IP-10, MIG, IL-8, RANTES was measured using cytometric bead array in 44 children with atopic asthma and 17 healthy subjects. RESULTS: The concentration of RANTES was significantly higher and the MIG level was lower in all children with asthma as compared to their control counterparts. We observed increased RANTES and decreased MIG levels also in patients with stable asthma when compared with children in the control group. The IP-10 concentration was similar between the whole asthma group and healthy controls, while significantly increased levels of this chemokine in acute asthma have been observed when compared to stable asthma. For MCP-1 and IL-8, the serum concentration was similar in all compared groups. The MIG concentration correlated positively with IP-10, IL-8, and CRP levels and negatively with the eosinophil count. A negative correlation between the IP-10 and eosinophil count and a negative correlation between FEV1 and IP-10 were found. CONCLUSIONS: An increased serum RANTES level in children with asthma may result in enhancement of Th2 lymphocyte recruitment into the airway. A decreased expression of Th1 chemokine MIG in children with stable asthma may contribute to a diminished antagonizing effect on Th2 cytokine production and hence intensify Th2 predominance. An increased IP-10 level in children during an asthma attack suggest that this chemokine is a serological marker of disease exacerbation.

Circulating concentration of markers of angiogenic activity in patients with sarcoidosis and idiopathic pulmonary fibrosis.

BACKGROUND: Angiogenesis is an important process involved in the pathogenesis of diffuse parenchymal lung diseases. The aim of the study was to compare the angiogenic profile of patients with sarcoidosis and idiopathic pulmonary fibrosis (IPF) based on analysis of circulating factors. METHODS: Serum concentrations of angiopoietin-2 (Ang-2), follistatin, granulocyte-macrophage-colony stimulating factor (GM-CSF), interleukin-8 (IL-8), platelet derived growth factor-BB (PDGF-BB), platelet endothelial cellular adhesion molecule-1 (PECAM-1) and vascular endothelial growth factors (VEGF) were measured in the patients and the healthy subjects. RESULTS: Serum concentrations of G-CSF, follistatin, PECAM-1 and IL-8 were significantly higher in the IPF patients in comparison with the control group and the sarcoid patients. PDGF-BB concentrations were also significantly higher in serum of IPF patients than in sarcoid patients, but not than in the controls. In contrast, Ang-2 and VEGF concentrations did not differ significantly between the three groups. In the sarcoid patients, irrespective of the disease activity or the radiological stage, serum concentrations of these cytokines were similar to the control group. CONCLUSIONS: These results indicate that differences may exist in angiogenic activity between patients with parenchymal lung diseases. In contrast to sarcoidosis, IPF is characterized by a higher serum concentration of different molecules involved in the angiogenic processes .

Fatigue in patients with inactive sarcoidosis does not correlate with lung ventilation ability or walking distance. Pilot Study.

INTRODUCTION: Fatigue is one of many symptoms reported by patients with sarcoidosis. It is believed that fatigue may be the cause of exercise intolerance and reduced quality of life in patients with sarcoidosis. The purpose of the work was to present the frequency of fatigue prevalence in patients with sarcoidosis and to investigate the correlation between fatigue and the results of pulmonary function tests and walking distance. MATERIAL AND METHODS: A total of 74 patients with sarcoidosis in a stable phase of the disease, not treated in the past with glucocorticoids or immunosuppressive drugs, and without indications for treatment at the time of the study were examined. In all patients fatigue evaluation was carried out with the use of the Fatigue Assessment Scale questionnaire (FAS); dyspnoea was assessed with the use of the Medical Research Council scale (MRC). Body Mass Index (BMI), spirometry, and a 6-minute walk test were additionally performed. The control group included 30 healthy volunteers who completed the FAS. RESULTS: In the examined group of patients fatigue was diagnosed in 36 patients (50%), and in 5 (6.94%) - strong fatigue was observed. The remaining 31 (43.06%) patients felt no fatigue. The average value of points obtained by FAS questionnaire in sarcoidosis patients was significantly higher than the respective value in the control group (p = 0.02). A significantly higher number of points by FAS questionnaire was observed in female patients with sarcoidosis (p = 0.04) in comparison to men. No significant statistical correlation between fatigue index FAS and BMI (r = 0.22, p = 0.11), FEV1 (r = -0.11, p = 0.3), FEV1% pred. (r = 0.01, p = 0.9), FVC (r = -0.03, p = 0.77), FEF25-75 (r = -0.23, p = 0.1) and the distance in the six-minute walk test (6MWT) (r = -0.01, p = 0.9) was observed. However, there was a weak negative correlation between the age of the patients and the FAS index (r = -0.29, p = 0.01). CONCLUSIONS: Fatigue in patients with sarcoidosis does not correlate with the results of lung function tests or with walking distance in 6MWT.

Elevated levels of exhaled nitric oxide in patients with anorexia nervosa.

BACKGROUND: Nitric oxide (NO) is involved in eating behavior and inflammatory response. Moreover, there is evidence that NO production is altered in patients with anorexia nervosa (AN). AIM: To assess whether the overproduction of NO in AN can affect NO level in exhaled air. MATERIALS AND METHODS: Exhaled NO level was studied in 23 girls with AN and compared with that of healthy age- and gender-matched nonatopic controls. RESULTS: Exhaled NO levels were significantly higher in girls with AN compared with healthy age-matched controls. CONCLUSIONS: It appears that anorexia nervosa was accompanied by a higher level of exhaled NO, likely resulting from a systemic increase in NO production because of the severe catabolic state.

Chemerin serum levels in girls with anorexia nervosa.

BACKGROUND: The regulatory function of chemerin (CHEM) in the process of adipogenesis and the metabolism of adipocytes has been confirmed. Data from several studies have shown higher serum CHEM in obesity. To date, there are no available studies on serum CHEM concentrations in patients with anorexia nervosa (AN), which is recognized as a good biological model of the chronic atrophy of adipose tissue and energy metabolism disorders in humans. OBJECTIVES: The aim of the study was to assess serum CHEM concentrations in girls with AN in comparison to healthy and obese subjects and determine its relationship with body mass, BMI and insulin. METHODS: CHEM serum concentrations were evaluated using commercially available ELISA kit in 65 Polish girls with restrictive AN, in 39 healthy controls (H) and 64 girls with simple obesity (OB). RESULTS: The mean serum CHEM concentration in the AN group was significantly lower than in the H and OB groups. After adjusting for BMI, CHEM concentrations in the AN group were significantly lower than in the H group, but statistically higher than in the OB group. Significant correlations between serum CHEM and body mass (r=0.77), BMI (r=0.82), Cole index (r=0.81) and serum insulin (r=0.78) were observed.

Evaluation of adipokines: apelin, visfatin, and resistin in children with atopic dermatitis.

Very little is known about the role of adipokines in atopic dermatitis (AD) in children. This study aimed at analyzing the serum levels of resistin, apelin, and visfatin in children with AD in relation to body weight, AD severity, and gender. Serum concentration of adipokines was measured in 27 children with AD and in 46 healthy subjects. Selected biochemical parameters were evaluated and skin prick test was performed. Serum levels of resistin and apelin were significantly higher, whereas serum visfatin concentration was significantly lower in children with AD versus healthy controls, although an increase in resistin levels was exclusively demonstrated in boys. In AD group, a significant increase in apelin levels in girls was documented. There was no relationship between adipokines levels and the degree of allergic sensitization. Receiver operating characteristic curve analysis demonstrated that the serum apelin cutoff value differentiating children with AD from those without was >137.8 pg/mL. Resistin and visfatin cutoff values were >3.8 ng/mL and ≤ 2.13 ng/mL, respectively. Apelin and visfatin can serve as excellent indicators to distinguish children with AD from those without disease.

Serum resistin levels are elevated in schoolchildren with atopic asthma.

OBJECTIVES: There are limited data on the role of adipokines in atopic asthma. DESIGN AND SETTING: To determine serum levels of resistin in asthmatic children in relation to body weight, asthma severity and gender, serum resistin (RES) levels were measured using ELISA in 89 asthmatic children (61 boys and 28 girls, aged 7.0-17.0 years) and in 33 healthy children. Among examined asthmatics 59 (19 girls and 40 boys) had normal weight (ANW) and 30 (9 girls and 21 boys) were obese (AO). RESULTS: The mean serum levels of resistin were significantly (p<0.01) higher in all non-obese asthmatic children (4.11±0.1 ng/mL) than in healthy children (3.83±0.1 ng/mL). After stratifying by gender only ANW boys and AO boys had significantly higher RES levels than boys from control group. Both AO (4.4±0.2 ng/mL) and ANW girls (4.38±0.2 ng/mL) as well as girls from control (4.09±0.1) group showed significantly higher mean RES serum concentrations than boys from corresponding groups (3.99±0.1 ng/ml, 3.83±0.17 ng/ml and 3.44±0.06 ng/ml, respectively). No relationship between examined adipokine levels and asthma severity, spirometric parameters, degree of allergic sensitization, BMI, BMI-SDS was stated. CONCLUSION: Increased serum RES in children with atopic asthma suggest that this adipokine may be implicated in its pathogenesis.

Determinants of daily physical activity limitation in patients with idiopathic pulmonary fibrosis.

The purpose of the study was to determine the level of physical fitness assessed based on the physiological parameters and intensity of daily physical activity (PA) of patients with idiopathic pulmonary fibrosis (IPF). Additionally, we aimed to determine the intensity and duration of exercise that would bring beneficial modifications in the cardio-respiratory system of the patients with IPF. Eighteen patients with IPF (61.7 ± 4.3 years) and fifteen healthy volunteers performed a graded exercise test to exhaustion on a treadmill (Bruce protocol). Spirometry, dyspnea (mMRC, Borg scale) and fatigue (FAS) were measured. Total daily PA (kcal/day, MET) was monitored for seven days. The linear regression of PA (kcal/day) vs. peak oxygen uptake (%pred. peakVO2) was used to determine the intensity of daily PA that should be used in the rehabilitation of the patients with IPF. The average energy expenditure of daily PA of patients with IPF was 147.9 ± 86.4 kcal/day and it was significantly lower compared to healthy individuals. The linear regression indicated that the predicted energy expenditure of daily PA (PAEE) is 280.0 kcal/day, estimated based on VO2peak 100%pred. Therefore, the patients should add about 30 min of exercise of the intensity of 4.5 ± 0.2 kcal (calculated at the anaerobic threshold) or about 3700 steps/day to their daily PA. Diffusion for carbon monoxide and physiological variables of aerobic capacity seem to be the most important determinants of PA limitation in patients with IPF. The method of estimating PAEE should be used to plan training loads in IPF rehabilitation.

Impact of physical functional capacity on quality of life in patients with interstitial lung diseases.

This study aimed to investigate the physical functioning predictors for health-related quality of life (HRQL) decline in patients with idiopathic interstitial fibrosis (IPF), sarcoidosis and other interstitial lung disease (ILD). The study enrolled 52 patients with ILD and 16 healthy individuals. Participants' HRQL was assessed using the 36-item Short-Form Health Survey questionnaire. Spirometry, physical performance, and daily physical activity (PA) were monitored. Patients with IPF showed significantly lower PA compared to patients with other ILD (p = 0.002)and sarcoidosis (p = 0.01). The type of disease aetiology had no significant effect on aerobic capacity, HRQL and fatigue. Patients with ILD showed significant greater fatigue, lower physical functioning and greater physical aspects scores compared to the control group (F=6.0; p = 0.018; F=12.64; p = 0.001, respectively). A significant positive correlation was observed between 6-minute walking distance (6MWD) and the physical domain of HRQL (r = 0.35, p = 0.012) and PA and the physical aspects of HRQL (r = 0.37, p = 0.007). This study revealed that the key predictors for HRQL decline were lower lung function, lower PA and physical performance.

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.

Expectations, symptoms, and quality of life before and after 1 year of Pirfenidone treatment in patients with idiopathic pulmonary fibrosis: A single-arm, open-label nonrandomized study.

Background and Aims: Antifibrotic therapies reduce lung function decline in patients with idiopathic pulmonary fibrosis (IPF). This single-arm, open-label, nonrandomized study aimed to determine the influence of antifibrotic treatment on patients' reported symptoms and expectations of the therapy. Methods: Fifty-two patients with confirmed IPF at a mean age of 65 ± 8.63 years (73% male) completed the following surveys at baseline and after 12 months of Pirfenidone treatment: Short Form Healthy Survey (SF-36), St. George's Respiratory Questionnaire (SGRQ), Baseline Dyspnea Index (BDI), Fatigue Assessment Scale (FAS), Leicester Cough Questionnaire (LCQ), and Patient's Needs and Expectations Authors' Survey. Results: The most important patients' needs were access to novel therapy, fast and easy access to health centers specializing in IPF treatment, and the improvement of the general condition or the maintenance of its level. These needs did not change with time, except for the significantly more important right of deciding on disease management after 12 months of treatment (p = 0.014). The quality of life per SF-36, after 1 year of Pirfenidone treatment, significantly improved in the physical cumulative score (p = 0.004) and mental cumulative score (p = 0.003). Significant deteriorations were observed in bodily pain and vitality. For the remaining questionnaires (SGRQ, BDI, FAS, and LCQ), no significant changes in the course of the study were noticed. Around one in 10 patients subjected to Pirfenidone therapy had achieved general symptom improvement in all areas; that is, quality of life improvement as well as cough and dyspnea reduction. Conclusions: One year of antifibrotic treatment resulted in a general improvement in the quality of life per the SF-36 questionnaire. Patients' expectations of disease management did not change; also, access to novel therapies and easy access to health centers specializing in IPF management remained their top needs.

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.

Assessment of serum visfatin levels in girls with anorexia nervosa.

OBJECTIVE: Visfatin (VISF) is a recently described peptide regulating the process of adipocyte differentiation. Only one pilot study of VISF expression in the fat tissue and its circulating concentrations in a small group of patients with anorexia nervosa (AN) have been published, yet. DESIGN AND PATIENTS: Cross-sectional assessment of VISF serum concentrations in 195 girls aged 11-18·9 years with AN (n = 87), eating disorders not otherwise specified (NOS; n = 17), simple obesity (OB; n = 30), and healthy controls (H; n = 61). MEASUREMENTS: Blood samples were collected during the fasting state between 7:00 am-8:30 am. VISF serum concentrations were determined using enzyme immunoassay. Comparisons of VISF levels between groups were performed. RESULTS: Mean serum VISF concentrations in girls with AN and NOS were significantly lower than those in the H and OB groups. Serum VISF concentrations were higher in the OB than in the H groups. When were calculated per body mass index (BMI), VISF concentrations were significantly lower in the AN, NOS, and OB groups than in healthy controls. Among participants with a normal BMI, serum VISF concentrations correlated positively with BMI (r = 0·27; P < 0·05). In the OB group, a significant, negative correlation between BMI and VISF levels (r = -0·38; P = 0·04) was observed. CONCLUSIONS: Compared with healthy girls, serum VISF concentrations are decreased in girls with AN. Conversely, obese girls have elevated VISF levels. When calculated per BMI (VISF/BMI), the results in AN and OB groups were lower than in healthy participants.