There are limited data on referral rates and the number of patients with idiopathic pulmonary fibrosis (IPF) who are eligible for lung transplantation. The aim of the present study was to assess adherence to the consensus of the International Society for Heart and Lung Transplantation (ISHLT) for the referral of patients with IPF among Czech interstitial lung disease (ILD) centers. Czech patients who were diagnosed with IPF between 1999 and 2021 (n = 1584) and who were less than 65 years old at the time of diagnosis were retrospectively selected from the Czech Republic of the European Multipartner Idiopathic Pulmonary Fibrosis Registry (EMPIRE). Nonsmokers and ex-smokers with a body mass index (BMI) of <32 kg/m2 (n = 404) were included for further analyses. Patients with a history of cancer <5 years from the time of IPF diagnosis, patients with alcohol abuse, and patients with an accumulation of vascular comorbidities were excluded. The trajectory of individual patients was verified at the relevant ILD center. From the database of transplant patients (1999-12/2021, n = 541), all patients who underwent transplantation for pulmonary fibrosis (n = 186) were selected, and the diagnosis of IPF was subsequently verified from the patient's medical records (n = 67). A total of 304 IPF patients were eligible for lung transplantation. Ninety-six patients were referred to the transplant center, 50% (n = 49) of whom were referred for lung transplantation. Thirty percent of potentially eligible patients not referred to the transplant center were considered to have too many comorbidities by the reporting physician, 19% of IPF patients denied lung transplantation, and 17% were not referred due to age. Among Czech patients with IPF, there may be a larger pool of potential lung transplant candidates than has been reported to the transplant center to date.

• MeSH
• dodržování směrnic statistika a číselné údaje   MeSH
• dospělí MeSH
• idiopatická plicní fibróza * chirurgie   MeSH
• intersticiální plicní nemoci chirurgie   MeSH
• konziliární vyšetření a konzultace * statistika a číselné údaje   MeSH
• lidé středního věku MeSH
• lidé MeSH
• registrace MeSH
• retrospektivní studie MeSH
• senioři MeSH
• transplantace plic * statistika a číselné údaje   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

INTRODUCTION: The antifibrotic drug nintedanib is used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the effect of nintedanib on antifibrotic treatment outcome in real-world cohorts of Czech EMPIRE registry. PATIENTS/METHODS: Data of 611 Czech IPF subjects, 430 (70%) treated with nintedanib (NIN group), 181 (30%) with no-antifibrotic treatment (NAF group) were analysed. The influence of nintedanib on overall survival (OS), pulmonary function parameters as forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO), as well as GAP score (gender, age, physiology) and and CPI (composite physiological index) were investigated. RESULTS: During 2 year follow-up we observed that nintedanib treated patients had longer OS, compared to those treated with no-antifibrotic drugs (p < 0.00001). Nintedanib reduces risk of mortality over no-antifibrotic treatment by 55% (p < 0.001). We have observed no significant difference in the rate of FVC and DLCO decline between the NIN and NAF group. Changes within 24 months from baseline in CPI were not significant between the groups (NAF and NIN). CONCLUSION: Our real-practice study showed the benefit of nintedanib treatment on survival. There were no significant differences between NIN and NAF groups in changes from baseline in FVC %, DLCO % predicted and CPI.

• MeSH
• idiopatická plicní fibróza * farmakoterapie   MeSH
• lidé MeSH
• plíce MeSH
• registrace MeSH
• vitální kapacita MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

There are concerns about altered vascular functions that could play an important role in the pathogenesis and influence the severity of chronic disease, however, increased cardiovascular risk in paediatric cystic fibrosis (CF) has not been yet fully understood. Aim was to analyse vascular disease risk and investigate changes over times in CF and controls. We prospectively enrolled 22 CF subjects (a median age of 16.07 years), and 22 healthy demographically matched controls (a median age of 17.28 years) and determined endothelial function. We utilised a combined diagnostic approach by measuring the plethysmographic Reactive Hyperemia Index (RHI) as the post-to preocclusive endothelium-dependent changes of vascular tone, and biomarkers that are known to be related to endothelial dysfunction (ED): asymmetric dimethyl arginine (ADMA), high-sensitive CRP (hsCRP), VCAM-1 and E-selectin. RHI values were significantly lower in CF young adults (p<0.005). HsCRP (p<0.005), E-selectin (p<0.001) and VCAM-1 (p<0.001) were significantly increased in CF patients since childhood. The findings have provided a detailed account of the ongoing process of microvascular dysfunction with gradual progression with the age of CF patients, making them further at risk of advanced vascular disease. Elevations of biomarkers in CF children with not yet demonstrated RHI changes but with significantly reduced RHI in adulthood and lipid profile changes indicate the possible occurrence of ED with CF-related specific risk factors over time and will enable us to provide the best possible support.

• MeSH
• biologické markery krev   MeSH
• cévní endotel patofyziologie   MeSH
• cystická fibróza krev   diagnóza   patofyziologie   MeSH
• dítě MeSH
• dospělí MeSH
• hyperemie * MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• progrese nemoci MeSH
• prospektivní studie MeSH
• studie případů a kontrol MeSH
• věkové faktory MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• pozorovací studie MeSH

Cystická fibróza (CF) se řadí mezi závažné, progredující, zatím nevyléčitelné nemoci. Klinicky se manifestuje zejména obrazem chronického sinopulmonálního onemocnění. Základní terapeutické principy založené na komplexní symptomatické léčbě jsou nově rozšířeny o léčbu modulátory chloridového kanálu a zintenzivnění respirační fyzioterapie využitím přístroje PhysioAssist (Simeox). Vzhledem k postupné progresi ireverzibilních plicních změn s věkem je nezbytné se zaměřit již od raného věku na techniku provádění mukociliární očisty dýchacích cest a u nemocných s dobrou compliance se snažit o rozšíření standardních metod airway clearance přístrojovou podporou s cílem zajistit efektivní expektoraci a minimalizovat tak rozvoj akutních exacerbací. Účinné odhlenění umožňuje snížit počet hospitalizací a potřebu antibiotické terapie, zpomaluje zhoršování plicních funkcí a vede ke zlepšení kvality života. PhysioAssist zaujímá nezastupitelnou roli v péči o nemocné s CF s poruchou expektorace, jak dokládá námi prezentovaná kazuistika 18leté dívky.

Cystic fibrosis (CF) is one of the serious, progressive, as yet incurable diseases. It is clinically manifested mainly by the image of chronic sinopulmonary disease. The basic therapeutic principles based on complex symptomatic treatment are extended by treatment with chloride channel modulators and intensification of respiratory physiotherapy using the PhysioAssist device (Simeox). Due to the age-related gradual progression of irreversible pulmonary changes, it is necessary to focus on the mucociliary airway clearance technique from an early age and to extend standard airway clearance methods with instrumental support in patients with good compliance to ensure effective expectoration and minimize the development of acute exacerbations. Effective attenuation leads to a reduction in the number of hospitalizations and antibiotics, to a slowdown in the deterioration of lung function, and thus to an improvement in the quality of life. PhysioAssist plays an irreplaceable role in the care of CF patients with expectorant disorder, as evidenced by the presented case report of an 18-year-old girl.

• MeSH
• bronchoskopie MeSH
• cystická fibróza * ošetřování   MeSH
• diagnostické zobrazování MeSH
• dítě MeSH
• lidé MeSH
• počítačová rentgenová tomografie MeSH
• poruchy dýchání rehabilitace   MeSH
• spirometrie MeSH
• techniky fyzikální terapie MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

BACKGROUND The antifibrotic drugs nintedanib and pirfenidone are used for the treatment of idiopathic pulmonary fibrosis IPF We analysed the association of common profibrotic polymorphisms in MUC5B mucin 5B rs35705950 and DSP desmoplakin rs2076295 on antifibrotic treatment outcomes in IPF METHODS MUC5B rs35705950 and DSP rs2076295 were assessed in IPF patients n 210 139 men 71 women from

• MeSH
• desmoplakiny * genetika   MeSH
• idiopatická plicní fibróza * farmakoterapie   genetika   MeSH
• indoly * terapeutické užití   MeSH
• lidé MeSH
• mutace MeSH
• pilotní projekty MeSH
• pyridony * terapeutické užití   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

• MeSH
• bronchiektazie diagnóza   etiologie   klasifikace   MeSH
• cystická fibróza * diagnóza   farmakoterapie   patofyziologie   terapie   MeSH
• lidé MeSH
• progrese nemoci MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• přehledy MeSH

• MeSH
• anamnéza MeSH
• biopsie metody   MeSH
• bronchoalveolární laváž metody   MeSH
• Churgův-Straussové syndrom diagnóza   farmakoterapie   MeSH
• diagnostické zobrazování klasifikace   MeSH
• diferenciální diagnóza MeSH
• granulomatóza s polyangiitidou diagnóza   farmakoterapie   patologie   MeSH
• hrudní chirurgie video-asistovaná metody   MeSH
• hypersenzitivní pneumonitida diagnóza   MeSH
• idiopatická plicní fibróza diagnóza   farmakoterapie   terapie   MeSH
• idiopatické intersticiální pneumonie klasifikace   MeSH
• interpretace obrazu počítačem MeSH
• intersticiální plicní nemoci * diagnóza   etiologie   farmakoterapie   klasifikace   patofyziologie   MeSH
• lidé MeSH
• pneumokonióza etiologie   klasifikace   MeSH
• rentgendiagnostika hrudníku MeSH
• sarkoidóza diagnóza   farmakoterapie   patofyziologie   MeSH
• stupeň závažnosti nemoci MeSH
• vaskulitida diagnóza   farmakoterapie   klasifikace   patologie   MeSH
• vzácné nemoci diagnóza   farmakoterapie   patologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• přehledy MeSH

INTRODUCTION: Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12 months, however limited data on the decline of lung function and overall survival (OS) in real-world cohorts on longer follow-up exists. PATIENTS/METHODS: Of the enrolled Czech IPF patients (n = 841) from an EMPIRE registry, 383 (45.5%) received pirfenidone, 218 (25.9%) no-antifibrotic treatment and 240 (28.5%) were excluded (missing data, nintedanib treatment). The 2- and 5-yrs OS and forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) were investigated at treatment initiation and 6, 12, 18 and 24 months' follow-up. RESULTS: During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ≥10% FVC (17.0%) and ≥ 15% DLCO (14.3%). On pirfenidone, the DLCO (≥10%) declines at 6, 12, 18 and 24 months' and DLCO (≥15%) declines at 6, 18 and 24 months' follow-up were associated with increased mortality. The DLCO decline showed higher predictive value for mortality than FVC decline. In patients with no-antifibrotics, FVC and DLCO declines were not predictive for mortality. Pirfenidone increased 5-yrs OS over no-antifibrotic treatment (55.9% vs 31.5% alive, P = 0.002). CONCLUSION: Our study observed the 2-yrs sustained effect of pirfenidone on the decline of lung function and survival in the real-world patient's IPF cohort. DLCO decline of ≥10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.

• MeSH
• antiflogistika nesteroidní farmakologie   terapeutické užití   MeSH
• idiopatická plicní fibróza diagnóza   farmakoterapie   epidemiologie   MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• míra přežití trendy   MeSH
• následné studie MeSH
• progrese nemoci * MeSH
• pyridony farmakologie   terapeutické užití   MeSH
• registrace * MeSH
• respirační funkční testy trendy   MeSH
• senioři MeSH
• vitální kapacita účinky léků   fyziologie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

INTRODUCTION: Prognostic factors of idiopathic pulmonary fibrosis (IPF) currently recognized include changes in vital capacity and radiologic findings. However, most of the prognostic studies in IPF are based on clinical studies with preselected IPF populations. Therefore, we decided to analyze the factors influencing IPF prognosis based on the real-practice data from our IPF registry. METHODS: Data of 514 subjects consecutively entered since 2012 into Czech EMPIRE IPF registry were analyzed. RESULTS: Median age of our patient cohort was 67 years (50-82). Median overall survival (OS) of the cohort was 63.1 months. The clinical course of IPF according to FVC (forced vital capacity) changes was stabilized in 32.8% of patients (29.7% according to DLCO [diffuse lung capacity] changes), slowly progressive in 39.5% (45%), rapidly progressive in 23.5% (20.7%); and 1.7% patients had at least one acute exacerbation during follow-up. Deterioration in FVC of ≥10% at month 12 and in DLCO of ≥15% at months 12, 18, and 24 influenced the OS significantly. The fast progressors defined by the DLCO decline rate had higher risk of death compared to those defined by the FVC change over time. In multivariate analysis, age ≥70 years, interstitial HRCT scores ≥3, and change in DLCO of ≥15% at month 12 were confirmed as factors negatively influencing OS. CONCLUSIONS: DLCO changes over time were shown as a better predictor of mortality compared with FVC changes in our study. In our opinion it is necessary to implement the DLCO analysis into clinical trials and routine practice.

• MeSH
• idiopatická plicní fibróza diagnóza   epidemiologie   patofyziologie   MeSH
• incidence MeSH
• lidé středního věku MeSH
• lidé MeSH
• měření objemu plic MeSH
• míra přežití trendy   MeSH
• následné studie MeSH
• plíce diagnostické zobrazování   patofyziologie   MeSH
• počítačová rentgenová tomografie metody   MeSH
• prognóza MeSH
• progrese nemoci MeSH
• registrace * MeSH
• retrospektivní studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• vitální kapacita fyziologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• metaanalýza MeSH
• Geografické názvy
• Česká republika epidemiologie   MeSH