INTRODUCTION: Most patients with idiopathic pulmonary fibrosis (IPF) treated with antifibrotics (AF) have progressive disease despite treatment. A switch of AF may improve survival, but evidence from randomised controlled trials is missing. We aimed to evaluate the efficacy of an AF switch on survival and FVC decline in patients from the European MultiPartner IPF registry (EMPIRE). METHODS: The study included 612 patients who discontinued the first antifibrotic therapy. Patients were grouped and analysed from two perspectives: (1) whether they had received a second antifibrotic treatment after the discontinuation of the first therapy, and (2) a reason for discontinuation of the first AF - "lack of efficacy" (LE) and "intolerance" (INT). RESULTS: While 263 (43%) of 612 patients received no second AF ("non-switched"), 349 (57%) patients switched. Overall survival was higher in patients who received a second AF (median 50 vs. 29 months; adjusted HR 0.64, P=0.023). Similarly, the annual FVC decline was significantly reduced in switched patients: -98ml/y in switched and -172ml/y in non-switched patients (P=0.023), respectively. The switched patients had similar risk for mortality in both LE and INT groups (adjusted HR 0.95, P=0.85). The high impact of switching on survival was demonstrated in LE patients (adjusted HR 0.27, P<0.001). CONCLUSION: The patients without a second AF had significantly shorter overall survival. Our analysis suggests the importance of switching patients with an ineffective first AF therapy to a second AF therapy.
- MeSH
- bronchiolitida * diagnostické zobrazování klasifikace patologie MeSH
- lidé MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
BACKGROUND: There is a lack of data on the long-term effect of nintedanib on survival in specific groups of idiopathic pulmonary fibrosis (IPF) patients with different phenotypes. We investigated the outcomes of nintedanib therapy in an observational study of a large multicentre real-world cohort of IPF patients with various initial characteristics. METHODS: The analysis included IPF patients treated with nintedanib (NIN) and IPF patients not receiving antifibrotic treatment (NAF) enrolled for the EMPIRE registry in 2015-2020. The patients were stratified according to their initial FVC predicted, dyspnoea, UIP pattern and age. All-cause mortality and annual rate of FVC decline were the main endpoints. Cox proportional hazards model for survival assessment and linear mixed-effects model for FVC decline modelling were used. RESULTS: A total of 869 NIN patients and 691 NAF patients were eligible for the analysis. The annual FVC decline rate was significantly different (adjusted values -0.053 l/yr vs -0.122 l/yr; p = 0.001). The adjusted hazard ratio (HR) for mortality was 0.40 (95 % CI 0.3 to 0.53, p < 0.001). The most significant effect of nintedanib was demonstrated in patients with impaired lung function, i.e., with an FVC predicted to be less than 80 % and a NYHA II to IV. Nintedanib therapy also reduced the difference in survival between men and women. CONCLUSIONS: Modelling confirmed that NIN therapy reduced differences in OS between patients with better and worse initial conditions and prognosis. Our results indicate that NIN is particularly beneficial for patients with advanced IPF and more severe phenotypes. TRIAL REGISTRATION: EMPIRE was registered as a non-interventional post-registration study at the State Institute for Drug Control of the Czech Republic under ID 1412080000 on December 8, 2014.
- MeSH
- antifibrotické látky terapeutické užití MeSH
- časové faktory MeSH
- fenotyp MeSH
- idiopatická plicní fibróza * farmakoterapie mortalita patofyziologie MeSH
- indoly * terapeutické užití MeSH
- lidé středního věku MeSH
- lidé MeSH
- proporcionální rizikové modely MeSH
- registrace MeSH
- retrospektivní studie MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- vitální kapacita MeSH
- výsledek terapie 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
- multicentrická studie MeSH
- pozorovací studie MeSH
ntersticiální plicní onemocnění (ILD) je poměrně častou manifestací systémových revmatických onemocnění, zejména u systémové sklerodermie (SSc), revmatoidní artritidy, idiopatických zánětlivých myopatií, systémového lupus erythematodes, primárního Sjögrenova syndromu a mikroskopické polyangiitidy ze skupiny ANCA asociovaných vaskulitid. Přítomny mohou být nejrůznější typy postižení, nejčastěji se jedná o nespecifickou intersticiální pneumonii (NSIP); u pacientů s revmatoidní artritidou a s mikroskopickou polyangiitidou bývá častější výskyt obvyklé intersticiální pneumonie (UIP). Ve screeningu ILD jsou využívány funkční plicní testy a zobrazovací metody (prostá radiografie hrudníku, HRCT plic), důležitá je včasná identifikace pacientů vyžadujících léčbu a správný výběr terapie s ohledem na její možnou toxicitu. U převažujícího zánětlivého postižení je možné použít kortikosteroidy, cyklofosfamid, mykofenolát mofetil, azathioprin, kalcineurinové inhibitory, B-depleční terapii či tocilizumab. U převážně fibrotizujících forem se naopak uplatňují antifibrotické léky, zejména nintedanib.
Interstitial lung disease (ILD) is a relatively frequent manifestation of systemic autoimmune rheumatic disorders, especially in systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, primary Sjögren ́s syndrome and microscopic polyangitis from the group of ANCA associated vasculitis. ILD may present different patterns, most commonly nonspecific interstitial pneumonia (NSIP); usual interstitial pneumonia (UIP) is more often present in patients with rheumatoid arthritis and microscopic polyangitis. Functional lung testing and imaging methods (plain radiography, HRCT) are used for screening of ILD, identifying patients who need treatment and choosing appropriate therapy is important to minimize the risk of therapy-related toxicity. For inflammatory forms of the disorders it is possible to use glucocorticoids, cyclophosphamide, mycophenolate mofetil, azathioprine, calcineurin inhibitors, B-depletion therapy or tocilizumab. There is an indication for the use of antifibrotic drugs, especially nintedanib in predominantly fibrotic forms of the disease.
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
OBJECTIVES: Physical activity is reduced in patients with interstitial lung disease (ILD) and physical inactivity is related to poor health outcomes. We investigated the effect of a telecoaching intervention to improve physical activity in patients with ILD. METHODS: Eighty patients with ILD were randomized into the intervention or control group. Patients in the intervention group received a 12-week telecoaching program including a step counter, a patient-tailored smartphone application, and coaching calls. Patients in the control group received usual care. Physical activity (primary outcome), physical fitness and quality of life were measured at baseline and 12 weeks later with an accelerometer, 6-min walking test and quadriceps muscle force and the King's Brief Interstitial Lung Disease questionnaire (K-BILD). RESULTS: Participation in telecoaching did not improve physical activity: between-group differences for step count: 386 ± 590 steps/day, p = .52; sedentary time: 4 ± 18 min/day, p = .81; movement intensity: 0.04 ± 0.05 m/s2, p = .45). Between-group differences for the 6-min walking test, quadriceps muscle force and K-BILD were 14 ± 10 m, p = .16; 2 ± 3% predicted, p = .61; 0.8 ± 1.7 points, p = .62 respectively. CONCLUSIONS: Twelve weeks of telecoaching did not improve physical activity, physical fitness or quality of life in patients with ILD. Future physical or behavioural interventions are needed for these patients to improve physical activity.
- MeSH
- cvičení MeSH
- intersticiální plicní nemoci * terapie MeSH
- kvalita života MeSH
- lidé MeSH
- mentoring * MeSH
- průzkumy a dotazníky MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- randomizované kontrolované studie MeSH
Sarkoidóza je multisystémové granulomatózní onemocnění neznámé etiologie. Koincidence sarkoidózy a lymfoproliferativního onemocnění byla v literatuře popsána již dříve, avšak jako ojedinělé případy. Výskyt lymfoproliferativního onemocnění u pacientů s chronickou aktivní sarkoidózou vytváří diagnosticky náročný úkol. Obě nemoci mají mnoho společných projevů, včetně klinických symptomů, periferní a mediastinální lymfadenopatie, hematologických abnormalit, hyperkalcemie a postihují podobné orgány - plíce, játra a slezinu. Tato kazuistika popisuje případ muže, u kterého došlo k postupnému rozvoji obou onemocnění.
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- MeSH
- diagnostické zobrazování metody MeSH
- difúzní velkobuněčný B-lymfom * diagnóza terapie MeSH
- dospělí MeSH
- lidé MeSH
- protinádorové látky terapeutické užití MeSH
- sarkoidóza * diagnóza terapie MeSH
- transplantace kostní dřeně metody MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
Transplantace je poslední možností léčby u terminálních stadií řady plicních nemocí. Jednou z limitací dlouhodobé prognózy pacientů je rejekce štěpu. Některé faktory vzniku a progrese odmítnutí transplantátu jsou známy a lze je léčebně ovlivnit. Poznatky z recentních studií odhalují, že jedním z dosud přehlížených faktorů může být také znečištění ovzduší. Článek shrnuje aktuální poznatky o vlivu chronické expozice inhalačním noxám na pacienty po plicní transplantaci, a to zejména s ohledem na vznik rejekce štěpu.
