Short- and long-term clinical outcomes of nintedanib therapy in IPF patients with different phenotypes: A retrospective registry-based study.

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.

Adherence to the ISHLT Protocol for the Referral of Patients with Idiopathic Pulmonary Fibrosis to the Transplantation Center among of Czech Centers for Interstitial Lung Diseases.

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 ( = 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).

Physical activity coaching in patients with interstitial lung diseases: A randomized controlled trial.

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.

The Impact of Switching to a Second Antifibrotic in Patients With Idiopathic Pulmonary Fibrosis: A Retrospective Multicentre Study From the EMPIRE Registry.

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).

The effect of nintedanib on lung functions and survival in idiopathic pulmonary fibrosis: real-life analysis of the Czech EMPIRE registry.

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.

Sex transition from female to male as a risk factor for sleep-disordered breathing.

Aim: The female-to-male (FtM) sex transition requires lifelong supplementation with male sex hormones, resulting in high prevalence of weight gain, fat redistribution and other metabolic changes. Although sleep-disordered breathing (SDB) data for this group of patients are very limited, increased prevalence is expected. We report a mini-series of six case reports of FtM transsexuals treated in our centre.

rs2076295 variants influence nintedanib and pirfenidone outcomes in idiopathic pulmonary fibrosis: a pilot study.

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 (mucin 5B, rs35705950) and (desmoplakin, rs2076295) on antifibrotic treatment outcomes in IPF.

Methods: rs35705950 and rs2076295 were assessed in IPF patients ( = 210, 139 men/71 women) from the Czech EMPIRE registry and age- or sex-matched healthy individuals ( = 205, 125 men/80 women).

Drug induced lung toxicity by nitrofurantoin.

We present the clinical case of the patient with nitrofurantoin (FUR) lung toxicity. Diagnosis was made from detailed history of the patient and by studying CT images before the start of FUR treatment. An extensive interstitial changes were evident on HRCT scan at the presentation at our clinic.

Comparison of lymphocyte immune phenotypes in bronchoalveolar lavage of non-smoking patients with sarcoidosis and other interstitial lung diseases.

Background: Bronchoalveolar lavage (BAL) as complementary method is still used as ancillary tool in diagnosis of interstitial lung diseases. Tobacco smoking has been described to affect the BAL lavage cellular profile. To our knowledge, only few reports have so far investigated CD3CD4 and CD3CD8 lymphocyte subsets in non-smoking sarcoidosis patients additionally stratified according to CXR stage, and compared them to other non-smoking patients with interstitial lung diseases (ILDs).

Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry.

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.

A case of accelerated silicosis.

Silicosis, caused by inhaling dust containing free crystalline silica, typically has a chronic course, with the numbers of silicosis patients declining globally. Much rarer are the acute and subacute forms. Presented is a case of severe subacute (accelerated) silicosis.

[The complications after lung transplantation].

Lung transplantation (LuTx) is an important treatment for a selected group of patients in the terminal stage of a number of lung diseases, which can bring them a significant improvement in quality of life and long-term survival. Nowadays a perioperative period is not significant limitation for patient survival due to the development of transplant surgery, but the period of months to years after LuTx is crucial for survival. The post-transplant period is very complicated for LuTx patients due to a special treatment regimen, special medication, especially immunosuppressive drugs and the possibility of many complications, whether early or late or acute or chronic.

[Sarcoidosis - enigmatic disease still unresolved].

Sarcoidosis is a systemic disease of unknown etiology, characterized by the presence of granulomatous inflammation in affected tissues. In about 90 % it affects the lungs, but it may basically affect any organ, the most frequently the skin, lymph nodes and eyes. In the case of classic lung manifestation this disease is not difficult to diagnose.

[A case report of peritoneal tuberculosis: the diagnostic role of PET/CT and laparoscopy].

Objective: We present a case of peritoneal tuberculosis of young woman with focus on difficult diagnosis of this low incidence disease in developed countries.

Design: Case report.

Setting: Department of Gynaecology and Obstetrics, Faculty Hospital, Olomouc.

EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis.

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.

[Sarcoidosis of the female genital tract].

Objective: To present the findings of sarcoidosis on female genital tract.

Design: Review.

Setting: Department of Obstetric and Gynecology, Silesian Hospital Opava.

[Idiopathic pulmonary fibrosis prognostic factors - analysis of the Czech registry].

Unlabelled: Idiopathic pulmonary fibrosis (IPF) is a rare, progressive and usually fatal form of idiopathic interstitial pneumonia. IPF is characterized by failure of alveolar re-epithelization, persistence of fibroblasts, deposition of extracellular matrix, and distortion of lung architecture, which ultimately results in respiratory failure.We analysed 202 consecutive patients with IPF diagnosed at the Departments of Pulmonary Diseases and Tuberculosis in the Czech Republic, who they were included in the nationwide Czech IPF registry.

Correlation Network Analysis Reveals Relationships between MicroRNAs, Transcription Factor T-bet, and Deregulated Cytokine/Chemokine-Receptor Network in Pulmonary Sarcoidosis.

Sarcoidosis is an inflammatory granulomatous disease with unknown etiology driven by cytokines and chemokines. There is limited information regarding the regulation of cytokine/chemokine-receptor network in bronchoalveolar lavage (BAL) cells in pulmonary sarcoidosis, suggesting contribution of miRNAs and transcription factors. We therefore investigated gene expression of 25 inflammation-related miRNAs, 27 cytokines/chemokines/receptors, and a Th1-transcription factor T-bet in unseparated BAL cells obtained from 48 sarcoidosis patients and 14 control subjects using quantitative RT-PCR.

Sarcoidosis of female reproductive organs in a postmenopausal woman: a case report and review of the literature: is there a potential for hormone therapy?

Objective: Sarcoidosis is a multisystem inflammatory disorder of unknown cause that affects multiple organs. To date, only isolated cases of extrapulmonary sarcoidosis of the female reproductive tract, which rarely affects postmenopausal women, have been reported.

Methods: We describe the case of a postmenopausal woman with sarcoidosis of multiple structures of the genital tract accompanied by pulmonary involvement.

Extrapulmonary involvement in patients with sarcoidosis and comparison of routine laboratory and clinical data to pulmonary involvement.

Introduction: Patients with pulmonary and pulmonary plus extrapulmonary sarcoidosis differ in symptom severity and health status impairment. To date there is no information on differences in clinical and laboratory parameters between these phenotypes and limited information on extrapulmonary involvement in Czech sarcoidosis patients exists.

Methods: We therefore compared clinical data (age, gender, organ involvement, lung function tests) and laboratory data (blood counts, bronchoalveolar fluid (BAL) cellular profile, serum levels of CRP, SACE, sIL-2R, neopterin) between patients with newly diagnosed pulmonary sarcoidosis (n=107) and those with pulmonary plus extrapulmonary sarcoidosis (n=54).

Functional variant ANXA11 R230C: true marker of protection and candidate disease modifier in sarcoidosis.

In the recent genome-wide association study the polymorphisms of annexin A11 (ANXA11) gene were associated with susceptibility to sarcoidosis. Beside the replication of this finding and analysis of local ANXA11 expression in bronchoalveolar lavage cells, we wondered whether 'leading' ANXA11 rs1049550 (R230C) variant might also be related to the clinical manifestation of sarcoidosis. The study included 245 Czech patients with sarcoidosis and 254 healthy control subjects.

Interleukin-1 gene cluster polymorphisms in sarcoidosis and idiopathic pulmonary fibrosis.

Members of the interleukin-1 (IL-1) family are implicated in the pathogenesis of sarcoidosis and idiopathic pulmonary fibrosis (IPF). We have, therefore, performed a case-control study to investigate a plausible association between sarcoidosis and the polymorphisms in the IL-1alpha, IL-1beta, and IL-1 receptor antagonist (IL-1Ra) genes. Further, as a separate question, we explored whether the aforementioned genes of the IL-1 cluster are associated with IPF.