The Impact of COVID-19 Infection on Patients with Severe Chronic Pulmonary Hypertension: A Prospective Study from a Single Referral Center.

Background and Objectives. COVID-19 infection has a significant burden on global morbidity and mortality, especially in elderly people and in patients with chronic respiratory and cardiovascular diseases, such as pulmonary hypertension (PH). We aimed to evaluate the impact of COVID-19 infection on patients diagnosed with severe chronic PH. Materials and Methods. A single-center prospective cohort study was performed. Patients were enrolled from 1 November 2020 to 31 December 2022. Follow-up was until 31 December 2023. Data were collected on PH diagnosis, clinical presentation, outcomes, brain natriuretic peptide (BNP) levels, pulmonary function test with lung diffusion capacity for carbon monoxide (DLCO), and computed tomography pulmonary angiography (CTPA) analysis. Results. During the 26 months of our study, 51 PH patients were diagnosed with COVID-19 infection. The majority, 44 (86.3%) of all COVID-19 infected patients, were treated on an outpatient basis, and 7 (13.7%) required hospitalization. During the follow-up period, 8 (15.7%) patients died: 4 (7.8%) due to complications of COVID-19 infection, and the other 4 (7.8%) died in the later stages of the follow-up period after recovery from acute COVID-19 infection. Therefore, the in-hospital mortality in our study was 43% (n = 3). As mentioned above, the overall mortality was 7.8% (n = 4). Higher BNP levels in the third month after COVID-19 were associated with higher mortality rates (p = 0.028). Lung function, including DLCO, did not significantly worsen with COVID-19. In our study, 24 patients (47.1%) were referred for a follow-up CTPA scan and one of them developed typical fibrotic lung changes after COVID-19. Conclusions. The incidence of COVID-19 infection in patients diagnosed with PH was 34%. In our patients with severe chronic PH, the overall mortality rate due to COVID-19 infection was low. Pulmonary fibrosis was a rare complication in our cohort. COVID-19 infection in severe PH may increase the risk of worsening chronic heart failure.

Unmasking a Silent Threat: Improving Pulmonary Hypertension Screening Methods for Interstitial Lung Disease Patients.

This article provides a comprehensive overview of the latest literature on the diagnostics and treatment of pulmonary hypertension (PH) associated with interstitial lung disease (ILD). Heightened suspicion for PH arises when the advancement of dyspnoea in ILD patients diverges from the expected pattern of decline in pulmonary function parameters. The complexity of PH associated with ILD (PH-ILD) diagnostics is emphasized by the limitations of transthoracic echocardiography in the ILD population, necessitating the exploration of alternative diagnostic approaches. Cardiac magnetic resonance imaging (MRI) emerges as a promising tool, offering insights into hemodynamic parameters and providing valuable prognostic information. The potential of biomarkers, alongside pulmonary function and cardiopulmonary exercise tests, is explored for enhanced diagnostic and prognostic precision. While specific treatments for PH-ILD remain limited, recent studies on inhaled treprostinil provide new hope for improved patient outcomes.

Therapeutic Success of Tiotropium/Olodaterol, Measured Using the Clinical COPD Questionnaire (CCQ), in Routine Clinical Practice: A Multinational Non-Interventional Study.

BACKGROUND: The Clinical COPD Questionnaire (CCQ) is a simple patient-reported tool to measure clinical control of chronic obstructive pulmonary disease (COPD). OBJECTIVE: This open-label, single-arm, non-interventional study (NCT03663569) investigated changes in CCQ score during treatment with tiotropium/olodaterol in clinical practice. METHODS: Data were included from consenting COPD patients, enrolled in Bulgaria, Czech Republic, Hungary, Israel, Lithuania, Poland, Romania, Russia, Slovenia, Switzerland and Ukraine, who were receiving a new prescription for tiotropium/olodaterol according to the treating physician in a real-world environment. The primary endpoint was the occurrence of therapeutic success, defined as a 0.4-point decrease in CCQ score after treatment with tiotropium/olodaterol for approximately 6 weeks. RESULTS: Overall, 4819 patients were treated; baseline and Week 6 CCQ scores were available for 4700 patients, mostly classified as Global Initiative for Chronic Obstructive Lung Disease (GOLD) B (51.6%) or D (42.7%). After 6 weeks' treatment, 81.4% (95% confidence interval [95% CI] 80.24-82.49) of patients achieved therapeutic success; mean improvement in overall CCQ score was 1.02 points (95% CI 1.00-1.05). Improved CCQ score was seen in 92.2% of patients (95% CI 91.43-92.98), 2.5% had no change and 5.3% showed a worsening. When stratified by prior treatment, the greatest benefit was seen in treatment-naïve patients, with 85.7% achieving therapeutic success, compared with 79.5% of those pretreated with long-acting ß2-agonist (LABA)/inhaled corticosteroid (ICS) and 74.2% of those pretreated with LABA or long-acting muscarinic antagonist (LAMA) monotherapy. Overall, rescue medication decreased by 1.25 puffs/day (95% CI 1.19-1.31) versus baseline. In total, 29 patients (0.6%) reported drug-related adverse events and 7 patients reported serious adverse events (0.15%). CONCLUSION: In 4700 COPD patients, 6 weeks' treatment with tiotropium/olodaterol, as initial treatment or follow-up to LAMA or LABA monotherapy or LABA/ICS, improved CCQ and decreased rescue medication use. The adverse event profile was consistent with the known safety profile of tiotropium/olodaterol.

Successful treatment of diffuse pulmonary lymphangiomatosis with sirolimus.

Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease characterized by uncontrolled proliferation of anastomosing lymphatic channels in the lungs, pleura and mediastinum. Several palliative treatment options have been suggested for this condition, such as surgical interventions, radiotherapy and systemic medications. However, the existing treatment modalities yield inconsistent results, and their use is often limited by toxic side effects. The aim of this case report is to demonstrate the diagnostic challenges of a rare disease and improvement in the condition of a DPL patient treated with sirolimus. A 27-year-old man presented to the pulmonologist with exertional dyspnea, chronic cough and intermittent hemoptysis. Upon medical investigation, a chest computed tomography (CT) scan revealed soft tissue masses infiltrating the mediastinum and bilateral interlobular septal thickening. A surgical biopsy was performed, and pathological tissue analysis showed findings consistent with the diagnosis of DPL. Treatment with sirolimus was initiated, maintaining trough concentrations between 10 and 15 ng/ml. At 21 months of treatment, the patient reported reduced symptoms of cough and dyspnea. A CT scan showed decreased interstitial thickening and reduced infiltrations in the mediastinum. Moreover, pulmonary function tests revealed a significant increase in FEV1 and FVC. The authors believe this is the first article reporting pulmonary function improvement in an adult DPL patient treated with sirolimus. Therefore, sirolimus therapy should be considered for DPL patients as it may be effective in improving their condition and preventing disease progression.

Pulmonary artery coil embolisation prevented tumour progression in a patient with advanced squamous cell lung carcinoma.

Background: Squamous cell lung carcinoma (SqCLC) is a type of non-small-cell lung cancer, accounting for 25-30% of all lung cancer cases with a median advanced stage survival of 8-11 months. Here we present a rare case of long-term survival with metastatic SqCLC following coil embolisation of the right pulmonary artery.Case presentation: The 49-year-old patient was diagnosed with stage IV (cT4N3M1) SqCLC in 2007 due to a biopsy-proven central malignant tumour in the right lung and bilateral mediastinal lymphadenopathy. A magnetic resonance imaging scan also revealed a metastatic lesion in the liver. Soon after the diagnosis, the patient experienced pulmonary haemorrhage, which was managed by obturating the intermediate bronchus and performing coil embolisation of the right pulmonary artery. The patient also received chemotherapy in 2007 and 2009 without radiological changes. At three different time points in years 2010-2019, biopsies of the primary tumour were taken. All showed dense connective tissue with no indication of cancer growth. In 2020, a positron emission tomography scan showed no pathological metabolic activity in the lungs and liver. Currently, the patient remains in a stable clinical condition with a good performance status.Conclusion: The long-term clinical benefit indicates a direct effect of coil embolisation on tumour progression. We suggest that coil embolisation of tumour-feeding arteries could be considered as a potential treatment method for patients with SqCLC.

Insights to correlations and discrepancies between impaired lung function and heart failure in Eisenmenger patients.

Impaired lung function and spirometric signs of airway obstruction without common risk factors for chronic obstructive pulmonary disease could be found in patients with Eisenmenger syndrome. This study aimed to analyse the association between lung function parameters and disease severity (including heart failure markers, associated congenital heart defect) as well as the possible reasons for airflow obstruction in Eisenmenger syndrome. The data of 25 patients with Eisenmenger syndrome were retrospectively evaluated. The patients were divided into groups according to airflow obstruction and a type of congenital heart defect. Airflow obstruction was found in nearly third (32%) of our cases and was associated with older age and worse survival. No relation was found between airway obstruction, B-type natriuretic peptide level, complexity of congenital heart defect and bronchial compression. Most of the patients (88%) had gas diffusion abnormalities. A weak negative correlation was noticed between gas diffusion (diffusing capacity of the lung for carbon monoxide) and B-type natriuretic peptide level (r = -0.437, p = 0.033). Increased residual volume was associated with higher mortality (p = 0.047 and p = 0.021, respectively). A link between B-type natriuretic peptide and lung diffusion, but not airway obstruction, was found. Further research and larger multicentre studies are needed to evaluate the importance of pulmonary function parameters and mechanisms of airflow obstruction in Eisenmenger syndrome.

Different course of silicosis in four brothers of one family.

Silicosis remains a common occupational respiratory disease. Even in this era of highly sophisticated hygiene in European countries, new occupational cases of silicosis continue to be reported. Four cases of silicosis which developed after a relatively short occupational exposure to respirable silica among the members of one family are described. Four young men worked illegally abroad in mining in one of European countries. All of them were employed together in the same working conditions. One of the brothers died due to the acute form of the disease (lipoproteinosis). Two of the brothers suffered from simple nodular silicosis, and the fourth brother developed very early nodular silicosis and small airway dust disease. A one year follow-up revealed moderate/severe worsening of the disease in all surviving brothers.

Levels of CD4+ CD25+ T regulatory cells in bronchial mucosa and peripheral blood of chronic obstructive pulmonary disease indicate involvement of autoimmunity mechanisms.

INTRODUCTION: Many theories have been proposed to explain pathogenesis of COPD; however, remains unclear why the majority of smokers (~80%) do not develop COPD, or only develop a mild disease. To explore if COPD has an autoimmune component, the role of T regulatory lymphocytes (Tregs) in the lung tissue of COPD patients is of crucial importance. MATERIAL AND METHODS: Bronchial tissue biopsy samples were prospectively collected from 64 patients (39 COPD and 25 controls - 15 smokers and 10 non-smokers). The patients with COPD were subdivided into mild/moderate (GOLD stage I-II) and severe/very severe (GOLD stage III-IV) groups. Digital image analysis was performed to estimate densities of CD4+ CD25+ cell infiltrates in double immunohistochemistry slides of the biopsy samples. Blood samples were collected from 42 patients (23 COPD and 19 controls) and tested for CD3+ CD4+ CD25+ bright lymphocytes by flow cytometry. RESULTS: The number of intraepithelial CD4+ CD25+ lymphocytes mm-2 epithelium was significantly lower in the severe/very severe COPD (GOLD III-IV) group as well as in the control non-smokers (NS) group (p < 0,0001). Likewise, the absolute number of Treg (CD3+ CD4+ CD25+ bright) cells in the peripheral blood samples was significantly different between the four groups (p = 0.032). The lowest quantity of Treg cells was detected in the severe/very severe COPD and healthy non-smokers groups. CONCLUSION: Our findings suggest that severe COPD is associated with lower levels of Tregs in the blood and bronchial mucosa, while higher Tregs levels in the smokers without COPD indicate potential protective effect of Tregs against developing COPD.

Relationships between pulmonary function test parameters and quantitative computed tomography measurements of emphysema in subjects with chronic obstructive pulmonary disease.

OBJECTIVE: CT is able to precisely define the pathological process in COPD. There are a number of previous articles discussing the distribution of emphysema and its connection with pulmonary function tests. However, the results obtained by the researchers are not identical. PURPOSE: To assess relationships between emphysema and pulmonary function test parameters in COPD patients. MATERIALS AND METHODS: Fifty-nine patients diagnosed to have COPD underwent chest CT examinations and pulmonary function tests.For the quantitative assessment, percentages of low attenuation volume LAV 950 HU (%) of a both lungs, the right lung, the left lung, and each lobe were obtained. Quantitative CT measurements were compared with forced expiratory volume in 1 s (FEV1), the ratio of FEV1 to forced vital capacity (FEV1/FVC), the diffusing capacity for carbon monoxide (DLco) and total lung capacity (TLC). RESULTS: Except for the right middle lobe and the right upper lobe, respectively, all the quantitative CT measurements showed weak to moderate negative correlations with diffusing capacity (DLco) (r = -0.35 to -0.61, p < 0.05) and weak positive correlations with TLC (r = 0.34 to 0.44, p < 0.05). Group analysis indicated that LAV-950 HU (%) values of both lungs, right lung, left lung, and each lobe, except for right middle lobe, were increased in patients with GOLD stages 3 and 4 of COPD compared to GOLD stages 1 and 2 (p < 0.05). CONCLUSION: CT measurements of emphysema are significantly related to pulmonary function tests results, particularly DLco.

A fast and fatal course of bronchiectasis: an unusual rare expression of chronic graft versus host disease. A case report.

Introduction. We report a case of a patient with acute myeloid leukaemia whose treatment with bone marrow transplantation (BMT) was followed by chronic graft versus host disease (GVHD) with lung involvement and bronchiectasis. This report illustrates an unusual course of a fast progression of the bronchiectasis due to BMT. Case description. A 33-year-old female was diagnosed with acute myeloid leukaemia. An allogeneic BMT was performed. One month after the transplantation, acute GVHD with skin involvement occurred. Treatment with prednisolone and mycophenolate mofetil (MMF) has been started. Nine months later, the patient was examined by a pulmonologist due to progressive dyspnoea. A pulmonary computed tomography (CT) scan showed normal parenchyma of the lungs and no changes to the bronchi. A CT scan performed 7 months later revealed bronchiectasis for the first time. No clinical response was associated with the treatment and the patient's respiratory status progressively deteriorated. During the final hospitalization, a CT scan performed 1 year later revealed huge cystic bronchiectasis in both lungs. Despite the prophylaxis and treatment of GVHD and aggressive antimicrobial therapy, the patient died one year after the diagnosis of bronchiectasis. Conclusions. This case demonstrates that a fast and fatal course of bronchiectasis, that occurs after BMT, should always be considered as a possible manifestation of chronic graft versus host disease (cGVHD) following allogeneic BMT.

Fatal pulmonary complication during induction therapy in a patient with ANCA-associated vasculitis.

ANCA-associated vasculitis (AAV) is an inflammatory systemic disorder affecting small to medium sized vessels and likely leading to any organ dysfunction. Adequate treatment is important to avoid mortality or severe organ damage. In most cases initial treatment (induction therapy) allows to achieve remission. Induction therapy leads to immunosuppression and may cause severe infections. However, in vasculitis patients even an intensive immunosuppressive therapy is rarely complicated by an invasive fungal infection. We present a case in a 29-year old male patient with newly diagnosed AAV. He suffered a fatal pulmonary complication of the induction immunosuppressive treatment. Pathological (infectious) changes in the lungs were misinterpreted as progression of the vasculitis and he died due to disseminated angioinvasive aspergillosis. A clinical course, imaging and histopathology of this case are described and discussed.

Cardiorespiratory parameters of exercise capacity in a healthy Lithuanian population: the pilot study.

INTRODUCTION: The normative values of exercise capacity used for the interpretation of exercise testing results are influenced by a variety of internal and external factors specific to certain populations. Therefore, in clinical practice it is recommended that population-specific reference values be employed. Cardiorespiratory fitness norms have not yet been established for a healthy Lithuanian population over a wide age span. The purpose of the present study was to determine the main cardiorespiratory fitness parameters for healthy adults living in Lithuania and to compare these parameters with the reference values established for different foreign populations. METHODS: This was a cross-sectional, community-based study involving 168 healthy adults aged from 20 to 60 years who were randomly selected from the general population. All subjects performed a progressive incremental exercise test on the cycle ergometer. The main cardiorespiratory fitness parameters analysed were peak oxygen consumption (VO2peak), ventilatory anaerobic threshold, and peak heart rate (HRpeak). RESULTS: The average estimated VO2peak was 35.02 ± 7.37 mL.kg(-1).min(-1) for men and 28.27 ± 6.33 mL.kg(-1).min(-1) for women. According to the results presented by other authors, this parameter is approximately 9-22% lower compared to other populations in all age groups, with the exception of the 20-29 year old group who alone satisfied fair aerobic fitness criteria. The average age-related decline in VO2peak was 0.016 L.min(-1) per year for men and 0.011 L.min(-1) per year for women. However, age itself explained only 12-14% of the variance. After VO2peak was adjusted relative to body mass, the difference in the decline between men and women remained insignificant: VO2peak decrease was 0.34 mL.kg(-1).min(-1) per year for men (coefficient of determination R(2) 0.250) and 0.32 mL.kg(-1).min(-1) per year for women (R(2) 0.330). A decline in peak heart rate of approximately 9 beats per minute was observed in each following age decade, which was well explained by the advancing age (R(2) 0.512 for men and R(2) 0.484 for women). CONCLUSIONS: Cardiorespiratory fitness parameters estimated for healthy adults living in Lithuania appeared to be lower compared to different foreign populations, despite the relatively similar general trends in the age-related decline in exercise capacity. Exercise testing laboratories and rehabilitation clinics in Lithuania may use these results in clinical practice when evaluating patients' exercise capacity, or as a promotional tool for physical activity in the general public.