Nikoleta Bizymi
Current Organizations / Institutes
The organizations/institutes from the scholar's latest article
Previous Organizations / Institutes
The organizations / institutes from the scholar's other articles in the past ten years
Research Subjects
Nikoleta Bizymi has a total of 221 co-authors. Here are the top co-authors by number of shared publications:
- Helen A. Papadaki
Hemopoiesis Research Laboratory, School of Medicine University of Crete Heraklion Greece
9 shared publications - Andreas M. Matthaiou
15th Department of Respiratory Medicine, Sotiria Thoracic Diseases General Hospital of Athens, Athens, Greece
8 shared publications Nikolaos Tzanakis
Department of Respiratory Medicine, University Hospital of Heraklion & Laboratory of Molecular and Cellular Pneumonology, School of Medicine, University of Crete, Heraklion, Greece
8 shared publicationsAndreas M. Matthaiou
15th Department of Respiratory Medicine, Sotiria Thoracic Diseases General Hospital of Athens, Athens, Greece
5 shared publicationsKaterina M. Antoniou
Department of Respiratory Medicine, University Hospital of Heraklion & Laboratory of Molecular and Cellular Pneumonology, School of Medicine, University of Crete, Heraklion, Greece
5 shared publicationsGeorgios Pitsidianakis
Department of Respiratory Medicine, University Hospital of Heraklion & Laboratory of Molecular and Cellular Pneumonology, School of Medicine, University of Crete, Heraklion, Greece
4 shared publications
Latest Publications
- 5 September 2026
- Published by European Respiratory Society (ERS)
- Vol. 68 (suppl 70) , PA4707
Predominance of inflammatory monocyte subsets in the peripheral blood of patients with stable bronchiectasis
Background: Bronchiectasis is a condition characterised by sustained neutrophilic inflammation in the context of the vicious vortex. The possible involvement of the monocyte subsets in the pathophysiology of the immune dysregulation of bronchiectasis has not been studied yet. Aim and Objectives: The aim of this study was to evaluate the circulating monocyte subsets in patients with stable bronchiectasis, i.e., classical (CD14brightCD16–) displaying phagocytic properties, intermediate (CD14brightCD16+) producing inflammatory cytokines, and non-classical (CD14dimCD16+) subsets displaying proinflammatory and antimicrobial functions. Methods: The proportion of circulating monocyte subsets was evaluated in 15 patients and 15 age- and sex-matched healthy subjects by flow cytometry based on their relative CD14 and CD16 surface expression, using a panel of directly conjugated monoclonal antibodies and a whole blood lysis technique. Statistical analysis was performed with the Mann–Whitney t-test. Results: The patients displayed lower total monocyte counts compared to controls (p <0.0001), attributed to a decreased proportion of classical subsets (median 80% / interquartile range 6.7% vs. 91.2%/10.8%, p=0.0015). On the contrary, increased counts of intermediate and non-classical subsets were found in patients (4%/2.2% and 8.3%/8.7%, respectively) compared to controls (3.5%/4.2% and 3.1%/3%, respectively), failing to reach statistical significance probably due to the small sample size. Conclusions: Our preliminary data show that monocyte subset counts are altered in bronchiectasis, contributing to immune dysregulation. Further studies with larger sample sizes and mechanistic assays are needed to better characterise them in bronchiectasis. *NB, APB & AMM: equally contributing authors.
- 5 September 2026
- Published by European Respiratory Society (ERS)
- Vol. 68 (suppl 70) , PA4707
Predominance of inflammatory monocyte subsets in the peripheral blood of patients with stable bronchiectasis
Background: Bronchiectasis is a condition characterised by sustained neutrophilic inflammation in the context of the vicious vortex. The possible involvement of the monocyte subsets in the pathophysiology of the immune dysregulation of bronchiectasis has not been studied yet. Aim and Objectives: The aim of this study was to evaluate the circulating monocyte subsets in patients with stable bronchiectasis, i.e., classical (CD14brightCD16–) displaying phagocytic properties, intermediate (CD14brightCD16+) producing inflammatory cytokines, and non-classical (CD14dimCD16+) subsets displaying proinflammatory and antimicrobial functions. Methods: The proportion of circulating monocyte subsets was evaluated in 15 patients and 15 age- and sex-matched healthy subjects by flow cytometry based on their relative CD14 and CD16 surface expression, using a panel of directly conjugated monoclonal antibodies and a whole blood lysis technique. Statistical analysis was performed with the Mann–Whitney t-test. Results: The patients displayed lower total monocyte counts compared to controls (p <0.0001), attributed to a decreased proportion of classical subsets (median 80% / interquartile range 6.7% vs. 91.2%/10.8%, p=0.0015). On the contrary, increased counts of intermediate and non-classical subsets were found in patients (4%/2.2% and 8.3%/8.7%, respectively) compared to controls (3.5%/4.2% and 3.1%/3%, respectively), failing to reach statistical significance probably due to the small sample size. Conclusions: Our preliminary data show that monocyte subset counts are altered in bronchiectasis, contributing to immune dysregulation. Further studies with larger sample sizes and mechanistic assays are needed to better characterise them in bronchiectasis. *NB, APB & AMM: equally contributing authors.
- 5 September 2026
- Published by European Respiratory Society (ERS)
- Vol. 68 (suppl 70) , PA1833
Improving health literacy on vaccination against respiratory pathogens among frail older adults in Greece: a Healthy Lungs for Life campaign action
Background: Respiratory infections disproportionately affect frail older adults and may lead to severe morbidity and mortality. Despite the availability of effective vaccines, uptake remains suboptimal due to misinformation, limited awareness, and access barriers among elderly individuals and caregivers. Action: In December 2023, a non-profit initiative supported by the European Lung Foundation Healthy Lungs for Life campaign was implemented in nursing homes and public elderly care centres in Attica, Greece, aiming to increase awareness of respiratory infection risks and promote vaccination. Implementation: Coordinating staff of selected nursing homes were informed about recommended vaccinations for prevention of respiratory infections. In collaboration with local authorities, interactive small-group educational events were organised in public elderly care centres using plain-language audiovisual presentations followed by discussion, focusing on vaccination against Streptococcus pneumoniae, Bordetella pertussis, influenza virus, RSV, VZV, and SARS-CoV-2. Outcomes: More than 100 elderly individuals and caregivers participated and actively engaged, demonstrating improved understanding of infection risks and vaccine benefits. Personalised guidance on accessing healthcare services, electronic prescriptions, and vaccination pathways was provided, facilitating adherence to the national immunisation programme and addressing practical barriers to uptake. Conclusions: This Healthy Lungs for Life campaign action effectively targeted a high-risk population by combining education with practical vaccine access support, enhancing health literacy. Similar outreach initiatives may strengthen respiratory infection prevention and improve vaccination uptake among frail elderly populations.
- 5 September 2026
- Published by European Respiratory Society (ERS)
- Vol. 68 (suppl 70) , PA1833
Improving health literacy on vaccination against respiratory pathogens among frail older adults in Greece: a Healthy Lungs for Life campaign action
Background: Respiratory infections disproportionately affect frail older adults and may lead to severe morbidity and mortality. Despite the availability of effective vaccines, uptake remains suboptimal due to misinformation, limited awareness, and access barriers among elderly individuals and caregivers. Action: In December 2023, a non-profit initiative supported by the European Lung Foundation Healthy Lungs for Life campaign was implemented in nursing homes and public elderly care centres in Attica, Greece, aiming to increase awareness of respiratory infection risks and promote vaccination. Implementation: Coordinating staff of selected nursing homes were informed about recommended vaccinations for prevention of respiratory infections. In collaboration with local authorities, interactive small-group educational events were organised in public elderly care centres using plain-language audiovisual presentations followed by discussion, focusing on vaccination against Streptococcus pneumoniae, Bordetella pertussis, influenza virus, RSV, VZV, and SARS-CoV-2. Outcomes: More than 100 elderly individuals and caregivers participated and actively engaged, demonstrating improved understanding of infection risks and vaccine benefits. Personalised guidance on accessing healthcare services, electronic prescriptions, and vaccination pathways was provided, facilitating adherence to the national immunisation programme and addressing practical barriers to uptake. Conclusions: This Healthy Lungs for Life campaign action effectively targeted a high-risk population by combining education with practical vaccine access support, enhancing health literacy. Similar outreach initiatives may strengthen respiratory infection prevention and improve vaccination uptake among frail elderly populations.
- Open Access
- 8 June 2026
- Published by BMJ in BMJ Open Respiratory Research
- Vol. 13 (1) , e003482
Application of the Idiopathic Pulmonary Fibrosis Patient-Reported Outcome Measure (IPF-PROM) scale to a Greek IPF patient population and its interrelation with depressive symptoms and health-related quality of life
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease which affects physical and mental well-being. The IPF Patient-Reported Outcome Measure (IPF-PROM) scale is a validated and reliable tool for the self-report of physical and psychological well-being in IPF. Aims and objectives: This study aimed to validate a Greek version of the IPF-PROM scale and further investigate its correlation with clinical features of IPF patients and its interrelation with depressive symptoms and health-related quality of life (HRQoL). Methods: This was a two-centre, observational, cross-sectional study, in which IPF patients completed three scales: IPF-PROM, Patient Health Questionnaire-9 (PHQ-9), an index of depressive symptoms, and Health Survey Questionnaire Short Form-12 (SF-12), an index of HRQoL, at the IPF Outpatient Clinics of two University Hospitals in Greece during 2023–2024. Logistic regression analysis was conducted to assess severe status of IPF-PROM compared with mild/moderate status. Results: The study involved 136 IPF patients (87.9% males) with a mean age of 73.5±7.9 years. Patients were classified overall with moderate disease according to IPF-PROM mean scores (41.7±31.3), particularly in the combined Breathlessness/Fatigue and the Psychological well-being components (40.1 and 43.8, respectively, p>0.050). Patients with severe symptoms, as measured by IPF-PROM, scored higher levels of depressive symptoms on PHQ-9 compared with those with moderate or mild symptoms (17.1, 12.3 and 1.4, respectively, p<0.001) and lower levels of HRQoL in physical (32.6, 34.7 and 47.9, p<0.001) and mental health on SF-12 (23.9, 34.5 and 50.3, p<0.001). Patients with incrementally higher levels of oxygen saturation had lower odds for severe health status according to IPF-PROM (OR=0.83, p=0.018). Conclusions: A significant percentage of IPF patients present with impaired health status and symptoms suggestive of depression. The IPF-PROM scale represents a useful tool that may predict impairment of mental health and HRQoL in IPF, with potential utility for clinical practice and research.
- 8 June 2026
- Published by BMJ in BMJ Open Respiratory Research
- Vol. 13 (1) , e003482
Open AccessApplication of the Idiopathic Pulmonary Fibrosis Patient-Reported Outcome Measure (IPF-PROM) scale to a Greek IPF patient population and its interrelation with depressive symptoms and health-related quality of life
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease which affects physical and mental well-being. The IPF Patient-Reported Outcome Measure (IPF-PROM) scale is a validated and reliable tool for the self-report of physical and psychological well-being in IPF. Aims and objectives: This study aimed to validate a Greek version of the IPF-PROM scale and further investigate its correlation with clinical features of IPF patients and its interrelation with depressive symptoms and health-related quality of life (HRQoL). Methods: This was a two-centre, observational, cross-sectional study, in which IPF patients completed three scales: IPF-PROM, Patient Health Questionnaire-9 (PHQ-9), an index of depressive symptoms, and Health Survey Questionnaire Short Form-12 (SF-12), an index of HRQoL, at the IPF Outpatient Clinics of two University Hospitals in Greece during 2023–2024. Logistic regression analysis was conducted to assess severe status of IPF-PROM compared with mild/moderate status. Results: The study involved 136 IPF patients (87.9% males) with a mean age of 73.5±7.9 years. Patients were classified overall with moderate disease according to IPF-PROM mean scores (41.7±31.3), particularly in the combined Breathlessness/Fatigue and the Psychological well-being components (40.1 and 43.8, respectively, p>0.050). Patients with severe symptoms, as measured by IPF-PROM, scored higher levels of depressive symptoms on PHQ-9 compared with those with moderate or mild symptoms (17.1, 12.3 and 1.4, respectively, p<0.001) and lower levels of HRQoL in physical (32.6, 34.7 and 47.9, p<0.001) and mental health on SF-12 (23.9, 34.5 and 50.3, p<0.001). Patients with incrementally higher levels of oxygen saturation had lower odds for severe health status according to IPF-PROM (OR=0.83, p=0.018). Conclusions: A significant percentage of IPF patients present with impaired health status and symptoms suggestive of depression. The IPF-PROM scale represents a useful tool that may predict impairment of mental health and HRQoL in IPF, with potential utility for clinical practice and research.
- Open Access
- 28 April 2026
- Published by MDPI AG in Journal of Clinical Medicine
- Vol. 15 (9) , 3383
Plasma Fibrinogen-to-Fractional Exhaled Nitric Oxide Ratio (FFR) as an Emerging Biomarker in Bronchiectasis
Background and Aims: Plasma fibrinogen and fractional exhaled nitric oxide (FeNO) reflect neutrophilic and eosinophilic airway inflammation, respectively, and are associated with disease activity and severity in different directions in bronchiectasis. This study aimed to concurrently investigate fibrinogen and FeNO and further evaluate the clinical importance of fibrinogen-to-FeNO ratio (FFR) as a composite biomarker in bronchiectasis. Methods: This was a two-centre, observational, cross-sectional study involving stable bronchiectasis patients. Fibrinogen, FeNO, and the ratio of their normalised values (FFR) were investigated in relation to clinical indicators of disease activity and severity, including respiratory symptoms, inflammatory markers, pulmonary function, radiological extent, airway infection, severity scores, and patient-reported outcomes. Results: FFR was correlated with both circulating neutrophils (r = 0.36, p = 0.04) and eosinophils (r = −0.39, p = 0.03) and, more strongly compared to fibrinogen and FeNO, with the percentage of predicted forced expiratory volume in the 1st second (r = −0.61, p < 0.001). Interestingly, only FFR was found to be higher in patients with Pseudomonas aeruginosa isolation in respiratory secretions (p < 0.01). In receiver operating characteristic curves, FFR showed good discriminatory ability to differentiate patients with any level (AUC: 0.80, 95% CI: 0.64–0.96) or a severe level (AUC: 0.83, 95% CI: 0.64–1.00) of pulmonary functional impairment and patients with severe disease (AUC: 0.78, 95% CI: 0.62–0.94). Conclusions: FFR emerges as a candidate biomarker capturing the balance between neutrophilic and eosinophilic inflammation and the net disease activity and severity in bronchiectasis.
- 28 April 2026
- Published by MDPI AG in Journal of Clinical Medicine
- Vol. 15 (9) , 3383
Open AccessPlasma Fibrinogen-to-Fractional Exhaled Nitric Oxide Ratio (FFR) as an Emerging Biomarker in Bronchiectasis
Background and Aims: Plasma fibrinogen and fractional exhaled nitric oxide (FeNO) reflect neutrophilic and eosinophilic airway inflammation, respectively, and are associated with disease activity and severity in different directions in bronchiectasis. This study aimed to concurrently investigate fibrinogen and FeNO and further evaluate the clinical importance of fibrinogen-to-FeNO ratio (FFR) as a composite biomarker in bronchiectasis. Methods: This was a two-centre, observational, cross-sectional study involving stable bronchiectasis patients. Fibrinogen, FeNO, and the ratio of their normalised values (FFR) were investigated in relation to clinical indicators of disease activity and severity, including respiratory symptoms, inflammatory markers, pulmonary function, radiological extent, airway infection, severity scores, and patient-reported outcomes. Results: FFR was correlated with both circulating neutrophils (r = 0.36, p = 0.04) and eosinophils (r = −0.39, p = 0.03) and, more strongly compared to fibrinogen and FeNO, with the percentage of predicted forced expiratory volume in the 1st second (r = −0.61, p < 0.001). Interestingly, only FFR was found to be higher in patients with Pseudomonas aeruginosa isolation in respiratory secretions (p < 0.01). In receiver operating characteristic curves, FFR showed good discriminatory ability to differentiate patients with any level (AUC: 0.80, 95% CI: 0.64–0.96) or a severe level (AUC: 0.83, 95% CI: 0.64–1.00) of pulmonary functional impairment and patients with severe disease (AUC: 0.78, 95% CI: 0.62–0.94). Conclusions: FFR emerges as a candidate biomarker capturing the balance between neutrophilic and eosinophilic inflammation and the net disease activity and severity in bronchiectasis.
- Open Access
- 23 April 2026
- Published by European Respiratory Society (ERS) in ERJ Open Research
- Vol. 12 (4)
ERS Congress 2025: highlights from the Interstitial Lung Diseases Assembly
Shareable abstract A summary of the interstitial lung disease presentations and posters from the ERS 2025 meeting
- 23 April 2026
- Published by European Respiratory Society (ERS) in ERJ Open Research
- Vol. 12 (4)
Open AccessERS Congress 2025: highlights from the Interstitial Lung Diseases Assembly
Shareable abstract A summary of the interstitial lung disease presentations and posters from the ERS 2025 meeting
- Open Access
- 20 April 2026
- Published by Association of Basic Medical Sciences of FBIH in Biomolecules and Biomedicine
- Vol. 26 (10) , 1902-1912
Pulmonary actinomycosis and pulmonary nocardiosis mimicking lung cancer: A case series and narrative review
Pulmonary actinomycosis and pulmonary nocardiosis are rare chronic respiratory infections that may closely resemble lung cancer due to their overlapping clinical, radiological, and bronchoscopic characteristics. This hybrid case series and narrative review aims to elucidate the presenting patterns, diagnostic pathways, management strategies, and practical implications of these infections in the differential diagnosis of lung cancer. We analyzed four consecutive cases managed at the Sotiria Thoracic Diseases General Hospital in Athens from 2021 to 2025, supplemented by eight additional cases identified through a review of Greek grey literature from the same period. In the 12 cases reviewed, patients commonly presented with cough, dyspnea, fever, hemoptysis, malaise, and weight loss. Chest imaging frequently revealed consolidation, cavitation, atelectasis, nodules, or mediastinal lymphadenopathy, often prompting an oncological evaluation. Bronchoscopic findings occasionally mimicked malignant submucosal spread, and increased fluorodeoxyglucose uptake added to the diagnostic uncertainty. Notably, one case exhibited coexistence of pulmonary actinomycosis with lung adenocarcinoma. Microbiological and histopathological evaluations confirmed the diagnoses, and most patients showed clinical and radiological improvement following prolonged targeted antimicrobial therapy. Given these findings, pulmonary actinomycosis and nocardiosis should be considered in patients with suspected lung cancer, particularly when infectious features coexist with mass-like or cavitary lesions. Careful tissue sampling, specialized microbiological testing, and structured follow-up are essential for excluding occult or coexisting malignancy.
- 20 April 2026
- Published by Association of Basic Medical Sciences of FBIH in Biomolecules and Biomedicine
- Vol. 26 (10) , 1902-1912
Open AccessPulmonary actinomycosis and pulmonary nocardiosis mimicking lung cancer: A case series and narrative review
Pulmonary actinomycosis and pulmonary nocardiosis are rare chronic respiratory infections that may closely resemble lung cancer due to their overlapping clinical, radiological, and bronchoscopic characteristics. This hybrid case series and narrative review aims to elucidate the presenting patterns, diagnostic pathways, management strategies, and practical implications of these infections in the differential diagnosis of lung cancer. We analyzed four consecutive cases managed at the Sotiria Thoracic Diseases General Hospital in Athens from 2021 to 2025, supplemented by eight additional cases identified through a review of Greek grey literature from the same period. In the 12 cases reviewed, patients commonly presented with cough, dyspnea, fever, hemoptysis, malaise, and weight loss. Chest imaging frequently revealed consolidation, cavitation, atelectasis, nodules, or mediastinal lymphadenopathy, often prompting an oncological evaluation. Bronchoscopic findings occasionally mimicked malignant submucosal spread, and increased fluorodeoxyglucose uptake added to the diagnostic uncertainty. Notably, one case exhibited coexistence of pulmonary actinomycosis with lung adenocarcinoma. Microbiological and histopathological evaluations confirmed the diagnoses, and most patients showed clinical and radiological improvement following prolonged targeted antimicrobial therapy. Given these findings, pulmonary actinomycosis and nocardiosis should be considered in patients with suspected lung cancer, particularly when infectious features coexist with mass-like or cavitary lesions. Careful tissue sampling, specialized microbiological testing, and structured follow-up are essential for excluding occult or coexisting malignancy.
- 19 March 2026
- Published by European Respiratory Society (ERS)
- Vol. 12 (suppl 18) , PS232
Transcriptomic analysis of circulating myeloid-derived suppressor cells (MDSCs) and T cells in bronchiectasis
Background: Bronchiectasis involves upregulation of circulating MDSCs capable of suppressing T-cell responses. In this study, we evaluate MDSC and T-cell transcriptomes to better characterise their interactions. Methods: We studied the transcriptome of immunomagnetically isolated blood MDSCs and T cells from 3 patients and 3 healthy controls. RNA was extracted, and libraries were prepared using the 3’ Quant-seq FWD strand-specific protocol (Lexogen), then sequenced as 75 bp single-end reads (NextSeq500). Bioinformatic analysis (mapping to the genome, read counting/normalisation per gene, differential expression) was performed using R, Bioconductor packages, and Metascape. Results: Differentially expressed genes in patient MDSCs and T cells (Fig. 1a) were related to diverse inflammatory conditions (e.g., pneumonitis, bacterial infections, tuberculosis) according to DisGeNET. Enrichment analysis revealed complex protein-protein interactions (Fig. 1b). The enriched ontology clusters (Fig. 1c) reflected MDSC-mediated impaired immune response (e.g., response to bacterium, inflammatory response, neutrophil degranulation), and T-cell proliferation (e.g., cell cycle, DNA replication). Conclusion: Transcriptomics show complex innate and adaptive immunity interactions contributing to immune dysregulation and chronic airway infection in the context of the pathogenetic vicious vortex. *NB & AMM contributed equally. erjor;12/suppl_18/PS232/F1 F1 F1
- 19 March 2026
- Published by European Respiratory Society (ERS)
- Vol. 12 (suppl 18) , PS232
Transcriptomic analysis of circulating myeloid-derived suppressor cells (MDSCs) and T cells in bronchiectasis
Background: Bronchiectasis involves upregulation of circulating MDSCs capable of suppressing T-cell responses. In this study, we evaluate MDSC and T-cell transcriptomes to better characterise their interactions. Methods: We studied the transcriptome of immunomagnetically isolated blood MDSCs and T cells from 3 patients and 3 healthy controls. RNA was extracted, and libraries were prepared using the 3’ Quant-seq FWD strand-specific protocol (Lexogen), then sequenced as 75 bp single-end reads (NextSeq500). Bioinformatic analysis (mapping to the genome, read counting/normalisation per gene, differential expression) was performed using R, Bioconductor packages, and Metascape. Results: Differentially expressed genes in patient MDSCs and T cells (Fig. 1a) were related to diverse inflammatory conditions (e.g., pneumonitis, bacterial infections, tuberculosis) according to DisGeNET. Enrichment analysis revealed complex protein-protein interactions (Fig. 1b). The enriched ontology clusters (Fig. 1c) reflected MDSC-mediated impaired immune response (e.g., response to bacterium, inflammatory response, neutrophil degranulation), and T-cell proliferation (e.g., cell cycle, DNA replication). Conclusion: Transcriptomics show complex innate and adaptive immunity interactions contributing to immune dysregulation and chronic airway infection in the context of the pathogenetic vicious vortex. *NB & AMM contributed equally. erjor;12/suppl_18/PS232/F1 F1 F1
- 1 January 2026
- Published by American Physiological Society in American Journal of Physiology-Lung Cellular and Molecular Physiology
- Vol. 330 (1) , L33-L48
BALF gene expression profiling reveals a fibrosis associated innate immune activation bias in persistent post-COVID-19 ILD
Survivors of COVID-19 can experience long-term lung complications. We compared bronchoalveolar lavage fluid (BALF) cells from patients who recovered from COVID-19 lung injury and those who experienced significant long term residual damage. A prominent gene expression profile of increased monocyte chemotaxis coupled to decreased T cell activation was observed in persistent fibrotic post-COVID-19 ILD. These findings suggest that prolonged activation of innate immunity and subdued adaptive immune responses may be associated with persistent post-COVID-19 ILD and pulmonary fibrosis.
- 1 January 2026
- Published by American Physiological Society in American Journal of Physiology-Lung Cellular and Molecular Physiology
- Vol. 330 (1) , L33-L48
BALF gene expression profiling reveals a fibrosis associated innate immune activation bias in persistent post-COVID-19 ILD
Survivors of COVID-19 can experience long-term lung complications. We compared bronchoalveolar lavage fluid (BALF) cells from patients who recovered from COVID-19 lung injury and those who experienced significant long term residual damage. A prominent gene expression profile of increased monocyte chemotaxis coupled to decreased T cell activation was observed in persistent fibrotic post-COVID-19 ILD. These findings suggest that prolonged activation of innate immunity and subdued adaptive immune responses may be associated with persistent post-COVID-19 ILD and pulmonary fibrosis.
- Open Access
- 1 December 2025
- Published by MDPI AG in Journal of Personalized Medicine
- Vol. 15 (12) , 583
Birt–Hogg–Dubé Syndrome: A Mini Review of the Clinical Manifestations, Investigation, and Management
Birt–Hogg–Dubé (BHD) syndrome is a rare genetic disease, inherited in an autosomal dominant manner, that was first described in the mid-1970s and occurs due to pathogenic variants in the folliculin gene (FLCN) on chromosome 17p11.2. The syndrome has numerous clinical manifestations and primarily affects the lungs, kidneys, and skin. As far as the pulmonary features are concerned, more than 80% of patients appear to develop bilateral pulmonary cysts located in the lower lung zones, in the subpleural area, with cumulative risk of spontaneous pneumothorax depending on the number of cysts in the lungs. Another serious feature of the syndrome is the increased risk of renal cell carcinoma, which is often an incidental finding on screening or medical imaging. Cutaneous manifestations include benign fibrofolliculomas, trichodiscomas, and acrochordons (skin tags), which primarily affect the patients’ emotional status as a result of their cosmetic defects. BHD syndrome is generally an underdiagnosed condition due to the great variability of its clinical picture, thus highlighting the importance of genetic testing for FLCN mutations in suspected cases. The application of ERN GENTURIS guidelines in clinical practice can facilitate early, accurate diagnosis of the disease and optimal personalized management of the patients.
- 1 December 2025
- Published by MDPI AG in Journal of Personalized Medicine
- Vol. 15 (12) , 583
Open AccessBirt–Hogg–Dubé Syndrome: A Mini Review of the Clinical Manifestations, Investigation, and Management
Birt–Hogg–Dubé (BHD) syndrome is a rare genetic disease, inherited in an autosomal dominant manner, that was first described in the mid-1970s and occurs due to pathogenic variants in the folliculin gene (FLCN) on chromosome 17p11.2. The syndrome has numerous clinical manifestations and primarily affects the lungs, kidneys, and skin. As far as the pulmonary features are concerned, more than 80% of patients appear to develop bilateral pulmonary cysts located in the lower lung zones, in the subpleural area, with cumulative risk of spontaneous pneumothorax depending on the number of cysts in the lungs. Another serious feature of the syndrome is the increased risk of renal cell carcinoma, which is often an incidental finding on screening or medical imaging. Cutaneous manifestations include benign fibrofolliculomas, trichodiscomas, and acrochordons (skin tags), which primarily affect the patients’ emotional status as a result of their cosmetic defects. BHD syndrome is generally an underdiagnosed condition due to the great variability of its clinical picture, thus highlighting the importance of genetic testing for FLCN mutations in suspected cases. The application of ERN GENTURIS guidelines in clinical practice can facilitate early, accurate diagnosis of the disease and optimal personalized management of the patients.
- Top Cited PapersOpen Access
- 4 September 2025
- Published by European Respiratory Society (ERS) in European Respiratory Journal
- Vol. 67 (1) , 2402533
ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease
Background: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care. Methods: A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies. For both PICO and narrative questions, the Evidence to Decision framework was used to formulate the recommendations. Results: The task force committee concluded with recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). In four narrative questions, regarding screening and assessment of risk for ILD progression in MCTD, SjD and SLE and one PICO question regarding pirfenidone in CTD-ILD other than RA-ILD, the task force had insufficient evidence to support recommendations. Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice. Conclusions: We provide practical guidance by evidence-based recommendations to clinicians for each of the CTDs. In many cases there is low certainty or absence of evidence and we encourage further research to fill these gaps.
- 4 September 2025
- Published by European Respiratory Society (ERS) in European Respiratory Journal
- Vol. 67 (1) , 2402533
Top Cited PapersOpen AccessERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease
Background: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care. Methods: A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies. For both PICO and narrative questions, the Evidence to Decision framework was used to formulate the recommendations. Results: The task force committee concluded with recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). In four narrative questions, regarding screening and assessment of risk for ILD progression in MCTD, SjD and SLE and one PICO question regarding pirfenidone in CTD-ILD other than RA-ILD, the task force had insufficient evidence to support recommendations. Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice. Conclusions: We provide practical guidance by evidence-based recommendations to clinicians for each of the CTDs. In many cases there is low certainty or absence of evidence and we encourage further research to fill these gaps.