Pulmonary arterial hypertension (PAH) is rare disease that is categorized as idiopathic (IPAH) wh... more Pulmonary arterial hypertension (PAH) is rare disease that is categorized as idiopathic (IPAH) when no underlying cause can be identified. Lungs of most patients with IPAH contain increased numbers of T cells and dendritic cells (DCs), suggesting involvement of the immune system in its pathophysiology. However, our knowledge on circulating immune cells in IPAH is rather limited. We used flow cytometry to characterize peripheral blood DCs and T cells in treatment-naive IPAH patients, compared with connective-tissue disease-PAH (CTD-PAH) patients and healthy controls (HCs). At diagnosis, T-helper (Th) cells of IPAH patients were less capable of producing TNFα, IFNγ, IL-4 and IL-17 compared to HCs. IPAH patients showed a decreased frequency of Th2 cells and significantly enhanced expression of the CTLA4 checkpoint molecule in naive CD4+ T cells and both naive and memory CD8+ T cells. Frequencies and surface marker expression of circulating DCs and monocytes were essentially comparable ...
Introduction / Rationale Pulmonary Fibrosis (PF) not only has impact on physical but also on soci... more Introduction / Rationale Pulmonary Fibrosis (PF) not only has impact on physical but also on social and emotional well-being of patient and partner. In general, their needs in these domains are ill documented. The objective of this project was to interactively acquire knowledge about patient9s and partner9s perspectives on and preferences in care for social and emotional well-being. Methods During a pulmonary fibrosis patient meeting in our hospital we interviewed patients and partners using voting boxes. Participants could answer anonymously to the 28 questions posed. The system recorded the number of voters. The results were directly presented. Permission to use the data was obtained at forehand. Results 65 patients with PF and 63 partners were present. In brackets number of voters is shown. Self-reported diagnoses were Idiopathic PF (46), collagen vascular disease (9), exposure related (6) and unknown (3). 68% of patients (63) would prefer talking about end of life in an early st...
Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on... more Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on the premise that e-cigarettes are less harmful than tobacco. However, so far studies have not provided convincing evidence that using electronic cigarettes is a better method of smoking cessation than other methods such as motivational counselling, either with or without nicotine replacement therapy. In addition, there is an alarming growth in the popularity of vaping among young people who find e-cigarettes attractive. Recent studies show the harmful effects of the ingredients of the e-cigarette fluid have resulted in acute pulmonary complications such as organizing pneumonia, asthma attacks and diffuse alveolar bleeding. The long-term effects of e-cigarettes are largely unknown, but one doesn't need to be a soothsayer to predict that the long-term damage will be significant. In conclusion, as pulmonologists, we find it necessary to give a serious warning to the Dutch population an...
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease of unknown cause. IPF ... more Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease of unknown cause. IPF has a poor prognosis with a mean survival of 2 to 5 years after diagnosis. The diagnostic process is often complex and demands a multidisciplinary approach. To date, the only curative therapy available is lung transplant. New insights into the pathogenesis of IPF have brought about changes in standard treatment strategies. New drugs have recently become available and have been shown to slow down the decline in pulmonary function considerably and improve survival.
Introduction: Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease characterized by exc... more Introduction: Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease characterized by excess deposition and altered structure of extracellular matrix (ECM) in the lungs. The fibrotic ECM is paramount in directing resident cells toward a profibrotic phenotype. Collagens, an important part of the fibrotic ECM, have been shown to be structurally different in IPF. To further understand the disease to develop better treatments, the signals from the ECM that drive fibrosis need to be identified. Adipose tissue-derived stromal cell conditioned medium (ASC-CM) has demonstrated antifibrotic effects in animal studies but has not been tested in human samples yet. In this study, the collagen structural integrity in (fibrotic) lung tissue, its interactions with fibroblasts and effects of ASC-CM treatment hereon were studied. Methods: Native and decellularized lung tissue from patients with IPF and controls were stained for denatured collagen using a collagen hybridizing peptide. Primary ...
Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on... more Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on the premise that e-cigarettes are less harmful than tobacco. However, so far studies have not provided convincing evidence that using electronic cigarettes is a better method of smoking cessation than other methods such as motivational counselling, either with or without nicotine replacement therapy. In addition, there is an alarming growth in the popularity of vaping among young people who find e-cigarettes attractive. Recent studies show the harmful effects of the ingredients of the e-cigarette fluid have resulted in acute pulmonary complications such as organizing pneumonia, asthma attacks and diffuse alveolar bleeding. The long-term effects of e-cigarettes are largely unknown, but one doesn't need to be a soothsayer to predict that the long-term damage will be significant. In conclusion, as pulmonologists, we find it necessary to give a serious warning to the Dutch population an...
Background Recent studies have provided evidence for an important contribution of the immune syst... more Background Recent studies have provided evidence for an important contribution of the immune system in the pathophysiology of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). In this report, we investigated whether the inflammatory profile of pulmonary hypertension patients changes over time and correlates with patient WHO subgroups or survival. Methods 50 PAH patients (16 idiopathic (I)PAH, 24 Connective Tissue Disease (CTD)-PAH and 10 Congenital Heart Disease (CHD)-PAH), 37 CTEPH patients and 18 healthy controls (HCs) were included in the study. Plasma inflammatory markers at baseline and after 1-year follow-up were measured using ELISAs. Subsequently, correlations with hemodynamic parameters and survival were explored and data sets were subjected to unbiased multivariate analyses. Results At diagnosis, we found that plasma levels of interleukin-6 (IL-6) and the chemokines (C-X3-C) motif legend CXCL9 and CXCL13 in CTD-PAH patients we...
Background: The development of pulmonary hypertension (PH) is an important risk factor for early ... more Background: The development of pulmonary hypertension (PH) is an important risk factor for early mortality in sarcoidosis. The prevalence is of SAPH in Europe is largely unknown. Aim: To investigate the prevalence of and clinical parameters associated with SAPH in the Netherlands. Methods: We analyzed clinical data and transthoracic echocardiograms (TTEs), made routinely at our PH/Interstitial Lung Disease clinic, from consecutive patients. Sarcoidosis patients with reliable echocardiography, without left heart dysfunction, were included. An estimated systolic pulmonary artery pressure (sPAP) > 50 mmHg was considered PH. Possible PH was defined as sPAP 37-50 mmHg or sPAP ≤ 36 mmHg with signs of right ventricular dysfunction. Results: From 139 sarcoidosis patients, 130 were included with stage 0 (11), I (46), II (39), III (7), IV (16). 5 patients (3,8%) had PH and 6 (4,6%) possible PH. Patients with PH were all women, predominantly black (4 out of 5) with stage IV disease (4 out o...
BACKGROUND The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing heal... more BACKGROUND The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing health status using five modules (General Health Status, Lung, Eyes, Skin, Medication) in patients with sarcoidosis. The KSQ was only validated in one English sarcoidosis cohort. OBJECTIVE The aim of this study was to validate the KSQ in a Dutch sarcoidosis population. METHODS The KSQ was translated according to international guidelines and tested in interviews with patients. Consecutive outpatients completed multiple questionnaires twice, two weeks apart. Construct validity, internal consistency and repeatability were determined. RESULTS Of the 98 patients included 85 had lung, 22 skin and 24 eye disease. There was good construct validity of the KSQ General Health Status module against the World Health Organization Quality of Life-BREF questionnaire. The Medication module correlated weak to moderate with most questionnaires. The correlations with organ-specific questionnaires varied from st...
Pulmonary arterial hypertension (PAH) is rare disease that is categorized as idiopathic (IPAH) wh... more Pulmonary arterial hypertension (PAH) is rare disease that is categorized as idiopathic (IPAH) when no underlying cause can be identified. Lungs of most patients with IPAH contain increased numbers of T cells and dendritic cells (DCs), suggesting involvement of the immune system in its pathophysiology. However, our knowledge on circulating immune cells in IPAH is rather limited. We used flow cytometry to characterize peripheral blood DCs and T cells in treatment-naive IPAH patients, compared with connective-tissue disease-PAH (CTD-PAH) patients and healthy controls (HCs). At diagnosis, T-helper (Th) cells of IPAH patients were less capable of producing TNFα, IFNγ, IL-4 and IL-17 compared to HCs. IPAH patients showed a decreased frequency of Th2 cells and significantly enhanced expression of the CTLA4 checkpoint molecule in naive CD4+ T cells and both naive and memory CD8+ T cells. Frequencies and surface marker expression of circulating DCs and monocytes were essentially comparable ...
Introduction / Rationale Pulmonary Fibrosis (PF) not only has impact on physical but also on soci... more Introduction / Rationale Pulmonary Fibrosis (PF) not only has impact on physical but also on social and emotional well-being of patient and partner. In general, their needs in these domains are ill documented. The objective of this project was to interactively acquire knowledge about patient9s and partner9s perspectives on and preferences in care for social and emotional well-being. Methods During a pulmonary fibrosis patient meeting in our hospital we interviewed patients and partners using voting boxes. Participants could answer anonymously to the 28 questions posed. The system recorded the number of voters. The results were directly presented. Permission to use the data was obtained at forehand. Results 65 patients with PF and 63 partners were present. In brackets number of voters is shown. Self-reported diagnoses were Idiopathic PF (46), collagen vascular disease (9), exposure related (6) and unknown (3). 68% of patients (63) would prefer talking about end of life in an early st...
Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on... more Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on the premise that e-cigarettes are less harmful than tobacco. However, so far studies have not provided convincing evidence that using electronic cigarettes is a better method of smoking cessation than other methods such as motivational counselling, either with or without nicotine replacement therapy. In addition, there is an alarming growth in the popularity of vaping among young people who find e-cigarettes attractive. Recent studies show the harmful effects of the ingredients of the e-cigarette fluid have resulted in acute pulmonary complications such as organizing pneumonia, asthma attacks and diffuse alveolar bleeding. The long-term effects of e-cigarettes are largely unknown, but one doesn't need to be a soothsayer to predict that the long-term damage will be significant. In conclusion, as pulmonologists, we find it necessary to give a serious warning to the Dutch population an...
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease of unknown cause. IPF ... more Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease of unknown cause. IPF has a poor prognosis with a mean survival of 2 to 5 years after diagnosis. The diagnostic process is often complex and demands a multidisciplinary approach. To date, the only curative therapy available is lung transplant. New insights into the pathogenesis of IPF have brought about changes in standard treatment strategies. New drugs have recently become available and have been shown to slow down the decline in pulmonary function considerably and improve survival.
Introduction: Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease characterized by exc... more Introduction: Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease characterized by excess deposition and altered structure of extracellular matrix (ECM) in the lungs. The fibrotic ECM is paramount in directing resident cells toward a profibrotic phenotype. Collagens, an important part of the fibrotic ECM, have been shown to be structurally different in IPF. To further understand the disease to develop better treatments, the signals from the ECM that drive fibrosis need to be identified. Adipose tissue-derived stromal cell conditioned medium (ASC-CM) has demonstrated antifibrotic effects in animal studies but has not been tested in human samples yet. In this study, the collagen structural integrity in (fibrotic) lung tissue, its interactions with fibroblasts and effects of ASC-CM treatment hereon were studied. Methods: Native and decellularized lung tissue from patients with IPF and controls were stained for denatured collagen using a collagen hybridizing peptide. Primary ...
Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on... more Electronic cigarettes have rapidly attracted interest as a method to help people quit smoking, on the premise that e-cigarettes are less harmful than tobacco. However, so far studies have not provided convincing evidence that using electronic cigarettes is a better method of smoking cessation than other methods such as motivational counselling, either with or without nicotine replacement therapy. In addition, there is an alarming growth in the popularity of vaping among young people who find e-cigarettes attractive. Recent studies show the harmful effects of the ingredients of the e-cigarette fluid have resulted in acute pulmonary complications such as organizing pneumonia, asthma attacks and diffuse alveolar bleeding. The long-term effects of e-cigarettes are largely unknown, but one doesn't need to be a soothsayer to predict that the long-term damage will be significant. In conclusion, as pulmonologists, we find it necessary to give a serious warning to the Dutch population an...
Background Recent studies have provided evidence for an important contribution of the immune syst... more Background Recent studies have provided evidence for an important contribution of the immune system in the pathophysiology of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). In this report, we investigated whether the inflammatory profile of pulmonary hypertension patients changes over time and correlates with patient WHO subgroups or survival. Methods 50 PAH patients (16 idiopathic (I)PAH, 24 Connective Tissue Disease (CTD)-PAH and 10 Congenital Heart Disease (CHD)-PAH), 37 CTEPH patients and 18 healthy controls (HCs) were included in the study. Plasma inflammatory markers at baseline and after 1-year follow-up were measured using ELISAs. Subsequently, correlations with hemodynamic parameters and survival were explored and data sets were subjected to unbiased multivariate analyses. Results At diagnosis, we found that plasma levels of interleukin-6 (IL-6) and the chemokines (C-X3-C) motif legend CXCL9 and CXCL13 in CTD-PAH patients we...
Background: The development of pulmonary hypertension (PH) is an important risk factor for early ... more Background: The development of pulmonary hypertension (PH) is an important risk factor for early mortality in sarcoidosis. The prevalence is of SAPH in Europe is largely unknown. Aim: To investigate the prevalence of and clinical parameters associated with SAPH in the Netherlands. Methods: We analyzed clinical data and transthoracic echocardiograms (TTEs), made routinely at our PH/Interstitial Lung Disease clinic, from consecutive patients. Sarcoidosis patients with reliable echocardiography, without left heart dysfunction, were included. An estimated systolic pulmonary artery pressure (sPAP) > 50 mmHg was considered PH. Possible PH was defined as sPAP 37-50 mmHg or sPAP ≤ 36 mmHg with signs of right ventricular dysfunction. Results: From 139 sarcoidosis patients, 130 were included with stage 0 (11), I (46), II (39), III (7), IV (16). 5 patients (3,8%) had PH and 6 (4,6%) possible PH. Patients with PH were all women, predominantly black (4 out of 5) with stage IV disease (4 out o...
BACKGROUND The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing heal... more BACKGROUND The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing health status using five modules (General Health Status, Lung, Eyes, Skin, Medication) in patients with sarcoidosis. The KSQ was only validated in one English sarcoidosis cohort. OBJECTIVE The aim of this study was to validate the KSQ in a Dutch sarcoidosis population. METHODS The KSQ was translated according to international guidelines and tested in interviews with patients. Consecutive outpatients completed multiple questionnaires twice, two weeks apart. Construct validity, internal consistency and repeatability were determined. RESULTS Of the 98 patients included 85 had lung, 22 skin and 24 eye disease. There was good construct validity of the KSQ General Health Status module against the World Health Organization Quality of Life-BREF questionnaire. The Medication module correlated weak to moderate with most questionnaires. The correlations with organ-specific questionnaires varied from st...
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