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Long COVID-19 Syndrome, in addition to symptoms such as chronic cough, exertional dyspnea and fatigue, pulmonary fibrosis is presented as specific respiratory system problems. Post-COVID fibrosis is characterized by radiographic abnormalities consistent with pulmonary fibrosis. Patients with post-COVID fibrosis may benefit from pulmonary rehabilitation programs. This case report aimed to investigate the effects of an individualized pulmonary rehabilitation (PR) program on selected clinical parameters in a 46-year-old male who was diagnosed with post-COVID fibrosis. Spirometry and diffusion tests were investigated. Six-minute walk and shuttle walk tests for exercise capacity, hand-held dynamometer for quadriceps muscle strength, mMrc dyspnea scale for dyspnea, Fatigue Severity Scale for fatigue and London Chest Activity of Daily Living for activities of daily living were evaluated. An individualized PR program included breathing exercises, single-leg cycle training (for the right leg, 10 to 30 min) and bilateral neuromuscular electrical stimulation for quadriceps muscles (30 min) was prescribed 3 days a week for 8 weeks. Small but promising improvements were observed in FEV1, FVC, FEV1/FVC, DLCO, 6MWD, shuttle walk tests walking distance and time, quadriceps muscle strength, mMRC and FSS scores. PR program combined with antifibrotic therapy has the potential to improve respiratory functions, exercise capacity, muscle strength, dyspnea, fatigue, and participation in ADL in patient with post-COVID fibrosis.

Background: Association of chronic spontaneous urticaria (CSU) with sleep disturbance has not been evaluated in studies that involve a large number of patients. Objective: In this study, we aimed to evaluate the sleep attitude and circadian rhythm in patients with CSU. Methods: As the patient group, recently diagnosed 100 patients with CSU, 100 patients with allergic rhinitis (AR) as the patient control group, and 100 healthy controls (HCs) were included. The Pittsburgh Sleep Quality Index (PSQI) questionnaire, sleep hygiene index (SHI), Epworth Sleepiness Scale (ESS) questionnaire, and the morningness‐eveningness questionnaire (MEQ) were filled to assess sleep quality and circadian rhythm. CSU disease activity was evaluated by urticaria activity score-7 (UAS-7). Patients with concomitant diseases, e.g., psychiatric illnesses, that possibly affect sleep status or those who use related medications and at moderate or high risk of obstructive sleep apnea according to the STOP-Bang questionnaire were excluded from the study. Results: PSQI, SHI, and ESS scores were higher, and the MEQ score was lower in patients with CSU and patients with AR than those in the HCs (p < 0.001, for each score). However, the scores were not different among the patients with CSU and the patients with AR. UAS-7 was only correlated with PSQI scores (r = 0.402, p < 0.001). In addition, blood eosinophil counts and the serum C Reactive Protein (CRP) level were correlated with sleep quality (p = 0.02). Conclusion: The poor sleep quality, impaired sleep hygiene, increased daytime sleepiness, and intermediate type of circadian rhythm were observed in the patients with CSU and the patients with AR. Physicians should be aware of sleep problems in patients with CSU that might affect their quality of life and the success of their treatment.

Background Acute pulmonary embolism (APE) is a common clinical condition. Its severity ranges from asymptomatic radiological findings to fatal obstructive shock. Cardiac biomarkers have been studied to predict APE outcomes. This study aimed to explore their predictive power on prognosis in APE. Material and Method It was a prospective observational study between March 2008 and April 2010. All consecutive patients diagnosed with APE were categorized as massive/high-risk, submassive/moderate-risk, and non-massive/low-risk. Cardiac troponin T (cTnT), myoglobin, N-terminal pro-brain natriuretic peptide (NT-proBNP), heart-type fatty acid-binding protein (H-FABP), growth differentiation factor-15 (GDF-15), and D-dimer levels were measured. Results Of these patients, 14 (29.8%), 16 (34.0%), and 17 (36.2%) patients were categorized as low-risk, moderate-risk, and high risk-patients, respectively. There was no significant difference between the patient groups categorized based on the risk stratification in terms of demographic and clinical characteristics. The cTnT, myoglobin, HFABP, and D-dimer levels have also not differed significantly between the groups. There was a significant difference between the groups in respect of NT-proBNP and GDF-15 levels (p = 0.009 and p = 0.037, respectively). Nine (19.1%) patients had died by the 3rd-month follow-up. Adverse events were seen in 26 (55.3%) patients. GDF-15 had the highest area under the curve (AUC) value for predicting any adverse event (cut-off value = 9.3 ng/mL, AUC = 0.796, CI (confidence interval) 95%: 0.653–0.899). NT-ProBNP was determined as the best predictor for mortality (cut-off value = 229.2 pg/mL, AUC = 0.889, CI 95%: 0.756–0.964). Conclusion Higher levels of NT-proBNP and GDF-15 were found to be associated with more severe APE, worse outcomes, and mortality.

Objective: to evaluate clinical efficacy, safety and tolerability of long-term inhaled iloprost treatment in the daily practice for the management of pulmonary arterial hypertension (PAH). Methods: a total of 115 patients with PAH on inhaled iloprost treatment were included. New York Heart Association (NYHA) functional class, brain natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels, and 6-minute walk distance (6MWD) were recorded at baseline and at 3rd to 24th month visits. Safety and tolerability of iloprost treatment were also evaluated during follow-up, as were the survival, clinical worsening, and the related risk factors. Results: the treatment was associated with an increase in the percentage NYHA functional class II (from 0.0% at enrolment to 36.2% at 24th month visit) patients but no significant difference was noted in 6MWD values. Clinical worsening was observed in 63.5% patients, while survival rate was 69.6%. NT-proBNP levels were significantly higher in non-survivors than in survivors (p=0.042). Cox regression analysis revealed the association of female sex [odds ratio (OR)=0.318; 95% confidence interval (CI), 0.128-0.792; p=0.014] and scleroderma-related PAH (OR=0.347; 95% CI, 0.140-0.860; p=0.022) with significantly lower risk (3.14 fold and 2.88 fold, respectively) of mortality. Conclusion: our findings indicate favorable efficacy, safety, and tolerability of long-term iloprost treatment in the management of PAH, whereas improved NYHA functional class was not accompanied with a significant change in 6MWD values. Patient age was a risk factor for clinical worsening, while female sex, scleroderma subtype, and lower NT-proBNP levels were associated with significantly lower mortality risk., Bayer Türk

This study aimed to determine the effect of ground-based walking training on exercise capacity, physical activity, quadriceps muscle strength, and quality of life (QoL) in patients with pulmonary hypertension. A total of 24 patients were included in the study. Patients were randomly assigned to 2 groups as the walking group or the control group. The walking group participated in 30-minute supervised ground-based walking training 2 days/week for 8 weeks. Also, they walked unsupervised at least 1 day/week. The control group received no intervention. The number of weekly steps taken in both groups was recorded using a pedometer. In addition to the sociodemographic and clinic characteristics of the patients, the endurance shuttle walk test, incremental shuttle walk test, and 6-minute walk test were used for the evaluation of exercise capacity, and an activity monitor and pedometer for physical activity, a dynamometer for quadriceps muscle strength, and emPHasis-10 for QoL. After 8 weeks, endurance capacity, maximal exercise capacity, and the number of steps significantly improved in the walking group (p0.05). The 6-minute walk distance, physical activity, quadriceps muscle strength, and QoL were similar in both groups (p0.05). The results of the study showed that ground-based walking could improve endurance capacity, maximal exercise capacity, and the number of steps. Quadriceps muscle strength also improved in the walking group. No adverse effects were reported during the training period. Ground-based walking training can be performed safely in patients with pulmonary hypertension.

Background: Peripheral muscle strength and physical activity levels of patients with pulmonary hypertension (PH) are lower than healthy individuals. Decreased peripheral muscle strength can lead to fatigue and increased perception of exertion which may cause decreased endurance in patients with PH. Objective: This study aimed to determine the relationship between lower extremity muscle strength, physical activity level and endurance capacity in patients with PH. Methods: 27 patients (5 CTEPH, 22 IPH) who were diagnosed as PH included in the study. Quadriceps and iliopsoas muscle strengths were measured with a dynamometer. Physical activity was assessed with activity monitor and total energy consumption was recorded. Endurance capacity was determined by Endurance Shuttle Walking Test (ESWT). Before and after ESWT, dyspnea and leg fatigue symptoms were questioned with a modified Borg scale. Results: There were correlations found between quadriceps muscle strength and total energy consumption and, iliopsoas muscle strength and leg fatigue symptom (r=0.439, p=0.025, r=-0.385, p=0.047, respectively). No correlations were found between endurance capacity and lower extremity muscle strength and physical activity (p>0.05). Conclusion: In this study, it was found that the decrease in lower extremity muscle strength affected physical activity level and leg fatigue in PH. Relationship between lower extremity muscle strength, physical activity and endurance capacity should be evaluated with a larger sample size. In future studies, the effects of structured physical activity programs on lower extremity muscle strength and leg fatigue in PH may be investigated.

Rationale, aims, and objectives The aim of the study was to evaluate the reliability and validity of the Turkish version of the EmPHasis-10 questionnaire to ensure cultural adaptation. Methods This study involved translation, back translation, and cross-cultural adaptation. One hundred and one patients who were diagnosed as having pulmonary hypertension (PH) for at least 6 months were evaluated using the Turkish version of EmPHasis-10. Turkish version of the Minnesota Living with Heart Failure Questionnaire (MLHFQ) was used as gold standard to assess the validation of the Turkish version of the EmPHasis-10 questionnaire. Relationship between MLHFQ and EmPHasis-10 was analysed using Spearman correlation analysis to assess the validation. Cronbach alpha (internal consistency) and exploratory factor analyses were used to assess the questionnaire's reliability. Results The statistical analysis showed that the EmPHasis-10 questionnaire showed a high validity with MLHFQ (r = 0.85) (P = 0.001). Reliability analysis showed that EmPHasis-10 had a high level of Cronbach alpha (α = 0.98) and internal consistency (ICC = 0.97). Conclusions The Turkish version of EmPHasis-10 is a quality of life questionnaire specific to PH. It has a high-level validity and reliability questionnaire that can be used by researchers and physicians.

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping riociguat treatment. Primary safety outcomes were adverse events (AEs) and serious adverse events (SAEs) coded using Medical Dictionary for Regulatory Activities preferred terms and System Organ Classes version 21.0, collected during routine clinic visits and collated via case report forms. Results: In total, 956 patients with CTEPH were included in the analysis. The most common AEs in these patients were peripheral edema/edema (11.7%), dizziness (7.5%), right ventricular (RV)/cardiac failure (7.7%), and pneumonia (5.0%). The most common SAEs were RV/cardiac failure (7.4%), pneumonia (4.1%), dyspnea (3.6%), and syncope (2.5%). Exposure-adjusted rates of hemoptysis/pulmonary hemorrhage and hypotension were low and comparable to those in the long-term extension study of riociguat (Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase–Stimulator Trial [CHEST-2]). Conclusion: Data from EXPERT show that in patients with CTEPH, the safety of riociguat in routine practice was consistent with the known safety profile of the drug, and no new safety concerns were identified. © 2020 The Authors Eli Lilly and Company; Pfizer; Bayer; GlaxoSmithKline; Merck; Actelion Pharmaceuticals; Meso Scale Diagnostics; United Therapeutics Corporation; Novartis Pharma; Merck Sharp and Dohme; GlaxoSmithKline Australia; Deutsche Forschungsgemeinschaft; Grupo Ferrer Internacional, S.A The EXPERT registry was funded by Bayer AG (Berlin, Germany) and Merck Sharp & Dohme Corp., a subsidiary of Merck & Co. Inc., Kenilworth, NJ, USA. The authors acknowledge the database administration by Torsten Tille, Dresden, and the project administration of Mrs Romy Hoppenz and Mrs Linda Kottke at GWT-TUD GmbH, Dresden. Medical writing services provided by Richard Murphy PhD of Adelphi Communications Ltd, Macclesfield, UK were funded by Bayer AG in accordance with Good Publication Practice (GPP3) guidelines. The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Prof Marius M. Hoeper reports personal fees from Bayer AG, during the conduct of the study; personal fees from Actelion, personal fees from Acceleron, personal fees from MSD, personal fees from Jansen, personal fees from Pfizer, outside the submitted work. Dr Hans Klose reports speaker and consultancy fees from Actelion, Bayer AG, GSK, Novartis, Pfizer, and United Therapeutics and research support from Actelion, Bayer AG, GSK, Pfizer, and MSD. Dr Michael Halank reports personal fees and non-financial support from Actelion, AstraZeneca, Bayer AG, Berlin-Chemie, GSK, OMT, MSD, and Novartis. Dr George Giannakoulas reports speaker and consultancy fees from Actelion, Bayer, ELPEN Pharmaceuticals, GSK, Pfizer, Lilly, and United Therapeutics, and research support from GSK, ELPEN Pharmaceuticals, and Galenica. Dr Henning Gall has received honoraria and/or other support from Actelion, AstraZeneca, Bayer, BMS, GSK, Janssen-Cilag, Lilly, MSD, Novartis, OMT, Pfizer, and United Therapeutics. Dr Pavel Jansa reports consultancy and speaker fees from MSD, AOP Orphan, and Actelion. Prof Ekkehard Grünig reports research grants and speaker honoraria/consultancy fees from Actelion and Bayer/MSD, research grants from GSK, United Therapeutics, Bellerophon, OMT GmbH, Pfizer, Reata, and Novartis, and speaker honoraria from Bial, Medscape, and OrPha Swiss GmbH. Prof David Pittrow reports personal fees from Actelion, Bayer AG, Aspen, Boehringer Ingelheim, Sanofi, Biogen, Shire, and MSD outside the submitted work. Silvia Ulrich reports research grants and personal fees from Actelion, Bayer, MSD, and Orpha Swiss. Tobias J. Lange has received personal fees from Actelion, MSD, Pfizer, and OMT orphan. Dr Iraklis Tsangaris reports speaker and consultancy fees from Actelion, Bayer AG, ELPEN, GSK, MSD, Pfizer, and United Therapeutics. Stephan Rosenkranz reports remunerations for lectures and/or consultancy from Abbott, Actelion, Arena, Bayer, Ferrer, GSK, MSD, Novartis, Pfizer, and United Therapeutics; and research support to his institution from Actelion, Bayer, Novartis, Pfizer, and United Therapeutics. Repke J. Snijder reports grants from Pfizer and Actelion Pharmaceuticals. Prof Iveta Šimková reports consultancy and speaker fees from MSD, AOP Orphan, and Actelion. Dr Marc Humbert reports grants and personal fees from Bayer and GSK, and personal fees from Actelion, Merck, and United Therapeutics. Marion Delcroix has received investigator, speaker, consultant, and steering committee member fees from Actelion, Bayer AG, Bellerophon, Eli Lilly, GlaxoSmithKline, MSD, Pfizer, and Reata, and research grants from Actelion. Joan A. Barberà reports receipt of honoraria for consultation or speaker fees from Actelion and Merck; and research support through his institution from Actelion, Merck, GlaxoSmithKline, and Ferrer. Joanna Pepke-Zaba reports research grants and speaker honoraria/consultancy fees from Actelion, Bayer/MSD, and GSK. Jean-Luc Vachiéry reports ongoing consultancies to Actelion, Sonnivie, Arena Pharma, Bial Portela, and Respira Therapeutics, past consultancies to AstraZeneca, BayerShering, CardioMEMS, GlaxoSmithKline, Pfizer, Merck, and United Therapeutics, and current membership of an advisory board or similar group for Actelion and GlaxoSmithKline. Jean-Luc Vachiéry's institution receives funding from Actelion Pharmaceuticals for performing clinical studies. Regina Steringer-Mascherbauer, Jens Klotsche, and Miguel-Angel Gomez Sanchez have no conflicts of interest relevant to the EXPERT study. Hossein-Ardeschir Ghofrani reports personal fees for advisory board work, and payment for lectures including service on speaker bureaus, from Actelion, Bayer, GSK, Novartis, and Pfizer; consultancy fees from Actelion, Bayer, Bellerophon Pulse Technologies, GSK, MSD, Novartis, and Pfizer; and grants from Deutsche Forschungsgemeinschaft (DFG). Pilar Escribano Subias reports personal fees from Actelion, Bayer AG, GlaxoSmithKline, and Merck Sharp & Dohme, and grants from Actelion, Bayer AG, GlaxoSmithKline, and Ferrer, outside the submitted work. Gérald Simonneau reports personal fees and non-financial support from Actelion, personal fees and non-financial support from Bayer, personal fees and non-financial support from MSD, outside the submitted work. Douglas S. Helmersen reports industry-sponsored research with Bayer AG, United Therapeutics, and Gilead Sciences, Inc., and advisory board/speaker fees from Bayer AG and Actelion. David Langleben reports honoraria, consultation fees, research support, and/or travel expenses from Actelion, Arena, Bayer AG, Northern Therapeutics, PhaseBio, Acceleron, Janssen, and United Therapeutics. Anco Boonstra reports consultancy fees from Pfizer BV and hospitality from Teva Nederland. Lisa M. Mielniczuk reports speaker fees and honoraria from Bayer AG, and speaker fees, consultancy fees, and travel fees from Actelion. Evgenia Williams and Christian Meier are employees of Bayer AG.

Introduction: We investigated the frequency of granulomatous lung diseases (GLD) in patients with granulomatosis ocular pathologies who referred to our clinic for differential diagnosis. Methods: Diagnosis of sarcoidosis or definitive tuberculosis was made with microbiological and/or histopathological findings. Patients who did not respond to immunosuppressive treatments, and had latent tuberculosis infection proven with PPD and/or Quantiferon-TB test were considered as possible ocular tuberculosis and antituberculosis treatments were given to these patients. Treatment response was evaluated with vision tests and clinical response. Results: Totally 200 patients (58% female, 42% male, age:46±3.9years) included. Ocular pathologies were uveitis in 65.6%, choroiditis in 20%, vascular pathologies in 6.5% and others 8%. Immunosuppressive treatment were given to 132 (66%) cases; 56 (41%) of them received steroid treatment. PPD performed in 173 patients (PPD:16.6±7mm) and PPD was ≥15mm in 125 of them. Quantiferon-TB test was positive in 85.5% of 166 cases. Sarcoidosis was diagnosed histopathologically in 7.5% (n=15/200), tuberculosis was diagnosed microbiologically or pathologically in 2% (n=4/200). Eighty-nine cases were considered as possible tuberculosis with clinical-radiological findings and the presence of latent tuberculosis. Antituberculosis treatment was given to these patients and 50 of them (56%) responded well. Conclusion: Only 9.5% of cases with ocular granulomatosis disease diagnosed definitively tuberculosis or sarcoidosis. In cases who do not respond to immunosuppressive treatments and have latent tuberculosis infection; initiating anti-tuberculosis treatment can provide good results.

Background: Ground-based walking training, which can be applied easily and can be used in situations where special equipment is not available, may be useful for PH patients. Aim: The aim of this study was to determine the effects of ground-based walking training on exercise capacity, physical activity (PA), balance, quadriceps muscle strength and quality of life (QoL) in patients with pulmonary hypertension (PH). Method: Twenty people included in the study. Patients randomly assigned to two groups as walking and control group. The walking group participated in the 30 minutes supervised ground-based walking training 2 days/week for 8 weeks. In addition, they walked unsupervised at least one day in a week. The control group received no intervention. Both groups number of weekly steps recorded via a pedometer. Incremental Shuttle Walk Test, Endurance Shuttle Walk Test and Six Minute Walk Test were used for the evaluation of exercise capacity, activity monitor and pedometer for PA, Time Up-Go Test and Activities Specific Balance Confidence Scale for balance, dynamometer, for quadriceps muscle strength and Emphasis-10 for QoL. Results: After eight weeks, endurance time and the number of steps increased in the walking group and symptom of leg fatigue were improved (respectively; p0.05). Method: The results of the study showed that ground-based walking can improve endurance capacity, the number of steps and symptom of leg fatigue after exercise tests. No negative events occurred during the training period. Ground-based walking training can be performed safely in patients with PH.

Background T‐wave peak‐to‐end interval (TPEI) is a measure of repolarization dispersion on surface electrocardiogram (ECG). TPEI has been reported as a prognostic parameter with heart disorders. In this study, we aimed to evaluate the relationship between echocardiogram‐derived right heart parameters, right heart catheterization (RHC) measurements, and TPEI in patients with precapillary pulmonary arterial hypertension (PAH). Methods Thirty‐eight patients (29 females and 9 males, mean age of 54.9 ± 10.9 years) who had undergone RHC for a preliminary diagnosis of pulmonary hypertension (PH) were included in the study. We performed transthoracic echocardiography (TTE), and resting 12‐lead ECG was recorded before RHC. TPEI was measured from leads of V1‐V6, DII, DIII, and aVF, and these values are averaged to obtain the global TPEI. Results Duration of TPEI was significantly correlated with mean PAP, pulmonary vascular resistance (PVR), and cardiac index (CI). Longer TPEI was associated with higher N terminal probrain natriuretic peptide (NT pro‐BNP) level, lower 6‐min walk distance (6MWD), and lower tricuspid annular plane systolic excursion (TAPSE). Conclusion Prolongation of TPEI could be a new predictor of adverse outcome in PAH and may provide additional prognostic information for patients with PAH.

OBJECTIVES To evaluate the characteristics of patients who developed tuberculosis while receiving tumor necrosis factor-alpha (TNF-α) antagonists and the related factors with tuberculosis. METHODS Patient's demographics, tuberculin skin test (TST), isoniazid prophylaxis and type of TNF-α antagonist were recorded. TST conversion (≥5 mm increase) was evaluated for patients who had baseline and 1-year TST. RESULTS Files of 1887 patients who were receiving TNF-α antagonists between August 2005 and June 2015 were evaluated. TST significantly increased at the end of 1 year (n = 748 baseline:7.36 ± 7.2 mm vs. 1 year:9.52 ± 7.5 mm, P

Pulmonary hypertension (PH) is a fatal disease although significant improvements in treatment are achieved. Easily implemented and noninvasive prognostic techniques are needed while following-up these patients. The aim was to investigate the role of fractional exhaled nitric oxide (FeNO) in follow-up for patients with PH. In this longitudinal study, patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH) who were seen in PH Outpatient Clinic, Istanbul Faculty of Medicine, Istanbul University, were enrolled in the study. Echocardiography, 6-minute walking test, brain natriuretic peptide, and FeNO measurements were performed, and World Health Organization functional class was evaluated to all patients at baseline, and third, and sixth months. Right-heart catheterization and pulmonary function tests at the time of diagnosis were recorded. The study comprised 31 patients (23 women, 8 men; mean age: 53.4 ± 17.1 years) with PAH (n = 19) and CTEPH (n = 12) and 80 healthy controls. Patients with PH had lower FeNO values than the control group (16.5 ppb vs 19.8 ppb; P < .05). Fractional exhaled nitric oxide values did not change during follow-up and did not correlate with other follow-up measures except tricuspid annular plane systolic excursion values. Fractional exhaled nitric oxide was higher in the idiopathic PAH subgroup at baseline and at third month than patients with PAH associated with other diseases. Fractional exhaled nitric oxide did not change in patients who had clinical deterioration. As a conclusion; Patients with PH had lower FeNO values than healthy controls, but FeNO did not change significantly during follow-up. Large-scale studies with prolonged follow-up periods are needed to understand the role of FeNO in the follow-up of the patients with PH.

Kumbasar, Ozlem Ozdemir/0000-0003-4599-1461 WOS:000514100200008 PubMed ID: 32163363 OBJECTIVES: The aim of this study is to evaluate the approaches of Turkish pulmonologists to the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) in daily clinical practice. MATERIALS AND METHODS: A questionnaire containing 38 questions about the IPF diagnosis and treatment was given to pulmonologists between January 22 and 29, 2018, and the data of 158 physicians who responded to the questionnaire were evaluated. RESULTS: This survey showed that the mean number of patients that physicians followed up and managed annually was 8.3 and 5, respectively. The mean symptom duration before the diagnosis was 9-12 months. Patients were seen on average by three physicians prior to confirmed diagnosis. Almost 80% of the physicians have an opportunity to access a pathologist and radiologist specialized in IPF. However, only 26% of them have an opportunity to access regular multidisciplinary meetings. Although antifibrotics were the most commonly prescribed drugs, approximately 10% of patients were prescribed steroids, N-acetylcysteine, and immunosuppressants. Most of the physicians (81%) were aware of international guidelines; however, the Turkish Thoracic Society IPF Diagnosis and Treatment Consensus Report was read by only 41% of them. CONCLUSION: This survey may lead to the IPF awareness in Turkey, and it may help to close the gaps regarding the diagnosis and treatment.

WOS:000576587500002 PubMed ID: 33031719 OBJECTIVES: The number of studies on the frequency of obstructive sleep apnea (OSA) in subjects with sarcoidosis is low. Therefore, we aimed to investigate the frequency and predictors of OSA in subjects with clinically stable stage I and II sarcoidosis who were not taking corticosteroid and/or immunosuppressive drugs. We also evaluated restless legs syndrome (RLS) and periodic leg movements in sleep (PLMS). MATERIALS AND METHODS: Subjects with clinically stable stage I and II sarcoidosis and not receiving corticosteroid and/or immunosuppressive therapy were included in the study. Upper airway examination, lung function tests (forced vital capacity [FVC], forced expiratory volume in 1 second [FEV1], diffusing capacity of the lungs for carbon monoxide [DLCO]), and polysomnography were performed on all subjects. In addition, subjects' Epworth Sleepiness Scale (ESS) scores and the Pittsburgh Sleep Quality Index (PSQI) were recorded. RESULTS: Of the total number of 46 sarcoidosis subjects (35 women, 11 men; age: 44.4 +/- 10.7 years; body mass index (BMI): 29.3 +/- 5 kg/m2), 28 (60.9%) were detected with OSA (67.8% mild OSA). The recorded ESS score of the subjects was low (2.6 +/- 3.2), whereas the sleep quality was poor in 36.9% of these subjects. Rapid eye movements (REM) related OSA was diagnosed in 14.2% of the OSA subjects. Age was the only factor related to OSA diagnosis in a logistic regression analysis (p=0.048). None of the subjects were diagnosed with RLS and PLMS. CONCLUSION: OSA is common in stage I and II sarcoidosis subjects who did not receive corticosteroid therapy. The frequency of OSA diagnosis increases as the age of the subjects increases. Therefore, sarcoidosis subjects should be evaluated for OSA throughout the follow-up.

Introduction: Minor salivary gland biopsy is used as a minimally invasive method in the diagnosis of sarcoidosis. To date, a few studies have shown that minor salivary gland biopsy is an diagnostic tool in differentiating sarcoidosis from tuberculosis. In this study we evaluated the diagnostic yield of minor salivary gland biopsy in patients with sarcoidosis. Material and methods: The study included patients with sarcoidosis who were diagnosed during the last 10 years in Pulmonary Department of Istanbul Medical Faculty. Baseline diagnostic tests, histopathological results and clinical findings were recorded. Results: Totally, 354 patients (270 female, 84 male, mean age: 46.48) with sarcoidosis were analyzed. Radiologically, 7.3% was stage 0, 46.6% was stage I, 33% was stage II, 9% was stage III, and 3.9% was stage IV. Respiratory functions were normal in 70%, diffusion capacity was normal in 66% and 18.8% had obstructive pattern, 10.9% had restrictive pattern in spirometry. The most common extrapulmonary involvement was skin (%22.8) and eye (%16.9). Minor salivary gland biopsy was performed in 215 cases and 49.3% of them had findings compatible with sarcoidosis. In 13.8% of the cases, the diagnosis was based on only minor salivary gland biopsy and clinical-radiological findings. Conclusion: In our study, salivary gland biopsy was diagnostic in half of the cases, and in 13.8% of the cases was diagnosed without any other invasive procedure. Therefore, it can be used as a priority biopsy method in patients with clinical and radiological suspicion of sarcoidosis.

Background Impaired respiratory muscle function may be one of the causes of increased dyspnea, reduced exercise capacity, and physical activity (PA), and poor quality of life in pulmonary hypertension (PH). Objective To investigate the effects of threshold inspiratory muscle training (TIMT) on respiratory functions, functional exercise capacity, PA, and QoL in patients with PH. Methods Thirty patients with PH were randomly allocated to a TIMT (n = 15) and sham group (n = 15). Three patients in the sham group could not participate in the program. The TIMT group (n = 15) trained at 30% of the maximal inspiratory pressure (MIP), and the sham group (n = 12) performed at lowest pressure without change in threshold pressure. In both groups, patients performed TIMT at home for 15 min, twice per day, with the MIP load determined by the trainer, and were supervised once weekly at the hospital for eight weeks. The primary outcomes were MIP and maximal expiratory pressure (MEP). The secondary outcome measures included spirometric measurements, six-minute walking distance (6MWD), PA (SenseWear armband and International Physical Activity Questionnaire-Short Form-IPAQ-Short Form), and QoL (Minnesota Living with Heart Failure-MLHF). Results After the training, changes in MIP (p = 0.023) were higher in the intervention group compared with the sham group. Differences in MEP, FEV1 (%), FVC (%), FEV1/FVC (%), 6MWD, %6MWD, IPAQ-SF, MLHFQ, and armband parameters were not significantly different between the groups (p > 0.05). Conclusions The results of the study demonstrated that TIMT could increase MIP and did not improve other parameters of respiratory functions, functional exercise capacity, PA, and QoL in patients with PH.

Objective: To examine the serum levels of leptin and adiponectin in different obstructive sleep apnea (OSA) phenotypes. Methods: Obese patients who were admitted to our sleep laboratory were included. All patients underwent spirometry, daytime arterial blood gas analysis, polysomnography and transthoracic echocardiography. Serum levels of adiponectin and leptin were recorded. Results: Analysis included 146 OSA patients (81 females, 65 males, age: 49.8 ± 10.7 years, body mass index: 40.3 ± 4.9 kg/m2, 47.9% severe OSA, 42.5% severe obesity). Females had higher leptin and adiponectin levels (p

Introduction: Pulmonary hypertension (PH) is characterized by exertional dyspnea, fatigue, chest pain, dizziness, and syncope. Physical activity, peripheral and respiratory muscle strength reduces in pateints with PH. Little is known about respiratory muscle weakness and related outcomes. Objectives: The aims of the study were to determine respiratory muscle strength and to investigate the relationship between respiratory muscle strength and spirometric measurements, exercise capacity, physical activity level, quality of life, and pulmonary hemodynamics in patients with PH. Methods: In total, 33 patients aged 25-80 years who were diagnosed as having PH and 24 healthy volunteers were included in the study. To measure respiratory function, spirometry, maximal inspiratory (MIP), and expiratory pressures (MEP) were used. Physical activity level was determined with activity monitoring (SenseWear Armband) and the International Physical Activity Questionnaire-Short Form. Exercise capacity was determined using the six-minute walk test. Quality of life was evaluated with the Minnesota Living with Heart Failure Questionnaire (MLHFQ). Results: Maximal inspiratory pressure and MEP values of the patients with PH were significantly lower than the age- and sex-matched healthy controls (p

INTRODUCTION Patients with pulmonary hypertension (PH) show no symptoms at rest, but symptoms are triggered by physical activities. OBJECTIVES The primary aim of our study was to assess physical activity of patients with PH by using an activity monitor. The secondary aim was to evaluate the correlation between the activity monitor parameters and 6-min walk distance (6MWD), activity of daily living (ADL), quality of life, WHO functional class and PH classification. METHODS Thirty-eight patients with pulmonary arterial hypertension (PAH) and seven patients with chronic thromboembolic PH were included in the study. Physical activity was assessed using a SenseWear arm band. A 6-min walk test was performed. Daily living activities were assessed using the 'nottingham extended activity of daily living index' (NEADL). For quality of life assessments, 'Minnesota living with heart failure' (MLHF) and 'Short form-36' (SF-36) surveys were used. RESULTS Physical activity, exercise capacity, quality of life and contribution to ADL were lower in patients with PH. These data were associated with the 6MWD, quality of life and ADL index scores. We also found weak and moderate correlations between activity monitor data and SF-36 sub-group scores, MLHF and NEADL scores (P

Introduction Conflicting results has been achieved in a small number of clinical studies evaluating the efficiency of magnesium sulphate (MS) in COPD exacerbations. We aimed to investigate the efficiency of nebulised MS in COPD exacerbations. Patients and methods Twenty patients who met the study criteria were randomized into two groups. All patients were treated with O2, antibiotics and oral corticosteroids. Additionally one group received ipratropium bromide (IB) 500 µg together with MS 151 mg/dose, while the other group received IB together with placebo. The patients were followed-up with forced expiratory volume in 1 second (FEV1) and visual analogue scale dyspnea scores for 48 hours. Peak expiratory flow rates (PEFRs) were measured before and 10, 30, 60 and 120 minutes after each nebule treatment. Result The baseline characteristics of the patients in both groups were similar. The FEV1 values measured at 24 and 48 hours did not show significant changes compared to baseline in both groups. Dyspnea scores in both groups decreased significantly in the first day, and in only MS group in the second day. The % change in the dyspnea score at the end of first day was significantly more in the MS group [-23.8% (13.6)] compared with the placebo group [-9.4% (12.9)] (p= 0.002). The % changes in PEFRs at 10 minutes [4.7 (7.5) and -3.5 (6.0), p= 0.005] and 30 minutes [8.2 (6.7) and 1.3 (5.5), p= 0.03] were significantly greater in the MS group compared with the placebo group on the first day. No side effects developed due to MS. Conclusion Nebulised MS is a cheap, feasible and safe drug that can be added to the standart bronchodilator treatment since it provides additional relief of dyspnea in patients with COPD exacerbations. This needs to be evaluated in future clinical studies including greater number of patients.

Introduction: Risk of recurrence and bleeding, play a role in considering the optimal duration of treatment. The aim of the study was to determine the frequency of recurrence, risk factors for recurrence and bleeding in cases with VTE. Material-methods:: Study included 412 VTE patients (164 male, 248 female, mean age: 53.5years, follow-up period: 25.3months). Patient9s demographics, risk factors, comorbidities, clinical data, treatment and complications were recorded. Results: There was at least one risk factor in 341 (82.7%) patients and 71 (17.2%) of them were idiopathic. In 76 (18.4%) of the cases VTE recurrence developed. There was no difference between recurrent and non-recurrent groups according to age and sex. There was no relationship between recurrence and mortality. Recurrence was higher in DVT and / or PTE+DVT groups than PTE group (24% vs 14.2%, p=0.007). Idiopathic VTE was higher in recurrent cases (26.3% vs 15.2%, p=0.028). D-dimer levels were higher in recurrent cases at first year control (p=0.034). Hereditary risk factors were higher in recurrent cases (p=0.031). During VTE treatment, 37 (8.9%) of cases had bleeding however major bleeding rate was 3.6%. Duration of anticoagulant was longer in patients who had bleeding (22months vs 12months p=0.047). Bleeding rate was higher in patients with massive PTE (p=0.001) and in elderly patients (≥75years) (p=0.036). Conclusion: In our study, presence of DVT, idiopathic VTE, hereditary risk factors and high D-dimer levels at first year were found as related factors of recurrence. Risk of bleeding increased in recurrent cases, in elderly cases and in cases whose treatment duration was longer in the first VTE event.

28th International Congress of the European-Respiratory-Society (ERS) -- SEP 15-19, 2018 -- Paris, FRANCE

Introduction: Arterial blood gas (ABG) sampling is difficult in patients with obesity hypoventilation syndrome (OHS) and neuromuscular diseases (NMD). Our aim was to investigate the usability of venous blood gas (VBG) analysis, to replace ABG. Material and Methods: Study included stabilized OHS and NMD patients. ABG was taken from all of the cases in the room air and peripheral VBG samples were taken within 5 minutes after ABG analysis. Results: Totally 82 cases (female:33, male:49, mean age:46.9years) with OHS (n=36) and NMD (n=46) were included. For all patients, there was a moderate positive correlation between arterial and pH values (rs=0.590, p For NMD patienst; there was moderate positive correlation between arterial and venous pH and PCO2 values (rs=0.542, rs=0.540, p Conclusion: We found a relationship between ABG and VBG for the values of pH, PCO2 and HCO3. As a results, VBG values (pH, pCO2, HCO3) can be used safely during treatment and follow-up period of the patients with OHS and NMD, instead of ABG.

Introduction: The symptoms that pulmonary hypertension (PH) patients experience causes decreased quality of life (QoL). Assessment of QoL in daily clinical practice is often overlooked but today its importance is better understood. The aim of the study is to translate the EmPHasis-10 questionnaire into Turkish language, to ensure cultural adaptation, reliability and validity. Materials and Methods: This study involved translation, back translation, and cross-cultural adaptation. EmPHasis-10 is a questionnaire evaluating the QoL of patients with PH. The study included 101 cases (82 females, 19 males) who were followed up by pulmonary hypertension policlinic for at least six months. Sociodemographic data of the patients was recorded. Emphasis-10 Turkish version, Short Form-12 (SF-12), Minnesota Living with Heart Failure Questionnaire (MLHFQ), Hospital Anxiety and Depression Scale (HADS) was used and six-minute walk test was performed to determine the exercise capacities. The same procedure was repeated to the participants after one week for the test-retest. Results: As a result of the statistical analysis, EmPHasis-10 survey showed a high level of correlation with MLHFQ (r:0.85, p:0.001) to proving its validity. Reliability analysis revealed that EmPHasis-10 showed a high level of Cronbach alpha (α: 0.98) and internal consistency (ICC: 0.97). Conclusion: The EmPHasis-10 the Turkish version is a QoL questionnaire with a high level of validity and reliability, which can be used easily by academicians and clinicians conducting research.

Background: Pulmonary Hypertension (PH) is characterized by physical inactivity and decreased exercise capacity and quality of life. Limited studies showed that PH patients have kinesiophobia. The related factors are not clear in PH. Objective: The aim of this study is to determine the relation between kinesiophobia and exercise capacity, the activity of daily living and quality of life in patients with PH. Methods: Thirty-four patients (mean of age=52.62±15.58 and body mass index=24.28±5.28, 28 female, 6 male, 15 patients in PH Group 4, 19 patients in Group 1) diagnosed with PH were included in the study. Sociodemographic and clinical features of the participants were questioned. Exercise capacity was assessed with the six-minute walking test (6MWT), "Tampa Kinesiophobia Scale for Heart" (TKSH) were used for kinesiophobia, "London Chest Activity of Daily Living" (LCADG) for the activity of daily living and "Nottingham Health Profiles" (NSP) for the quality of life. Results: The mean value for six-minute walking distance was 402.7±82.5 meters, TKSH was 43.5±3.79, LCADG was 18.7±5.1 and NSP was 196.5±123.4. Significant correlations were found between TKSH and NSP total score (p=0.010, r=0.438) and NSP subgroup scores-emotional reactions (p Conclusion: The results of the study showed that kinesiophobia is related to the impaired quality of life. Although no relation was found with exercise capacity increased dyspnea can associate kinesiophobia in PH.

28th International Congress of the European-Respiratory-Society (ERS) -- SEP 15-19, 2018 -- Paris, FRANCE -- European Respiratory Soc [Abstract Not Available]

Summary Background Macitentan is beneficial for long-term treatment of pulmonary arterial hypertension. The microvasculopathy of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension are similar. Methods The phase 2, double-blind, randomised, placebo-controlled MERIT-1 trial assessed macitentan in 80 patients with CTEPH adjudicated as inoperable. Patients identified as WHO functional class II–IV with a pulmonary vascular resistance (PVR) of at least 400 dyn·s/cm 5 and a walk distance of 150–450 m in 6 min were randomly assigned (1:1), via an interactive voice/web response system, to receive oral macitentan (10 mg once a day) or placebo. Treatment with phosphodiesterase type-5 inhibitors and oral or inhaled prostanoids was permitted for WHO functional class III/IV patients. The primary endpoint was resting PVR at week 16, expressed as percentage of PVR measured at baseline. Analyses were done in all patients who were randomly assigned to treatment; safety analyses were done in all patients who received at least one dose of the study drug. This study is registered with ClinicalTrials.gov, number NCT02021292. Findings Between April 3, 2014, and March 17, 2016, we screened 186 patients for eligibility at 48 hospitals across 20 countries. Of these, 80 patients in 36 hospitals were randomly assigned to treatment (40 patients to macitentan, 40 patients to placebo). At week 16, geometric mean PVR decreased to 73·0% of baseline in the macitentan group and to 87·2% in the placebo group (geometric means ratio 0·84, 95% CI 0·70–0·99, p=0·041). The most common adverse events in the macitentan group were peripheral oedema (9 [23%] of 40 patients) and decreased haemoglobin (6 [15%]). Interpretation In MERIT-1, macitentan significantly improved PVR in patients with inoperable CTEPH and was well tolerated. Funding Actelion Pharmaceuticals Ltd.

Introduction: Limited data is available on Asthma-COPD Overlap Syndrome (ACOS. In this study, the patients followed in specialized centers with a diagnosis of asthma or COPD were evaluated by a standart approach to determine the prevalence of ACOS, as well as risk factors and clinical presentations to predict the factors for development of ACOS. Methods: The study was conducted in a cross-sectional design in 9 different asthma and COPD centers. The patients followed with a diagnosis of asthma or COPD i for at least for 1 year were enrolled in the study. The demographics, disease characteristics, results of diagnostic tests and medications were recorded. Results: The study included a total of 408 subjects. The prevalance of ACOS was 20.8% (n:85) in the whole group. The patients with ACOS had similarities to the patients with COPD in terms of advanced age, male gender, exposure to biomass during childhood, being raised in rural area and radiological features, whereas characteristics like history of childhood asthma and allergic rhinits, presence of comorbidities such as chronic sinusitis, NSAIDs hypersensitivity, atopy, and high eosinophil counts resembled to those of asthma patients (p Conclusion: This study showed that ACOS was common among asthma and COPD patients in Turkey. Results indicated that ACOS can be predicted particularly among male asthma patients with advanced age who raised in rural area and who had childhood exposure to biomass.COPD patients should be reviewed for possibility of ACOS in the presence of atopy, eosinophilia, childhood allergic rhinitis/ asthma, and sinusitis or NSAIDs hypersensitivity. This study is granted by Turkish Thoracic Society.

Patients with chronic obstructive pulmonary disease (COPD) who have nocturnal oxygen desaturation (NOD) can be treated with nocturnal oxygen therapy (NOT) to avoid possible morbidity and mortality. Although there is no definite data recommending NOT alone, our aim is to evaluate the relationship between desaturation during the six-minute walk test (6MWT) and NOD in COPD. Fifty-five stable patients with COPD were enrolled in this study. The 6MWT and nocturnal oximetry were performed. Patients with comorbid diseases and respiratory failure were excluded. In total, 55 patients (49 males and 6 females, mean age: 65.8 ± 8.4 years) were analysed. Twenty-seven of the patients had moderate COPD and the remainder ( n = 28) had severe COPD. Three patients (11%) with moderate COPD and 12 patients (42.9%) with severe COPD desaturated during 6MWT ( p = 0.003). NOD was observed in five patients with severe COPD (17.9%). There were no patients with NOD in the moderate COPD group. Three (25%) of patients with severe COPD who desaturated during the 6MWT also had NOD. NOD was more common in patients with severe COPD and the patients with higher carbon dioxide levels ( p = 0.02 and p = 0.001). Three patients (11%) with moderate COPD desaturated during the 6MWT; however they did not have NOD. Although the sample size in this study was too small to be conclusive, NOD was more common in desaturators during the 6MWT particularly in patients with severe COPD.

Introduction There is very few data on the epidemiological features of interstitial lung diseases (ILD) in the literature. These studies on this subject suffer from limited number of patients. Objective The goal of this study was to evaluate the epidemiological features of ILD in Turkey. Methods Fifty-four investigators, 31 centres in 19 cities from six regions of Turkey, participated in the study. Two thousand two hundred forty-five newly diagnosed patients (51.8% females), led by Turkish Thoracic Society Clinical Problems Study Group, enrolled in this prospective study. Results The mean age was 51.8 ± 16.7 years. The mean age among males was 50.5 ± 18.6 years and 53.0 ± 14.6 years among females (P

Introduction: Pulmonary hypertension (PH) is a chronic, progressive disease with a high morbidity and mortality. Its pathobiology involves interacting pathways of prostacyclin (PGI2), endothelin, and nitric oxide (NO). There are few studies which evaluated exhale NO levels in patients with PH. Aim of this study was to investigate the levels of exhaled NO in PH patients. Methods: Patients who were followed in Istanbul Faculty of Medicine, Pulmonary Hypertension Outpatient Clinic were enrolled in this study. Exhaled NO levels of patients with PH were compared to age and gender matched healthy controls. Results: Data of 38 patients with PH (28 females, 10 males, age:54.5±16.3years) were compared to 80 healthy controls (55 females, 25 males, age:49.8±14.4years). Etiologies of PH were pulmonary arterial hypertension (PAH, n=24) and chronic thromboembolic pulmonary hypertension (CTEPH, n=14). NYHA functional class of the patients were mostly II (68.4%). Thirteen of them (41.9%) were given PH specific treatment (prostacyclin derivatives, endothelin receptor antagonists, phosphodiesterase inhibitors). Estimated systolic pulmonary arterial pressure (PAP) by transthoracic echocardiography was 64.9±28.2mmHg and TAPSE was 1.88±0.48. ProBNP measured 1169.2±2858.9pg/ml. Right heart catheterization revealed systolic PAP 87.1±39.4mmHg, mean PAP 55.9±25.4mmHg, PVR 7.9±5.3wood. Exhaled NO levels were lower in patients with PH than healthy controls (16.7±6.8ppb vs 19.8±7.7ppb, p=0.036). Exhaled NO levels were not different between patients with PAH and CTEPH (16.7±6 vs 16.7±7.7, p=0.458). Conclusions: Our study showed a significant reduction in exhaled NO levels in PH patients.

Background and objective: Extrapulmonary sarcoidosis is common, and is almost always associated with concomitant thoracic involvement. Extrapulmonary manifestations vary on the basis of gender, age at presentation and ethnicity. The aim of this study was to investigate extrapulmonary involvement in patients with sarcoidosis in Turkey. Methods: This study was conducted by Turkish Thoracic Society Clinical Problems Study Group. New cases of sarcoidosis between 1 June 2004 and 31 May 2006 were recorded on electronic case record forms sent to all potential investigators and information about extrapulmonary involvement was collected. Results: One hundred and nineteen of 293 patients (83 female, 36 male, mean age = 45 ± 12 years) had extrapulmonary involvement in this study (40.6%). The median time to diagnosis was 6 months and this was longer than patients with just thoracic sarcoidosis (P = 0.001). Extrapulmonary symptoms were present in 181 (61.8%) patients, and skin lesions, arthralgia and back pain were the commonest (33.4%, 20.8% and 16.4%, respectively). Incidence of organ involvement was independent of age with the exception of ocular involvement, which was higher in those under the age of 40 years (P = 0.007). Conclusions: Skin and peripheral lymph node involvement were the most common sites of extrapulmonary involvement and ocular involvement was more common in those under the age of 40 years in patients with sarcoidosis in a Turkish population.