Assessment of quality of life in IPF Patients: a multicenter observational study.

AIM: This study aimed to examine how dyspnea, cough, sleep disruption, anxiety, depression, and physiological factors affect the quality of life in newly diagnosed, untreated IPF patients. METHODS: This study is a multicenter observational study. Patients not receiving antifibrotic treatment were included. To assess patients' quality of life, Leicester Cough Questionnaire (LCQ), St. George's Respiratory Questionnaire (SGRQ), Short Form-36 (SF-36), Hospital Anxiety and Depression Scale (HADS), Borg Dyspnea Index (BDI), Modified Medical Research Council Dyspnea Scale (MMRC) score, Composite Physiological Index (CPI), Gender Age and physiology (GAP) score, and Pittsburgh Sleep Quality Index (PSQI) were administered. RESULTS: Among 88 patients (mean age: 67.6±8.5 years), 81.9% were diagnosed with IPF through HRCT, 14.8% through surgery, and 3.4% via cryobiopsy. The average disease duration was 2.2±2.9 years. Over 50% experienced moderate to severe depression, and 40% had moderate to severe anxiety. In the IPF group, 13.6% had possible usual interstitial pneumonia (UIP), and 81.8% had definite UIP pattern. No significant differences were found between UIP groups in various scores. Anxiety and depression correlated negatively with respiratory function and positively with MMRC score and BDI. Sleep quality scores had similar correlations. Patients with good sleep quality had better respiratory parameters (p=0.013), lower MMRC (p=0.004), BDI (p=0.026), and CPI (p=0.047). -Conclusion: A notable number of IPF patients in follow-up show symptoms of anxiety and depression. Moreover, declining respiratory function not only diminishes sleep quality but also elevates dyspnea scores.

Comparative Transcriptomic Analyses of Peripheral Blood Mononuclear Cells of COVID-19 Patients without Pneumonia and with Severe Pneumonia in the First Year of Follow-Up.

The multisystemic effects of COVID-19 may continue for a longer time period following the acute phase, depending on the severity of the disease. However, long-term systemic transcriptomic changes associated with COVID-19 disease and the impact of disease severity are not fully understood. We aimed to investigate the impact of COVID-19 and its severity on transcriptomic alterations in peripheral blood mononuclear cells (PBMCs) following 1 year of the disease. PBMCs were isolated from the peripheral blood of healthy control donors who did not have COVID-19 (C; n = 13), from COVID-19 patients without pneumonia (NP; n = 11), and from COVID-19 patients with severe pneumonia (SP; n = 10) after 1-year of follow-up. Following RNA isolation from PBMCs, high-quality RNAs were sequenced after creating a library. Differentially expressed genes (DEGs) and differentially expressed long non-coding RNAs (DElncRNAs) were identified using Benjamini-Hochberg correction and they were analysed for hierarchical clustering and principal component analysis (PCA). Intergroup comparisons (C vs. NP, C vs. SP, and NP vs. SP) of DEGs and DElncRNAs were performed and hub genes were determined. Functional enrichment analyses of DEGs and DElncRNAs were made using Metascape (v3.5.20240101) and the first version of NCPATH. The RNA sequencing analysis revealed 4843 DEGs and 1056 DElncRNAs in "C vs. NP", 1651 DEGs and 577 DElncRNAs in "C vs. SP", and 954 DEGs and 148 DElncRNAs in "NP vs. SP", with 291 DEGs and 70 DElncRNAs shared across all groups, respectively. We identified 14 hub genes from 291 DEGs, with functional enrichment analysis showing upregulated DEGs mainly linked to inflammation and osteoclast differentiation and downregulated DEGs to viral infections and immune responses. The analysis showed that 291 common and 14 hub genes were associated with pneumonia and that these genes could be regulated by the transcription factors JUN and NFκB1 carrying the NFκB binding site. We also revealed unique immune cell signatures across DEG categories indicating that the upregulated DEGs were associated with neutrophils and monocytes, while downregulated DEGs were associated with CD4 memory effector T cells. The comparative transcriptomic analysis of NP and SP groups with 52 gene signatures suggestive of IPF risk showed a lower risk of IPF in the SP group than the NP patients. Our findings suggest that COVID-19 may cause long term pathologies by modulating the expression of various DEGs, DeLncRNAs, and hub genes at the cellular level.

Relationship between nailfold capillaroscopy findings and the etiology and prognosis of interstitial lung disease.

OBJECTIVES: Connective tissue-associated interstitial lung diseases (CTD-ILD) are believed to be caused by microvascular damage. The objective of this study was to assess the nailfold capillaroscopy (NFC) pattern in patients diagnosed with both CTD-ILD and non-CTD-ILD to identify microvascular changes and determine the relation between capillaroscopic parameters, clinical variables, and disease-related measurements. PATIENTS AND METHODS: This cross-sectional study included 95 patients with interstitial lung disease who applied to our Rheumatology and Chest Clinics between September 2021 and July 2023. The patients were divided into two groups based on their diagnosis: non-CTD-ILD (group 1) and CTD-ILD (group 2). Nailfold capillaroscopy was performed. RESULTS: Ninety-five patients, 49 (51% female, mean age 62.31 ± 11.027 years) in group 1 and 46 (69.6% female, mean age 62.09 ± 10.887 years) in group 2, were included in the study. Abnormal capillary morphologies were both detected in the CTD-ILD group and the non-CTD-ILD groups. In patients with a usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT), tortuosity was higher than in patients with non-specific interstitial pneumonia (NSIP) (P = 0.041), and the proportion of tortuosity increased significantly as the duration of the disease increased (P = 0.016). CONCLUSION: Our study highlights capillaroscopic abnormalities alone may not be sufficient to differentiate CTD-ILD (other than systemic sclerosis) from non-CTD-ILD. The presence of NFC abnormalities in non-CTD-ILD may suggest that fibrotic lung disease could potentially play a role in the deterioration of the microvascular structure or abnormal angiogenesis. Our study demonstrated that a multidisciplinary approach, incorporating clinical, morphological, pathological, and serological evaluations, is necessary for interpreting ILD. Key Points • Capillaroscopic abnormalities can also be seen in non-CTD-ILD. • Capillaroscopy findings do not distinguish the non-Ssc etiology of ILD. • Nailfold capillaroscopy may have the potential to serve as a useful tool in predicting prognosis and monitoring the disease progression in patients with idiopathic pulmonary fibrosis (IPF).

Elderly idiopathic pulmonary fibrosis patients remain on therapy despite higher incidence of adverse events and dose reductions.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) predominantly affects people over the age of 60 years and its incidence increases with age. Limited data is available on the use of antifibrotics in the elderly IPF population. We aimed to examine the tolerability and safety of antifibrotics (pirfenidone, nintedanib) in elderly patients with IPF in a real-world setting. METHODS: Medical records of 284 elderly (≥75 years) and 446 non-elderly IPF patients (<75 years) were retrospectively analyzed in this multi-center study. Patient characteristics, treatments, adverse events (AEs), tolerability, hospitalizations, exacerbations, and mortality were compared between the elderly and non-elderly group. RESULTS: In the elderly group, the mean age was 79 years and the mean antifibrotic treatment duration was 26.1 months. The most commonly reported AEs were weight loss, loss of appetite and nausea. Elderly IPF patients had a significantly higher incidence of AEs (62.9% vs. 55.1%, p = 0.039) and dose reductions (27.4% vs. 18.1%, p = 0.003) than the non-elderly did, but the rate of discontinuation of antifibrotics was not different between groups (13% vs. 10.8%, p = 0.352). In addition, the severity of the disease, frequency of hospitalizations, exacerbations, and mortality rates were higher in elderly patients. CONCLUSION: The present study showed that elderly IPF patients experienced significantly increased AEs and dose reductions due to antifibrotic use, while the discontinuation rates of the drugs were similar to those of drugs used by non-elderly patients.

Microvascular damage evaluation based on nailfold videocapillarosopy in sarcoidosis.

OBJECTIVES: Microvascular damage is thought to play a role in the pathogenesis of sarcoidosis. We aimed to evaluate the nailfold capillaroscopy (NVC) pattern to detect microvascular changes in patients with sarcoidosis and the relationship of capillaroscopic parameters with clinical variables and disease-related measurements. PATIENTS AND METHODS: Forty-two patients with sarcoidosis and 42 age- and sex-matched patients with systemic sclerosis (SSc) and healthy individuals were included in this cross-sectional case-control study. Patients aged 18-80 years who met the current American Thoracic Society criteria for sarcoidosis were included. NVC was performed by a digital microscope under a magnification of × 200. Capillary density, number of dilated, giant and neoangiogenic capillaries, capillary loop diameter, capillary shape, micro-hemorrhages, and number of avascular areas, were evaluated by an assessor who was blind to the groups allocation. RESULTS: Among the capillaroscopic parameters, neoangiogenesis and dilated capillaries, which can be seen in the pattern of scleroderma, were detected in patients with sarcoidosis but not significantly different from the control group (p = 0.055; p = 0.433, respectively). The rate of tortuosity and crossing capillaries of 50% and above was significantly higher in the sarcoidosis group than in SSc and healthy controls (p < 0.05). In patients with sarcoidosis, the only statistically significant finding was; forced expiratory volume (FEV1) in one second was lower in patients with a crossed capillary rate > 50% than in patients with a crossed capillary rate of less than 50% (FEV1; 87.21 ± 19.3, 102.5 ± 14.8, p = 0.04; respectively). CONCLUSION: Patients with a diagnosis of sarcoidosis have different capillaroscopic patterns. The presence of these nonspecific abnormal patterns may reflect microvascular damage in the pathophysiology of sarcoidosis. Key Points • Microvascular damage may play a role in the pathogenesis of sarcoidosis. • There may be some nonspecific abnormal findings in capillaroscopy findings in sarcoidosis. • Capillaroscopy may be valuable in reflecting sarcoidosis lung injury.

Transatrial balloon atrial septostomy to facilitate weaning off venoarterial ECMO after pulmonary endarterectomy.

Atrial septostomy has been the last resort in the management of patients with the right ventricular failure related to pulmonary hypertension before lung transplantation. In this paper, we present a simple and safe technique (transatrial approach) for balloon atrial septostomy in a patient who required central venoarterial extracorporeal membrane oxygenation after pulmonary endarterectomy.

Characteristics and outcomes of COVID-19 patients with IPF: A multi-center retrospective study.

BACKGROUND: There are few data on severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (COVID-19) infection in patients with idiopathic pulmonary fibrosis (IPF). The objective of this study is to describe the characteristics and outcomes of IPF patients confirmed COVID-19 infection. METHODS: In this retrospective, multi-center, cohort study, patients from 4 hospital medical records with known IPF and a COVID-19 diagnosis were identified. Demographic and clinical outcome data were abstracted through a review of electronic medical records. RESULTS: Records for 46 patients with IPF and COVID-19 were abstracted. The mean age was 65±10 years. The most common symptom was dyspnea, followed by fever and cough. Ground-glass opacities (n = 35, 83.3%) and consolidations (n = 11, 26.1%) were the main imaging features of the disease in thorax computed tomography (CT). Twenty-four patients (52.1%) required hospitalization. Among the hospitalized patients, 16 (66.6%) were admitted to the intensive care unit (ICU), and 10 (41.6%) underwent invasive mechanical ventilation. Thirteen patients (28.2%) died of COVID-19 complications. Mortality rate was significantly associated with lower DLCO/VA, long term oxygen therapy and consolidation finding on CT of thorax (p<0.05). On multivariable analysis, neither factor was associated with hospitalization or mortality. CONCLUSIONS: IPF patients represent a vulnerable population for COVID-19, according to the high rate of hospitalization, ICU requirement, and mortality rate. Measures to minimize the risk of COVID-19 infection remain key to protect IPF patients.

Clinical efficacy, safety, tolerability, and survival outcome of long-term inhaled iloprost treatment in the management of pulmonary arterial hypertension: Data from prospective multicenter observational OPTION study.

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.

Evaluation of efficacy and safety of pirfenidone 200 mg tablets in patients with idiopathic pulmonary fibrosis in a real-life setting

Background/aim: Phase III trials have demonstrated a significant efficacy and an acceptable safety for pirfenidone in patients having mild to moderate idiopathic pulmonary fibrosis (IPF). Real-life data on the use of pirfenidone 200 mg tablets are limited. This study aimed to investigate the efficacy and safety of pirfenidone 200 mg tablets for the treatment of IPF in a real-life setting. Materials and methods: A retrospective, multicenter study conducted in four university hospitals in Turkey between January 2017 and January 2019. Clinical records of patients diagnosed with mild to moderate IPF and receiving pirfenidone (200 mg tablets, total 2400 mg/day) were reviewed retrospectively and consecutively. Pulmonary function measurements including forced vital capacity (FVC%) and diffusing capacity of the lungs for carbon monoxide (DLCO%) were analyzed at baseline and after 6-month of pirfenidone treatment. Descriptive statistics were expressed as mean, standard error or median (minimum-maximum), number and percentage, where appropriate. Results: The study included 82 patients, of whom 87.8% were males (mean age, 66 years). After 6-month of treatment, 7 patients discontinued the treatment. Of the remaining 75 patients, 71 (94.6%) remained stable, 4 (5.4%) had progressive disease as evident by a decline in the FVC% of at least 10% while on treatment, and 45 (61.3%) had improved cough. At least one adverse event (AE) associated with the treatment was observed in 28 (37.3%) patients. Conclusion: Pirfenidone 200 mg was effective and well tolerated and associated with relatively mild and manageable AEs in IPF patien

Effects of the COVID-19 pandemic on the follow-up and treatment of patients with idiopathic pulmonary fibrosis: a cross-sectional, multicentre phone call survey.

OBJECTIVE: To learn about the attitudes and behaviours of patients with idiopathic pulmonary fibrosis (IPF) in relation to the difficulties experienced during the COVID-19 pandemic. DESIGN: A cross-sectional, multicentre phone call survey. SETTING: Four university hospitals in Turkey. PARTICIPANTS: The study included patients with IPF receiving antifibrotics for at least 3 months and with doctor appointment and/or scheduled routine blood analysis between March and May 2020 (the first 3 months after the official announcement of the COVID-19 pandemic in Turkey). INTERVENTIONS: Phone calls (a 5 min interview) were performed in June 2020. A questionnaire and the Hospital Anxiety-Depression Scale were applied. MAIN OUTCOME MEASURES: Patients' preferences for disease monitoring, patients' attitudes and behaviours towards IPF, drug continuation, COVID-19 diagnosis and anxiety/depression status. RESULTS: The study included 115 patients with IPF (82 male; mean age, 68.43±7.44 years). Of the patients, 73.9% had doctor appointment and 52.2% had scheduled routine blood testing; 54.5% of patients with doctor appointment self-cancelled their appointments and 53.3% of patients with scheduled routine blood testing did not undergo testing. Of the patients, 32.2% were on nintedanib and 67.8% were on pirfenidone; self-initiated drug discontinuation rate was 22.6%. The percentage of patients communicating with their physicians was 35.7%. The route of communication was by phone (34.8%). The frequency of depression and anxiety was 27.0% and 38.3%, respectively. The rates of drug discontinuation (35.1% vs 16.7%, p<0.05) and depression (37.8% vs 21.8%, p=0.07) were higher in nintedanib users than in pirfenidone users. Only two (1.7%) patients had COVID-19 diagnosis. CONCLUSIONS: During the COVID-19 pandemic, a significant proportion (>50%) of patients self-cancelled their appointments and nearly a quarter of patients discontinued their medications. Providing a documentation of the problems experienced by patients with IPF about management of the necessary requirements during the COVID-19 pandemic, this study may be a model for patients with chronic diseases.

COVID-19 patients' sera induce epithelial mesenchymal transition in cancer cells.

Covid-19 Pneumonia of SARS-CoV-2 pandemic infection, persists to have high disease burden especially in cancer patients. Increased inflammation and thromboembolic processes are blamed to influence cancer patients more than the others but due to lack of knowledge regarding the pathophysiology of the both the virus itself and the response of the host, more basic and translational disease modeling research is needed to understand Cancer-Covid-19 interaction. In this study, serum samples from the patients, who were hospitalized due to Covid-19 pneumonia, applied to different cancer cells and cytotoxicity, motility, proliferation and gene expression analysis were performed. Serum samples derived from healthy volunteers and the fetal bovine serum that is used regularly in cell culture experiments used as controls. Hospitalized Covid-19 patients who had also cancer, were retrospectively screened, and their clinical course were recorded. Overall 12 Patient (PS) and 4 healthy serums (CS) were included in the experiments. PS applied cells showed increased motility in A549 cells as well as lost cell to cell connection in MCF7 and HCT116 cells, and induced expression of VIM, ZEB1 and SNAIL2 mRNA levels. Eight cancer diagnosed patients who were hospitalized due to Covid-19 between April and September 2020 were also reviewed retrospectively, which 5 of them were dead during SARS-CoV-2 infection. Thorax CT images of the 2 patients showed increased metastatic nodules in the lungs as of January 2021. The results of the study indicate that metastasis may be one of the prolonged consequences of COVID-19 pandemic in cancer sufferers.

Morbidity and mortality of surgical lung biopsy in the diagnosis of usual interstitial pneumonia.

BACKGROUND: Histology represents the major source of information to define a usual interstitial pneumonia (UIP) pattern. However, the procedure is associated with significant morbidity and mortality. The aim of this study was to evaluate morbidity and mortality of surgical lung biopsy (SLB) in diagnosing UIP. METHODS: Patients undergoing SLB with the ultimate diagnosis of UIP were studied. Clinical data concerning medical history, histology, pulmonary functions, radiology, length of hospital stay (LOS), morbidity and mortality status were retrospectively recruited from four hospitals. RESULTS: The study included consecutive 93 patients with a SLB diagnosis of UIP. Mean age was 61 ± 8 years, with one third of the patients were ≥65 years. In 58 cases (62.4%), the biopsy was performed by video-assisted thoracoscopic surgery, in 35 (37.7%) by limited thoracotomy. Eighty patients (86%) had possible UIP, 12 (12.9%) had inconsistent with UIP and one (1.1%) had UIP pattern on high-resolution computed tomography. The mean LOS was 5.47 ± 3.16 days. LOS was associated with smoking status (P = 0.024), type of biopsy (P = 0.00), 6-min walk test (P = 0.00) and number of biopsy (P = 0.00). There was no in-hospital and 30-day mortality in our cohort, and 90-day mortality rate was 1.1%. In seven patients (7.5%), we observed postoperative morbidities, predominantly prolonged air leakage (7.5% of all cases). Postoperative morbidity was only associated with the type of SLB. Patients with limited thoracotomy showed greater morbidity rates (17.1% versus 1.7%, P = 0.011). CONCLUSION: SLB is a relatively safe procedure in the diagnosis of UIP and can be performed in suitable patients with suspected UIP/idiopathic pulmonary fibrosis.

Predictive value of plasma copeptin level for diagnosis and mortality of pulmonary embolism.

OBJECTIVE: Early diagnosis and risk stratification may provide a better prognosis in pulmonary embolism (PE). Copeptin has emerged as a valuable predictive biomarker in various cardiovascular diseases. The aim of this study was to determine the levels of copeptin in patients with acute PE and to evaluate its relationship with disease severity and PE-related death. METHODS: Fifty-four patients and 60 healthy individuals were included in this study. Copeptin concentrations and right ventricular dysfunction were analyzed. The correlation between copeptin levels and hemodynamic and echocardiographic parameters was examined. After these first measurements, patients were evaluated with PE-related mortality at the one-year follow-up. RESULTS: The copeptin levels were higher in PE patients than in the control group (8.3 ng/mL vs 3.8 ng/mL, p<0.001). Copeptin levels were found to be significantly higher in patients with PE-related death and right ventricular dysfunction (10.2 vs 7.5 ng/ml, p=0.001; 10.5 vs 7.5 ng/ml, p=0.002, respectively). When the cut-off value of copeptin was ≥5.85, its sensitivity and specificity for predicting PE were 71.9% and 85.0%, respectively (AUC=0.762, 95% CI=0.635-0.889, p<0.001). CONCLUSIONS: The copeptin measurement had moderate sensitivity and specificity in predicting the diagnosis of PE, and the copeptin level was significantly higher in patients with PE-related death at the one-year follow-up. Copeptin may be a useful new biomarker in predicting diagnosis, risk stratification, and prognosis of PE.

Predictive value of plasma copeptin level for diagnosis and mortality of pulmonary embolism

SUMMARY OBJECTIVE: Early diagnosis and risk stratification may provide a better prognosis in pulmonary embolism (PE). Copeptin has emerged as a valuable predictive biomarker in various cardiovascular diseases. The aim of this study was to determine the levels of copeptin in patients with acute PE and to evaluate its relationship with disease severity and PE-related death. METHODS: Fifty-four patients and 60 healthy individuals were included in this study. Copeptin concentrations and right ventricular dysfunction were analyzed. The correlation between copeptin levels and hemodynamic and echocardiographic parameters was examined. After these first measurements, patients were evaluated with PE-related mortality at the one-year follow-up. RESULTS: The copeptin levels were higher in PE patients than in the control group (8.3 ng/mL vs 3.8 ng/mL, p<0.001). Copeptin levels were found to be significantly higher in patients with PE-related death and right ventricular dysfunction (10.2 vs 7.5 ng/ml, p=0.001; 10.5 vs 7.5 ng/ml, p=0.002, respectively). When the cut-off value of copeptin was ≥5.85, its sensitivity and specificity for predicting PE were 71.9% and 85.0%, respectively (AUC=0.762, 95% CI=0.635-0.889, p<0.001). CONCLUSIONS: The copeptin measurement had moderate sensitivity and specificity in predicting the diagnosis of PE, and the copeptin level was significantly higher in patients with PE-related death at the one-year follow-up. Copeptin may be a useful new biomarker in predicting diagnosis, risk stratification, and prognosis of PE.

RESUMO OBJETIVO: O diagnóstico precoce e a estratificação de risco podem proporcionar um melhor prognóstico em casos de embolia pulmonar (EP). A copeptina surgiu como um valioso biomarcador preditivo de várias doenças cardiovasculares. O objetivo deste estudo é determinar os níveis de copeptina em pacientes com EP aguda e avaliar a sua relação com a severidade da doença e mortes relacionadas à EP. MÉTODOS: Um total de 54 pacientes e 60 indivíduos saudáveis foram incluídos neste estudo. As concentrações de copeptina e disfunções ventriculares direitas foram analisadas. A correlação entre os níveis de copeptina e parâmetros ecocardiográficos e hemodinâmicos foi examinada. Após essas primeiras medições, os pacientes foram avaliados em relação à mortalidade relacionada à EP após um ano. RESULTADOS: Os níveis de copeptina foram maiores em pacientes com EP do que no grupo de controle (8,3 ng/mL vs 3,8 ng/mL, p<0,001). Os níveis de copeptina eram significativamente maiores em pacientes com mortes relacionadas à EP e disfunção ventricular direita (10,2 vs 7,5 ng/ml, p=0,001; 10,5 vs 7,5 ng/ml, p=0,002, respectivamente). Com um valor de corte ≥5,85 para a copeptina, sua sensibilidade e especificidade preditivas para EP foram 71,9% e 85,0%, respectivamente (AUC=0,762, 95% IC=0,635 - 0,889, p<0,001). CONCLUSÃO: A medição da copeptina teve sensibilidade e especificidade preditivas moderadas para o diagnóstico de EP, e o nível de copeptina foi significativamente maior em pacientes com mortes relacionadas à EP após um ano. A copeptina pode ser um novo biomarcador preditivo útil para o diagnóstico, a estratificação de risco e o prognóstico de PE.

The Effectiveness, Safety, and Tolerability of Pirfenidone in Idiopathic Pulmonary Fibrosis: A Retrospective Study.

INTRODUCTION: In this study we aimed to investigate the effectiveness and safety profile of pirfenidone for the treatment of idiopathic pulmonary fibrosis (IPF) in a real-life setting. METHODS: Clinical records of patients diagnosed with mild-to-moderate IPF and receiving pirfenidone treatment across three centers in Turkey between January and September 2017 were retrospectively collected. Pulmonary function measurements, including percentage of forced vital capacity (FVC%) and percentage of diffusion capacity (DLCO%) were analyzed in patients who received pirfenidone treatment for at least 6 months. Decline in lung function, defined as an absolute decline of at least 10% in FVC from baseline, or death at 6 months was also analyzed. Safety data were included for all follow-up visits. RESULTS: In the pooled cohort (n = 60), patients were mostly men (73.4%) and current or former smokers (61.7%). Average baseline FVC% and DLCO% were 68.4% and 48.7%, respectively. Forty-seven patients (78.3%) had a high-resolution computed tomography scan with a definite interstitial pneumonia (UIP) pattern, and 18 patients (30%) had a surgically proven UIP pattern. Forty-six (76.7%) patients with IPF remained stable and 14 (23.3%) patients had progressed according to decline in FVC of at least 10% during the therapy course. After 6 months of therapy, cough decreased in 58.3% of patients. At least one side effect due to therapy was encountered in 33 (55.0%) IPF patients. Dyspepsia (36.4%), nausea (27.3%), and rash/photosensitivity (24.2%) were the most frequent side effects in our cohort. Sixteen patients (26.7%) needed dose adjustment, one patient (1.7%) discontinued therapy, and one patient (1.7%) died in the study period. CONCLUSIONS: This study shows that pirfenidone seems to be an effective treatment for IPF and also had tolerable and relatively acceptable side effects. FUNDING: Roche.

Early prediction of sarcoidosis prognosis with HLA typing: a 5 year follow-up study.

Background: A wide range of HLA-DR alleles have been associated with sarcoidosis either in terms of disease phenotype or extra pulmonary involvement, however the effect on non-resolution in different ethnic groups is not fully understood. The aim of this study was to investigate whether disease characterics and HLA-DRB1 alleles may early reflect non resolution in sarcoidosis. Methods: 91 patients who were diagnosed in Cukurova University Faculty of Medicine Department of Chest Diseases between 1993-2012 and were followed up until June 2017 were included in the study. All patients underwent HLA analysis by the Sequence Specific Oligonucleotide Prob (SSOP) method. Fifteen of them were excluded from the study group due to lost of follow-up (n=6) and not yet passed 5 years since diagnosis (n=9). Complete resolution at 5th year was defined according to the predefined standard criteria (ACCESS). Results: The resolution rate was 51.3%. The HLA-DRB1*14 allele was significantly higher in patients without resolution (11.8 vs 1.3%)(p=0.006). According to multivariate logistic regression analysis the independent risk factors of non resolution were female gender (OR: 12.6; 95%CI: 2.1-74.9, p=0.005), HLA DRB1*14 allele (OR:51.9; 95%CI: 3.6-735.8, p=0.000), baseline TLCO<75%(predicted) (OR:3.8; 95%CI: 1.1-13.7, p=0.028), extra-pulmonary involvement (OR:3.7; 95%CI: 1.0-13.1, p=0.038) and advanced stage at baseline (OR: 8.3; 95%CI: 1.9-35.4, p=0.001). Conclusions: HLA-DRB1*14 alleles, lower baseline TLCO, advanced stage, female gender or the presence of extra-pulmonary involvement could predict long term non-resolution in sarcoidosis. Early prediction of long term prognosis may affect treatment decisions and avoid further deterioration in these patient groups. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 184-191).

Ultrasound Accelerated Thrombolysis May Be an Effective and Safe Treatment Modality for Intermediate Risk/Submassive Pulmonary Embolism.

Pulmonary embolism (PE) is a potentially life-threatening condition and the fact that 90% of PE originate from lower limb veins highlights the significance of early detection and treatment of deep vein thrombosis. Massive/high risk PE involving circulatory collapse or systemic arterial hypotension is associated with an early mortality rate of approximately 50%, in part from right ventricular (RV) failure. Intermediate risk/submassive PE, on the other hand, is defined as PE-related RV dysfunction, troponin and/or B-type natriuretic peptide elevation despite normal arterial pressure. Without prompt treatment, patients with intermediate risk PE may progress to the massive category with a potentially fatal outcome. In patients with PE and right ventricular dysfunction (RVD), in hospital mortality ranges from 5% to 17%, significantly higher than in patients without RVD.

Could HLA-DR B1*11 allele be a clue for predicting extra-pulmonary sarcoidosis?

BACKGROUND: Several HLA-DR alleles have been described as a potential risk factor for sarcoidosis between distinct ethnic groups however the relationship between HLA-DR alleles and extra-pulmonary sarcoidosis (EPS) is still scarce. OBJECTIVES: The aim of this prospective study is to investigate the relationship between extra-pulmonary involvement and HLA-DR genetic analysis in Turkish patients with sarcoidosis. METHODS: In this study, we HLA-typed sarcoidosis patients with and without extra-pulmonary involvement, and compared with healthy control subjects. The presence of EPS was evaluated with previously defined standard criteria (ACCESS) and only patients with definite and probable involvement were accepted as positive. Sequence Specific Oligonucletide Probes method was used for typing of HLA-DRB1 alleles from DNA samples in both groups. RESULTS: The frequency of HLA DRB1*15 allele was more frequent in patients with sarcoidosis than controls (% 20.4 vs % 9.6)(pcorr=0.017). According to multivariate analysis (MVA), the presence of HLA DRB1*15 was indicated as an independent risk factor for sarcoidosis (OR:2.37; 95% CI: 1.31-4.30, p=0.004). Extra-pulmonary involvement was present in 39 patients (42.9 %). When the patients with and without extra-pulmonary involvement compared, HLADRB1*11 allele was significantly higher in patients without extra-pulmonary sarcoidosis which may be concluded as a protective allele for systemic involvement (%30.8 vs. %15.4)(p<0.05). This result was also confirmed with the MVA (OR:0.35, %95 CI:0.15-0.84, p=0.018). CONCLUSIONS: We demonstrated a strong positive link between the haplotype HLA DRB1*15 and sarcoidosis in a Turkish Caucasian population and a potential protective effect of HLA DRB1*11 from extra-pulmonary involvement of disease.

[Endobronchial capillary hemangioma: a very rare cause of massive hemoptysis]. / Masif hemoptizinin nadir bir nedeni: Endobronsiyal kapiller hemanjiyom.

Tracheal capillary hemangioma is a very rare benign tumor of trachea which may present as massive hemoptysis. Minor to massive hemoptysis can be observed in these patients. Due to its small size and tracheal localization, diagnosis cannot be easily performed by using radiological investigations. Fifty-years-old male patient who was diagnosed as tracheal capillary hemangioma with bronchoscopic biopsy was presented in this case report. According to our knowledge, this is the eighth case report in the world literature. Tracheal capillary hemangioma must be kept in mind in patients with massive hemoptysis with normal radiologic features and bronchoscopic procedures (excision, argon, laser etc.) should be the first choice of therapy when diagnosed.

Detection of latent tuberculosis infection in rheumatologic diseases before anti-TNFα therapy: tuberculin skin test versus IFN-γ assay.

We aimed to evaluate tuberculin skin test (TST) and interferon-gamma (IFN-γ) test results for latent tuberculosis infection (LTBI) in patients with rheumatologic diseases prior to anti-TNFα therapy. Ninety patients were evaluated in the study at the Departments of Chest Diseases and Rheumatology for anti-TNFα therapy for their rheumatologic diseases. Tuberculin skin test was performed (Mantoux method) and peripheral blood samples were collected for IFN-γ assay (QuantiFeron TB-Gold In Tube) before the anti-TNFα therapy. Of 90 patients, TST positivity was detected in 56 (62.2%) patients, while IFN-γ positivity was detected in 34 (37.8%) patients. Among 56 TST positive patients, IFN-γ positivity was detected in 24 (42.9%) patients, and among 34 TST negative patients, IFN-γ positivity was detected in 10 (29.4%) patients. There was no significant agreement between TST and IFN-γ assay results (Kappa = 0.12, P = 0.2). Forty-three (47.8%) patients were using immunosuppressive drugs owing to their rheumatologic diseases. In this group, TST and IFN-γ positivity is significantly lower than in those who did not receive immunosuppressive treatment (P < 0.05). We conclude that the IFN-γ assay may not be preferred to TST as a diagnostic test in patients with rheumatologic diseases prior to anti-TNFα treatment.