Predictive factors of mortality in patients with idiopathic pulmonary fibrosis treated with antifibrotics: a novel prognostic scoring system.

BACKGROUND AND AIM: Any test that provides sufficient prognostic information to guide treatment decisions in idiopathic pulmonary fibrosis (IPF) is not available. The aim of our study was to determine the predictive factors of mortality in patients with IPF treated with antifibrotics. METHODS: Patients with diagnosis of IPF who were treated with antifibrotics between 2016 - 2021 were included in the study. Demographic, clinical and laboratory characteristics of the patients was derived from hospital records retrospectively. Kaplan Meier and multivariate cox regression analysis were achieved for detection of mortality predictors. RESULTS: Study population was composed of 119 IPF patients with a male predominance of 80.7% (n=96). Mean age of the patients was 67.9 ± 7.07 years. On univariate analysis, sex was not a significant predictor of mortality (HR 1.79; 95% CI: 0.87 - 3.69, p =0.11).  BMI ≤ 26,6 m2/kg, DLCO ≤ 3.11 ml/mmHg/min, age over 62 years, 6DWT ≤ 382 meters, NLR ≤ 2.67 and PDW ≤ 16.7% were found to be significant for predicting mortality. On multivariate cox regression analysis four parameters remained significant for prediction of mortality: RDW > 14%, NLR ≤ 2.67, BMI ≤ 26,6 m2/kg and DLCO ≤ 3.11 ml/mmHg/min (respectively, HR: 2.0. 95% CI: 1.02 - 3.91, p=0.44; HR: 2.68. 95% CI: 1.48 - 4.85, p=0.001, HR: 2.07. 95% CI: 1.14 - 3.76, p=0.02, HR: 3.46. 95% CI: 1.85 - 6.47, p<0.001). A scoring system with these parameters discriminated patients with worse prognosis with a sensitivity of 89.1 % and a specificity of 65.8 % when total point was over 2 (AUC0.83, p<0.001). Conclusions In this study, DLCO, BMI, RDW and NLR levels significantly predicted mortality in IPF patients. Along with GAP index, scoring system with these simple parameters may give information about the prognosis of an IPF patient treated with antifibrotics.

The comparison of exacerbation and pneumonia before and after conjugated pneumococcal vaccination in patients with chronic obstructive pulmonary disease, and the effect of inhaled corticosteroid use on results.

Introduction: Pneumococcal infections and exacerbations are important causes of mortality and morbidity in chronic obstructive pulmonary disease (COPD). The use of inhaled corticosteroids and pneumococcal vaccination are suggested for the control of the disease progression and exacerbations. The aim of this study is to assess the effect of pneumococcal conjugate vaccine on pneumonia and exacerbation in COPD patients using inhaled corticosteroids (ICSs). The secondary aim is to analyze the effect of ICS use and different ICS types, if administered, on exacerbation and pneumonia incidence in the study population. Materials and Methods: Medical records of 108 adult patients with COPD who were vaccinated with the pneumococcal conjugate vaccine (PCV13) were retrospectively evaluated. The number of acute exacerbations and pneumonia within one year before and after vaccination were evaluated in all included COPD patients. The comparison analysis was also performed based on the ICS types. Result: There were statistically significant differences between the mean numbers of pneumonia and exacerbations before and after vaccination (p<0.05). There were no significant differences in the mean pneumonia attacks and acute exacerbations between patients using ICS and not using ICS (p> 0.05). Conclusions: This study revealed that PCV13 provides a significant decrease in both exacerbation and pneumonia episodes in COPD patients. On the other hand, the use of ICSs and the types of ICSs were not found to have adverse effects on pneumonia and acute exacerbations in vaccinated COPD patients.

Clinical Differences Between Elderly and Non-elderly Patients with COVID-19.

OBJECTIVE: Comorbidity frequency and mortality rates are higher in elderly patients with COVID-19. The disease is also more severe in elderly patients. This study aims to examine the characteristics of the COVID-19 disease, severity, comorbidities, and mortality rates in elderly patients by comparing them with nonelderly patients. MATERIAL AND METHODS: This study was designed as a retrospective study. 469 patients who were followed up in outpatient, inpatient, and intensive care units with the diagnosis of COVID-19 between March 11, 2020, and June 01, 2020, were retrospectively included in the study. Patients were divided into two groups who were ≥65 years named as the "elderly group" and <65 years referred to as "nonelderly". Survival data was generated from the death notification system on August 02, 2020. RESULTS: A total of 469 patients including elderly(n=101) and nonelderly(n=368) were included in the study retrospectively. The inci- dence of severe pneumonia(31%/12.6%) and critical illness(16%/5.8%), comorbidity (85%/37.2%) and hospitalization time(8/5 days) were significantly higher in the elderly group(p<0.05). 23 (22.8%) of elderly patients and 27(7.3%) of nonelderly patients died (p=0.000). Mortality was found to be 3.5 times higher than in the non-elderly group. The expected survival time was 145.85 days(CI 95%:133- 158.66) in the elderly patients and 170.36 days(CI 95%:166-174.6) in the nonelderly patients (p<0.000). In ROC analysis, the sensitivity of age was 86%(73.3-94.2), specificity was 66.83%(62.1-71.3), and the cut-off>56 (AUC:0.775; p <0.001) in predicting mortality. CONCLUSION: Mortality is high, comorbidities are more frequent, and the disease is more severe in elderly patients with COVID-19. Age above 56 can be used as a cut-off to predict mortality.

Research Burden of Interstitial Lung Diseases in Turkey - RBILD.

Introduction: The aim of our study is to investigate the etiological distribution of ILD in Turkey by stratifying the epidemiological characteristics of ILD cases, and the direct cost of initial diagnosis of the diagnosed patients. Material-Method: The study was conducted as a multicenter, prospective, cross-sectional, clinical observation study. Patients over the age of 18 and who accepted to participate to the study were included and evaluated as considered to be ILD. The findings of diagnosis, examination and treatment carried out by the centers in accordance with routine diagnostic procedures were recorded observationally. Results: In total,1070 patients were included in this study. 567 (53%) of the patients were male and 503 (47%) were female. The most frequently diagnosed disease was IPF (30.5%). Dyspnea (75.9%) was the highest incidence among the presenting symptoms. Physical examination found bibasilar inspiratory crackles in 56.2 % and radiological findings included reticular opacities and interlobular septal thickenings in 55.9 % of the cases. It was observed that clinical and radiological findings were used most frequently (74.9%) as a diagnostic tool. While the most common treatment approaches were the use of systemic steroids and antifibrotic drugs with a rate of 30.7% and 85.6%, respectively. The total median cost from the patient's admission to diagnosis was 540 Turkish Lira. Conclusion: We believe that our findings compared with data from other countries will be useful in showing the current situation of ILD in our country to discuss this problem and making plans for a solution.

Original Article: Clinical Research.

AIM: In this study, we aimed to investigate the possible role of endotrophin, a profibrotic byproduct of collagen VI, in the complex process of fibrosis development in the disease group with pulmonary fibrosis among interstitial lung diseases. MATERIAL AND METHOD: When the patients' participation in the study were completed, smoking or alcohol drinking conditions, and family history were recorded. Their weights and heights were recorded and body mass index (BMI) was calculated. In every patient, Spirometry with bronchodilator testing, determination of single-breath DLCO, and plethysmographic measurement of thoracic gas volume and airway resistance were performed. Blood samples were obtained for the inflammation markers such as sedimentation rate, C-reactive protein (CRP), complete blood count, liver and renal function tests, and lactate dehydrogenase levels. Serum endotrophin levels were measured in all patients. RESULTS: Thirty-five patients with interstitial lung disease who were having pulmonary fibrosis, 35 patients with interstitial lung disease without pulmonary fibrosis, and 20 control patients without any signs or symptoms of interstitial lung disease were included in the study. Age distribution was similar between groups. The fibrotic ILD group was more commonly smoker or ex-smoker compared with the non-fibrotic ILD patients or control cases. Fibrotic ILD patients were leaner, having significantly decreased total lung capacity, diffusion capacity, and higher LDH levels. In the comparison of the 3 study groups regarding the endotrophin levels, there was a significant difference between groups. The fibrotic and non-fibrotic patient groups were compared for the Endotrophin levels and the difference was also significant. However, there was not any significant difference regarding the endotrophin levels between control cases and non-fibrotic ILD patients. Smoked cigarette pocket x year showed a significant positive correlation and DLCO % and KCO % showed a significant negative correlation with the endotrophin levels. CONCLUSION: Serum endotrophin levels significantly increase in fibrotic ILD patients compared with the non-fibrotic ILD patients and control cases. Endotrophin may be suggested as a diagnostic marker in fibrotic interstitial lung diseases.

A comparison trial of eight weeks versus twelve weeks of exercise program in interstitial lung diseases.

Background: Exercise training have been shown to be the effective approach for functional outcomes in interstitial lung diseases (ILD). In many studies, the duration of exercise programs (EPs) varies between 8-12 weeks. However, the optimal duration of EPs is still unknown. Objective: In our prospective non-controlled study, we aimed to compare the results of the 8th week with the results of the 12th week of the PR programs applied to the patients with ILD. Methods: A total of 14 patients [Age; 63(53,70) years, body mass index: 28(25,32) kg/m2, disease duration; 1.5 (1,4) years] with ILD [11 idiopathic pulmonary fibrosis, 2 sarcoidosis (stage 3 and 4) and 1 nonspecific interstitial pneumonia] were included in the study. 6-minute walk test, pulmonary function test, arterial blood gas analysis, mMRC dyspnea scale, quality of life questionnaires and hospital anxiety depression scale were performed at before and 8 and 12 weeks after the program. Results: 6-minute walk distance, dyspnea, anxiety, depression and quality of life improved both at 8th and 12th week after EP when compared the with the initial assessment(P<0.05). When compared with 8th week; mMRC dyspnea score, 6-minute walk distance and quality of life scores significantly improved at 12th weeks (P=0.046, P=0.016, P<0.05, respectively). Conclusions: Prolonging duration of the EPs results in more improvement in functional outcomes in patients with ILD. However, it has no effect on pulmonary functions and arterial blood gas results. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 299-307).

[Leptomeningeal metastasis of EGFR (+) lung adenocarcinoma: a case report]. / EGFR (+) akciger adenokarsinomunun leptomeningeal metastazi: Olgu sunumu.

Leptomeningeal metastasis is a very rare complication of infiltration of leptomeninges and subarachnoid space with malignant cells. It is an indicator of poor prognosis. Its incidence is 3.8% in non-small cell lung carcinoma (NSCLC). This rate is higher in patients with epidermal growth factor receptor (EGFR) mutation. Brain magnetic resonance imaging (MRI) is the first choice in the diagnosis. The diagnosis of leptomeningeal metastasis is difficult and often bypassed because it is rare and does not cause gross mass lesions such as brain metastasis. Systemic chemotherapy, intrathecal therapy, cranial radiotherapy and targeted treatment agents are an option in the treatment. It has been shown that targeted therapies can be promising because of the ability to switch to cerebrospinal fluid in appropriate patients. We present the case with EGFR positive lung adenocarcinoma whit leptomeningeal metastasis (LM) due to its rarity, difficulty in diagnosis and its association with EGFR mutation.

[A case of endometrium adenocarcinoma with multiple cavitary pulmonary metastasis]. / Multipl kaviter akciger metastazi yapan endometriyum adenokarsinom olgusu.

Pulmonary metastases are usually seen as parenchymal nodules, lymphatic-interstitial spread and pleural effusion, however cavitary and cystic metastatic lesions are rare. While breast cancer, gastrointestinal tumors, kidney tumors, malignant melanoma, sarcomas, lymphoma and leukemia mostly metastasize to the lung, endometrial adenocarcinoma rarely metastasizes. A 73-year-old woman with multiple cystic-cavitary lung lesions was referred to our clinic because of chronic cough. She had endometrial adenocarcinoma metastasis to the lung. We wanted to present our case because of the rare occurrence of cystic-cavitary lung metastases and the rare presentation of metastasis of endometrial carcinomas to the lung.

The incidence of chronic thromboembolic pulmonary hypertension secondary to acute pulmonary thromboembolism.

INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) is a curable and partially preventable complication, with a substantial incidence, leading to severe morbidity and mortality. The aim of the present study was to find out the incidence of CTEPH secondary to acute pulmonary thromboembolism (PTE) using non-invasive procedures such as ventilation/perfusion (V/Q) scintigraphy and pulmonary multidetector CT (MDCT) angiography in determining the diagnosis of CTEPH. MATERIALS AND METHODS: The study included a total of 99 patients diagnosed with initial PTE between January 2010 and December 2012. The patients who received anticoagulant therapy at least for three months underwent transthoracic echocardiography (TTE) (n= 85). Thirty one patients with a SPAP value > 30 mmHg and/or an evidence of right ventricular dysfunction in TTE underwent MDCT pulmonary angiography and V/Q scintigraphy. The patients with an evidence of residual chronic thromboembolic signs in MDCT pulmonary angiography and/or segmental perfusion defect(s) in V/Q scintigraphy underwent right heart catheterization (RHC) (n= 7). The mean PAP was measured, and a vasoreactivity test was performed. During RHC, a non-contrast medium was delivered to the pulmonary arteries for pulmonary arteriography imaging. RESULTS: Among patients diagnosed with PTE, 44 were male and 55 were female. The mean age was 60 ± 17 years. Of these patients, 63.6% had history of at least one additional disease and at least one risk factor for PTE. During diagnosis, 24 subjects were considered having massive, 61 submassive and 14 non-massive PTE. Nineteen (19.1%) patients received thrombolythic therapy. Other 80 (80.8%) patients received standard anticoagulant therapy with an INR value within the therapeutic range. In 79.8% of patients, thromboembolism was bilateral, and it was unilateral in 21.8%. After a minimum of 1 year, and maximum of 2 years follow up five subjects (5.5%) were diagnosed with CTEPH. The univariate analysis showed no association between the development of CTEPH and factors like; age, etiologic risk factors for PTE, receiving thrombolytic treatment, prevalence and type of PTE. CONCLUSION: Potentially preventabl complication of pulmonary embolism; CTEPH, had a substantial incidence during follow-up.