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238) or APACHE II (P = .918) scores on admission, and did not correlate with survival (Kruskal-Wallis test, P = .937). However, higher serum procalcitonin levels were associated with patients who had positive blood cultures (Kruskal-Wallis test, P = .0016 for Gram-positive and P = .0007 for Gram-negative bacteria). Lactic acid levels on admission strongly correlated with SOFA APACHE II (the Spearman correlation, P 30 ng/mL do not appear to correlate with the severity of disease in a sample of patients with markedly elevated initial procalcitonin levels.Introduction Considering limited data about the association between smoking and primary systemic vasculitides (PSV), present study aims to investigate smoking habit in PSV patients compared to healthy subjects as well as to examine the effect of smoking on clinical characteristics, disease activity and disease outcome in PSV patients. Methodology We included 126 patients diagnosed with PSV and 210 age- and sex-matched healthy controls. Demographic and clinical information and smoking history of patients and healthy controls were obtained by direct interview and questionnaire. Individuals who had smoked at least 100 cigarettes in their lifetime before the first symptom of vasculitis were classified as smokers; those who had never smoked or smoked less than 100 cigarettes in their lifetime were categorized as never smokers. Disease activity was evaluated by Birmingham Vasculitis Activity Score (BVAS). Disease outcome was assessed by vasculitis damage index (VDI) and the number of patients with disease in remissking in Azeri population. Although further studies are needed to confirm these preliminary results, it seems that smoking may not be a significant risk factor for PSV.This original research is a directional study that determined the habits of individuals using four analyses to find statistical significance in the data collected from the surveys of 801 qualified of 1,340 individuals who agreed to participate. Results from the self-reported diagnosis of individuals affected by sarcoidosis produced seven statistically significant indicators of future research needed. The demographics revealed a significantly greater number of women and African-Americans participants than other minorities in the United States and suggested a sense of urgency to find a cure. Most important are the seven statistically significant findings that also gave credence to the researchers' four subdiagnostic classifications. They are acute sarcoidosis (AS) and chronic sarcoidosis with limited dissemination (CSLD), while more severe cases include those with chronic sarcoidosis with full dissemination including cutaneous involvement (CSFDIC) and chronic sarcoidosis with neurosarcoidosis (CSN). The most severe sarcoidosis cases (CSN) were on the "most likely" side of every statistically significant category except drinking alcohol, and the "least likely" to participate in physical activities. Conversely, the least severe case of sarcoidosis (AS) was the opposite. The complete list of statistically significant areas was related to alcohol use, tobacco use, ciprofloxacin use, environmental exposure to metals (copper, iron), infectious diseases (candidiasis), genetics, and physical exercise. Statistically, the most crucial study needed; emerged from the Rh blood grouping of the participants.Background Pulmonary sarcoidosis patients who get disease progression despite corticosteroid treatment or can't tolerate corticosteroid required second-line drug. Methotrexate (MTX) is the most widely used in our clinical practice. Data on its safety and efficacy at different doses are still limited, especially for those without folic acid supplements. Objective To report effectiveness of different MTX dosages and tolerability of MTX in pulmonary sarcoidosis without folic acid supplements. Methods A retrospective study on pulmonary sarcoidosis patients receiving MTX therapy with various dose ≥3 months was conducted. The primary outcome was change in high-resolution computed tomography (HRCT) before and after MTX therapy. Other efficacy parameters included SGRQ score, prednisone dose change, discontinuation and relapse-free survival. Response-linked factors and safety outcomes were also analyzed. CM272 Results Overall, 49 patients (81.7%) were assessed as MTX responders by HRCT and there was no significant differenctinuing MTX therapies. The steroid-sparing effect, well drug tolerability and low drug withdrawal rate were observed in these patients even without folic acid supplements in clinical practice.Background The role of cytomorphology in differentiating sarcoidosis from tuberculosis is not fully elucidated. Herein, we evaluate the utility of cytological features in differentiating between these two diseases in subjects undergoing endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA). Methods Retrospective analysis of subjects who underwent EBUS-TBNA and had a final diagnosis of sarcoidosis or tuberculosis. The final diagnosis was based on the clinicoradiological features, microbiology and clinical course during follow-up (including response to treatment) at six months. A cytologist blinded to the clinical details and microbiology examined the aspirates. The primary outcome was the diagnostic accuracy of cytologist's impression to diagnose sarcoidosis as compared to the final diagnosis. Results 179 (145 sarcoidosis, 34 tuberculosis) subjects were included. Granuloma was identified in 135 (75.4%) subjects; amongst these, the cytologist made a correct diagnosis in 62.2% cases, misdiagnosed 28.9% cases, and in 8.9% cases differentiating sarcoidosis from tuberculosis was not possible. The sensitivity, specificity, positive and negative predictive values (PPV and NPV) of the cytologist in diagnosing sarcoidosis was 62%, 64%, 90%, and 25%, respectively. The identification of a non-necrotic granuloma, along with a negative TST and the lack of endosonographic features favouring tuberculosis (heterogeneous echotexture and coagulation necrosis sign), provided the best specificity (97%) and PPV (99%) to diagnose sarcoidosis. Conclusion Sarcoidosis cannot be reliably differentiated from tuberculosis based on cytomorphology alone. A combination of clinical features, endosonography, cytology and microbiology is required for accurate diagnosis.Objectives Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is one of antineutrophil cytoplasmic autoantibody (ANCA) - associated vasculitis. In patients with GPA an increased incidence of venous thromboembolism (VTE), mainly during active disease, has been described. The aim of the present study was to assess the incidence of VTE and its relation with classic risk factors for atherosclerosis, presence of coronary artery disease (CAD), echocardiographic parameters and laboratory findings in GPA patients. Methods The group of consecutive patients with GPA were followed in the study. In all patients echocardiography and laboratory tests were performed. Results Ninety six patients with GPA were followed for mean 3 years. In 16 patients (16.6%) VTEs occurred in association with GPA, of which 56% occurred 6 months before or one year after diagnosis of GPA. Classic risk factors for atherosclerosis were present in 77 patients (80.2%) at some moment during follow-up. In patients with VTE there were larger right ventricle diameter (p=0.041) and higher right ventricle systolic pressure (p=0.022) observed. VTEs occurred significantly less frequently in patients treated with cyclophosphamide (p=0.049). In this study group VTE occurred more frequently than CAD 16 (16.7%) vs. 4 (4.2%); p=0,0049. Patients with VTE were younger than those with CAD (p=0.053) and had higher levels of ANCA-PR 3 (p=0.016). Conclusions Patients with granulomatosis with polyangiitis in first years after diagnosis have higher risk of venous thromboembolism than coronary artery disease. This finding is probably related to hypercoagulability induced by the disease and its therapy.Background The association between interstitial lung disease (ILD) and myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCAs) has been described, but pathologic characteristics are not well characterized. Objectives We assessed the radiologic and pathologic characteristics of ILD in MPO-ANCA-positive patients and the association between ILD and vasculitis, particularly microscopic polyangiitis (MPA). Methods We retrospectively searched electronic health records to identify MPO-ANCA-positive patients with ILD who underwent surgical lung biopsy at our institution from January 1997 through August 2017. Demographic, clinical, imaging, and pathologic characteristics were analyzed. Results We identified 18 MPO-ANCA-positive patients with ILD. The median (range) age was 58 (43-75) years, and the cohort included 10 men (56%), 10 former smokers (56%), and 11 patients (61%) had clinical evidence of systemic vasculitis (MPA) at the time of diagnosis of ILD. On high-resolution computed tomography, the most common radiologic pattern was "inconsistent with usual interstitial pneumonia" (UIP) (n=14 [78%]); the other 4 patients (22%) fulfilled the radiologic criteria for the UIP pattern. Honeycombing was seen in 15 patients (83%). Ten patients (56%) had the UIP pattern on biopsy 4 of these patients had additional inflammatory changes that were not typical of UIP (as seen in patients with idiopathic pulmonary fibrosis), and the other 6 patients had other inflammatory patterns or findings. The presence or absence of MPA did not correlate with pathologic findings. Conclusions MPO-ANCA-positive patients with ILD do not show the typical UIP pattern as seen in patients with idiopathic pulmonary fibrosis on surgical lung biopsy.Background The 6-minute walking test, cardiopulmonary exercise testing, and spirometry are useful tools for evaluation of respiratory impairment and functional capacity in patients with lung disease. Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Objectives Since the pulmonary involvement can affect the quality of life in sarcoidosis patients, this study is aimed to evaluate the tests mentioned above in order to examine the functional capacity of sarcoidosis patients in different stages as well as the cause of exercise intolerance. Methods This cross-sectional study was carried out on 50 Iranian patients with sarcoidosis. Patients were classified into three groups based on the findings of the chest radiography as well as the pulmonary CT scan, reported by an expert radiologist. Pulmonary, cardiac, and activity function have been evaluated in the patients, using cardiopulmonary exercise testing, the 6-minutes walking test, and spirometry. Results In cardiopulmonary exercise testing, percent-predicted peak VO2 (57.75±15.49, p=0.015) and percent-predicted O2 pulse (70.54±17.37, p=0.013) were significantly lower in the third group, in comparison with the others. Also, VE/CO2 (AT) (34.99±5.67, p=0.000) was significantly higher in the third group, in comparison with the other ones. Percent-predicted VO2 showed a strong positive correlation with age (r=0.377, p=0.009), TSH (r= 0.404, p=0.007), and percent-predicted FVC (r=0.443, p=0.002). In addition, O2 pulse had a positive correlation with BMI (r=0.324, p=0.026), percent-predicted FVC (r=0.557, p= 0.000), and percent-predicted FEV1 (r=0.316, p=0.032). Conclusions According to this study, ventilatory limitation, pulmonary involvement, and deconditioning are the main causes of activity limitations in sarcoidosis patients.

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