Activity

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. OTS964 mouse 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.Background Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is often fatal. Although pharmacological treatments have been studied, outcomes remain poor. This study evaluated the effectiveness of pharmacological treatments for AE-IPF. Methods This retrospective study comprised 88 patients who received a diagnosis of AE-IPF and were admitted to our center during the period from January 2008 through April 2017. We reviewed the clinical features, treatments, and outcomes of the 88 patients. Cox proportional hazards regression analysis was used to identify variables that were significant predictors of 3-month death. Results Data from 88 AE-IPF patients (age range, 56-81 years) were analyzed. In all patients, corticosteroid (CS) pulse therapy was performed an average of 1.7 times, and the initial CS maintenance dose was 1 mg/kg for 65 patients and 0.5 mg/kg for 23 patients. The combination treatments received were sivelestat in 83 patients (94%), recombinant human thrombomodulin (rhTM) in 45 patients (51%), pirfenidone in 41 patients (47%), and cyclosporine in 71 patients (81%). Univariate analysis showed that use of rhTM, and an initial CS maintenance dose of 0.5 mg/kg were associated with better 3-month survival. In multivariate analysis, both use of rhTM and an initial CS maintenance dose of 0.5 mg/kg were associated with better 3-month survival. Other treatments, including sivelestat, cyclosporine, pirfenidone, and polymyxin B-immobilized fiber column-direct hemoperfusion, were not associated with better 3-month survival. Conclusion Addition of rhTM to CS, and a low initial CS maintenance dose (0.5 mg/kg), were associated with better 3-month survival in patients with AE-IPF.Purpose Family-based genetic linkage analysis and genome-wide association studies (GWASs) have identified many genomic loci associated with primary open-angle glaucoma (POAG). Several causative genes of POAG have been intensively analyzed by sequencing in different populations. However, few investigations have been conducted on the identification of variants of coding region in the genes identified in GWASs. Therefore, further research is needed to investigate whether they harbor pathogenically relevant rare coding variants and account for the observed association. Methods To identify the potentially disease-relevant variants (PDVs) in POAG-associated genes in Chinese patients, we applied molecular inversion probe (MIP)-based panel sequencing to analyze 26 candidate genes in 235 patients with POAG and 241 control subjects. Results The analysis identified 82 PDVs in 66 individuals across 235 patients with POAG. By comparison, only 18 PDVs in 19 control subjects were found, indicating an enrichment of PDVs in the POAG cohort (28.