This study demonstrated that the prognosis of CPFE is significantly worse than that of IPF alone. In particular, CPFE with paraseptal emphysema associated with high esPAP has an extremely poor prognosis.
Background:Germline alterations in the proapoptotic protein Bcl-2–like 11 (BIM) can have a crucial role in tumor response to treatment. To determine the clinical utility of detecting BIM deletion polymorphism in non–small-cell lung cancer positive for epidermal growth factor receptor (EGFR) mutation, we examined outcomes of patients with and without BIM alterations.Methods:We studied 70 patients with EGFR mutation-positive non–small-cell lung cancer who were treated with an EGFR tyrosine kinase inhibitor between January 2008 and January 2013. BIM deletion was analyzed by polymerase chain reaction in 58 samples of peripheral blood and 24 formalin-fixed paraffin-embedded slides of surgical specimens (20 of lung tissue and four of brain tissue); both blood and tissue specimens were available for 12 patients. We retrospectively analyzed clinical characteristics, response rate, toxicity, and outcomes among patients with and without BIM deletion.Results:BIM deletion was present in 13 of 70 patients (18.6%). There were no significant differences between patients with and without BIM deletion in clinical characteristics, rate of response to EGFR tyrosine kinase inhibitor, or incidence of adverse events. Patients with BIM deletion had significantly shorter progression-free survival (PFS) than those without BIM deletion (median, 227 versus 533 days; p < 0.001). Multivariate Cox regression analysis showed that BIM deletion was an independent indicator of shorter PFS (hazard ratio, 3.99; 95% confidence interval, 1.864–8.547; p < 0.001).Conclusions:Polymerase chain reaction successfully detected BIM deletion in samples of peripheral blood and formalin-fixed paraffin-embedded slides of surgical specimens. BIM deletion was the most important independent prognostic factor in shorter PFS.
These results suggested that COPD of Japanese patients may develop partly because of oxidative stress derived from a shortage of antioxidant nutrients, especially of AA and lycopene, as well as GSH while this may not be the case in both ACOS and BA.
Cyclosporine, a calcineurin inhibitor, is a potent immunosuppressive agent that acts chiefly through the inactivation of T-lymphocytes. Several clinical studies have demonstrated the effectiveness of cyclosporine for treating fibrotic lung disease, but the underlying mechanism remains elusive. We hypothesized that cyclosporine exerts direct effects against fibrogenesis of lung myofibroblasts, and aimed to elucidate the mechanism of this anti-fibrotic effect through gene-expression profiling using DNA microarray analysis. We found that cyclosporine suppressed the expression of alpha-smooth muscle actin and collagen type I in myofibroblasts that had been differentiated from a fetal human lung fibroblast cell line by induction with transforming growth factor (TGF)-β. Furthermore, microarray analysis revealed that cyclosporine downregulated 57 genes whose expression levels were increased by TGF-β, and up-regulated 73 genes, whose expression was decreased by TGF-β. Classifying these 57 down-regulated and 73 up-regulated genes with the Database for Annotation, Visualization and Integrated Discovery (DAVID) web tool, we have identified the involvement of several functional categories, including innate immunity, cytokine interaction, growth factor, and cancer pathway. Of the identified genes, we selected three fibrosis-related genes, insulin-like growth factor binding protein 2 (IGFBP2), inhibitor of DNA binding 1 (ID1) and peroxisome proliferator-activated receptor gamma (PPARG), and validated their expression patterns by quantitative reverse transcription-polymerase chain reaction. Cyclosporine treatment decreased the expression levels of IGFBP2 and ID1, but increased PPARG expression. These results suggest that cyclosporine is a potent anti-fibrotic agent acting on myofibroblasts. Therefore, cyclosporine shows potential as a novel remedy for fibrotic lung disease.
Immunoglobulin G4 (IgG4)-related lung diseases can occur in patients with autoimmune pancreatitis (AIP). However, the causal relationship between AIP and acquired hemophilia A (AH) is unknown. We herein report the first case of AH associated with IgG4-related lung disease that developed in a patient with AIP. A 65-year-old asymptomatic man with a history of AIP and sclerosing cholangitis diagnosed at the age of 57 was admitted to our hospital due to an abnormal reticulonodular shadow on chest X-ray. An examination of lung biopsy specimens revealed IgG4-positive plasma cell infiltration in the interstitium. The serum IgG4 level was elevated. One year later, the patient developed a progressive severe hematoma in the left femoral muscle. On admission, laboratory examinations revealed severe anemia with a markedly prolonged activated partial prothrombin time, a decreased level of factor VIII (FVIII) activity, and the existence of anti-FVIII antibodies. These findings were consistent with a diagnosis of AH. No relapse has been observed over the past 25 months, during which time, corticosteroid therapy has been continuously administered. Case ReportDiffuse abnormal shadows were evident on a chest X-ray performed as part of a routine health check-up in a 65-yearold man. The patient developed bronchial asthma at 10 years of age and had also suffered from autoimmune pancreatitis (AIP), sclerosing cholangitis and diabetes mellitus since 57 years of age. A laboratory examination conducted on admission showed high levels of total protein (10.8 g/ dL), immunoglobulin (Ig)G (8,688 mg/dL, normal range: 703-1,540), IgG4 (3,920 mg/dL, normal: <105), IgE (2,560 IU/mL, normal: <173) and sIL-2R (1,570 U/mL, normal range: 220-530). The results of peripheral blood tests were as follows: white blood cell count: 9,000/μL with 18.0% eosinophils, hemoglobin: 10.8 g/dL and platelet count: 325,000/μL. Multiple nodular lesions with thickening of bronchovascular bundles were evident in both lung lobes (Fig. 1). Lung biopsy specimens of the left S 1+2 c obtained with video-assisted thoracoscopic surgery revealed thickening of the alveolar walls with fibrosis and inflammatory cell infiltration corresponding to ground glass opacity and a subpleural small nodular lesion observed on high-resolution chest CT (HRCT) (Fig. 2). Lung biopsy specimens of the left S 10 c revealed a subpleural nodule containing marked lymphoplasmacytic infiltration with irregular fibrosis, narrowed bronchioli surrounded by lymphoplasmacytic infiltration and obliterative phlebitis and arteritis accompanied by intimal and mural inflammatory cell infiltration corresponding to the subpleural nodular lesion with spiculation observed on the chest HRCT. In addition, immunohistochemical staining for IgG4 revealed numerous IgG4-positive
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