IgG4-related disease (IgG4-RD) is a recently recognized systemic disease characterized by tumefactive lesions in various organ systems. The list of organs that can be involved continues to expand, and recently computed tomography (CT) descriptions of the pulmonary lesions found in the disease have been described. The clinical symptoms are nonspecific and may include cough, dyspnea, chest pain, and fever. The appropriate clinical presentation along with elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and storiform fibrosis is consistent with the disease. Steroids are used to treat this disease in addition to immunosupressives such as cyclosporine or rituxumab for steroid refractory disease. The pulmonary manifestations and imaging features can often mimic malignancy, and as such knowledge of the diagnostic, clinicopathologic, and radiographic features of the disease is required in order to provide appropriate diagnostic workup and treatment.
PURPOSE. The adult lacrimal gland (LG) is highly regenerative and is able to repair itself even after substantial damage; however, this ability to regenerate is lost with the development of dry eye conditions in chronically inflamed LGs.This study compares changes in the cell adhesion and cell matrix molecules and stem cell transcription factors in the LGs of healthy mice and of two mouse models of Sjögren's syndrome: nonobese diabetic (NOD) and MRLlpr/lpr (MRL/lpr) mice during the early stage of inflammation.
METHODS. TheLGs from 12-to 13-week-old female MRL/lpr and male NOD mice along with their respective control strains were harvested and divided into three pieces and processed for quantitative (q) RT-PCR and qRT-PCR Arrays, histology, immunohistochemistry, and Western blotting.
RESULTS. The extracellular matrix (ECM) and adhesion molecules RT2 -PCR array combined with protein expression data revealed changes in the expression of integrins, matrix metalloproteinases, and other molecules, which are associated largely with invasion, attachment, and expansion of the lymphocytic cells, whereas changes in the stem cell transcription factors revealed substantial decrease in expression of transcription factors associated with epithelial stem/progenitor cell lineage.
CONCLUSIONS.We concluded that the expression of several important ECM components is significantly deregulated in the LG of two murine models of Sjögren's syndrome, suggesting an alteration of the epithelial stem/progenitor cell niche. This may result in profound effects on localization, activation, proliferation, and differentiation of the LG stem/progenitor cells and, therefore, LG regeneration.
Broncholithiasis is the presence of calcific material within the tracheobronchial tree. Asymptomatic patients can be managed with observation only, whereas symptomatic disease requires surgery, rigid or flexible bronchoscopic removal. Recent reports have shown that flexible bronchoscopy can be a safe and effective option for removal of loose in addition to partially imbedded broncholiths. We present a case of a 65-yearold man with chronic cough that underwent successful cryotherapy assisted bronchoscopic removal of an imbedded broncholith. We will also review current literature regarding the management broncholithiasis.
Melanoma is the deadliest form of skin cancer with an estimated incidence of over 160,000 cases annually and about 41,000 melanoma-related deaths per year worldwide. Malignant melanoma (MM) primarily occurs in the skin but has been described in other organs. Although the respiratory system is generally afflicted by tumors such as lung cancer, it is also rarely affected by primary MM. The estimated incidence of pulmonary MM of the lung accounts for 0.01% of all primary lung tumors. The current understanding of pulmonary MM of the lung pathophysiology and its management are not well established. We aim to survey current clinical modalities with a focus on diagnostic imaging and therapeutic intervention to guide providers in the management of patients with a high index of suspicion.
Overlap syndrome subjects have higher PPN than those with COPD alone, regardless of their CPAP compliance. Our results could be used to motivate OS subjects to improve their lifestyles and to comply with drug therapies aimed at reducing cardiovascular disease (CVD).
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