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The article focuses on the rate of usual interstitial pneumonia (UIP) pattern in interstitial pneumonia related to undifferentiated connective tissue disease (UCTD). Topics discussed include the methodology and data used by the authors in their study, response to treatment of UCTD-IP and a comparison of the overall survival rate between UCTD-UIP and UCTD-nonspecific interstitial pneumonia (UCTD-NSIP).

Background: Because of the variable course of idiopathic pulmonary fibrosis (IPF), it is important to generate an accurate prognosis at the time of diagnosis. The aim of this study was to investigate the prognostic value of blood biomarkers in IPF.Methods: The plasma level of the biomarkers, matrix metalloproteinase-7 (MMP-7), Krebs von den Lungen-6 antigen, and surfactant protein (SP)-A and SP-D were retrospectively compared with the clinical course of 118 patients with IPF, 68 of whom had biopsy-proven IPF.Results: The median follow-up period was 24 months. Multivariate Cox analysis showed MMP-7 (HR, 1.056; P = .0063) and SP-A (HR, 1.011; P = .0001) were significant predictors of survival along with age, FVC, and extent of honeycombing. The patients with high levels of both MMP-7 (≥ 12.1 ng/mL) and SP-A (≥ 80.3 ng/mL) had shorter survival (1-year survival rate: 59%) and higher frequency (42%) of lung function decline (> 10% reduction in FVC in 6 months) compared with those with high levels of one biomarker (1-year survival rate: 81%; FVC decline: 27%) or low levels of both (1-year survival rate: 83.3%; FVC decline: 9%). Multivariate models demonstrated marginal improvement in the prediction of mortality (concordance index [C-index]: 0.731; P = .061) when MMP-7 and SP-A were included and compared with standard clinical predictors only (C-index: 0.686); however, it became significant with addition of MMP-7, SP-A, and Krebs von den Lungen-6 antigen (C-index: 0.730; P = .037).Conclusions: Our retrospective study suggested that at least three biomarkers are necessary to improve predictability of mortality in IPF compared with clinical parameters. Further study in a greater number of patients is warranted. [ABSTRACT FROM AUTHOR]

The article reports on acute exacerbation (AE) of interstitial pneumonia other than idiopathic pulmonary fibrosis (IPF) . It is emphasized that AE in IPF is increasingly recognized as a relatively common and morbid clinical event. The study established that AE occurred in patients with interstitial pneumonia (I-NSIP) with apparently better prognosis. Among patients with collagen vascular disease (CVD-IP), AE occurred with rheumatoid arthritis (RA)-usual interstitial pneumonia.

Investigates the histopathologic pattern and clinical features of patients with rheumatoid arthritis-associated interstitial lung disease. Surgical lung biopsy of patients; Results of pulmonary function test; Comparison of prevalence between usual interstitial pneumonia and nonspecific interstitial pneumonia patterns.

Study Objectives: Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown etiology, affecting almost exclusively women of childbearing age, that is associated with the proliferation of spindle cells and cystic changes in the affected lung. The underlying processes that contribute to this disease are poorly understood. Transforming growth factor (TGF)-beta(1) is a potent cytokine that promotes mesenchymal cell proliferation and regulates the synthesis of extracellular matrix (ECM) components, particularly fibronectins. Herein, we evaluate the expression of TGF beta(1) and matrix-associated fibronectin in lung specimens demonstrating LAM.Design: Lung biopsy specimens that were confirmed to contain pathologic LAM cells were obtained from 13 patients. The specimens were submitted to immunohistochemical evaluation for TGF beta(1) and fibronectin, as well as the typical markers of LAM cells. Healthy lung parenchyma surrounding resected neoplasms was studied in a parallel fashion as control tissues.Measurements and Results: In all 13 LAM cases and in healthy lung parenchyma, we demonstrated that TGF beta(1) localized consistently to airway epithelial cells. However, in LAM tissues, matrix-associated TGF beta(1) was also consistently found in regions containing pathologic LAM cells. Notably, more abundant TGF beta(1) was observed in highly cellular areas compared to the walls of chronic cystic regions in LAM tissues. Fibronectin, a matrix component that is strongly expressed in response to active TGF beta(1) was found to consistently colocalize with this protein in these highly cellular regions, supporting TGF beta(1) activity in these regions. The markers of proliferating LAM cells, including proliferating cell nuclear antigen, were also markedly present in these highly cellular LAM regions.Conclusion: These studies suggest that the proliferation of aberrant LAM cells may be associated with altered regional expression of TGF beta(1) and related ECM proteins. [ABSTRACT FROM AUTHOR]

Study objectives: Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown etiology, affecting almost exclusively women of childbearing age, that is associated with the proliferation of spindle cells and cystic changes in the affected lung. The underlying processes that contribute to this disease are poorly understood. Transforming growth factor (TGF)-β1 is a potent cytokine that promotes mesenchymal cell proliferation and regulates the synthesis of extracellular matrix (ECM) components, particularly fibronectins. Herein, we evaluate the expression of TGFβ1 and matrix-associated fibronectin in lung specimens demonstrating LAM. Design: Lung biopsy specimens that were confirmed to contain pathologic LAM cells were obtained from 13 patients. The specimens were submitted to immunohistochemical evaluation for TGFβ1 and fibronectin, as well as the typical markers of LAM cells. Healthy lung parenchyma surrounding resected neoplasms was studied in a parallel fashion as control tissues. Measurements and results: In all 13 LAM cases and in healthy lung parenchyma, we demonstrated that TGFβ1 localized consistently to airway epithelial cells. However, in LAM tissues, matrix-associated TGFβ1 was also consistently found in regions containing pathologic LAM cells. Notably, more abundant TGFβ1 was observed in highly cellular areas compared to the walls of chronic cystic regions in LAM tissues. Fibronectin, a matrix component that is strongly expressed in response to active TGFβ1 was found to consistently colocalize with this protein in these highly cellular regions, supporting TGFβ1 activity in these regions. The markers of proliferating LAM cells, including proliferating cell nuclear antigen, were also markedly present in these highly cellular LAM regions. Conclusion.. These studies suggest that the proliferation of aberrant LAM cells may be associated with altered regional expression of TGFβ1 and related ECM proteins. [ABSTRACT FROM AUTHOR]