Idiopathic pulmonary fibrosis (IPF) is characterized by progressive lung scarring, short median survival, and limited therapeutic options, creating great need for new pharmacologic therapies. IPF is thought to result from repetitive environmental injury to the lung epithelium, in the context of aberrant host wound healing responses. Tissue responses to injury fundamentally involve reorganizatio...

Transient adenovirus-mediated gene transfer of active TGF-β1 (transforming growth factor-β1) induces severe and progressive fibrosis in rodent lung without apparent inflammation. Alternatively, transfer of IL-1β (interleukin 1β) induces marked tissue injury and inflammation, which develops into progressive fibrosis, associated with an increase in TGF-β1 concentrations in lung fluid and tissue. ...

BACKGROUND Effective treatments for fibrotic diseases such as idiopathic pulmonary fibrosis are largely lacking. Transforming growth factor beta (TGFβ) plays a central role in the pathophysiology of fibrosis. We hypothesized that bone morphogenetic proteins (BMP), another family within the TGFβ superfamily of growth factors, modulate fibrogenesis driven by TGFβ. We therefore studied the role of...

BACKGROUND Acute lung injury (ALI) is a life-threatening hypoxemic respiratory disorder with high incidence and mortality. ALI usually manifests as widespread inflammation and lung fibrosis with the accumulation of pro-inflammatory and pro-fibrotic factors and collagen. Thymic stromal lymphopoietin (TSLP) has a significant role in regulation of inflammation but little is known about its roles i...

TOPIC: Lung Pathology TYPE: Original Investigations PURPOSE: It has been reported that AEP histopathologically exhibits diffuse alveolar damage (DAD) with eosinophilia. However, acute respiratory distress syndrome (ARDS), interstitial pneumonia (AIP), and exacerbation of idiopathic pulmonary fibrosis, also exhibiting DAD histopathologically, are all life-threatening diseases. AFP is rapidly imp...

BACKGROUND The excess and persistent accumulation of fibroblasts due to aberrant tissue repair results in fibrotic diseases such as idiopathic pulmonary fibrosis. Recent reports have revealed significant changes in microRNAs during idiopathic pulmonary fibrosis and evidence in support of a role for microRNAs in myofibroblast differentiation and the epithelial-mesenchymal transition in the conte...

Aging constitutes a significant risk factor for fibrosis, and idiopathic pulmonary fibrosis (IPF) is characteristically associated with advancing age. We propose that age-dependent defects in the quality of protein and cellular organelle catabolism may be causally related to pulmonary fibrosis. Our research found that autophagy diminished with corresponding elevated levels of oxidized proteins ...

BACKGROUND Alveolar Type II cell transplantation has been proposed as a cell therapy for the treatment of idiopathic pulmonary fibrosis. Its long-term benefits include repair of lung fibrosis, but its success partly depends on the restoration of lung homeostasis. Our aim was to evaluate surfactant protein restoration after alveolar Type II cell transplantation in an experimental model of bleomy...

To assess further the clinical significance of asbestos-induced pleural fibrosis, we used a computer algorithm to reconstruct images three dimensionally from the high-resolution computerized tomography (HRCT) scan of the chest in 60 asbestos-exposed subjects. Pulmonary function tests, chest radiographs, and HRCT scans were performed on all study subjects. The volume of asbestos-induced pleural ...

Cogswell, J. J., Risdon, R. A., and Taylor, B. (1974). Archives of Disease in Childhood, 49, 520. Chronic suppurative lung disease in sisters mimicking cystic fibrosis. Two sisters are described with fatal chronic obstructive lung disease of unknown origin. The lung condition was clinically and histologically indistinguishable from cystic fibrosis and the salivary glands showed pathological cha...