Modulation of chemokine CXCL12 and its receptor CXCR4 has been implicated in attenuation of bleomycin (BLM)-induced pulmonary fibrosis and carbon tetrachloride (CCl4)-induced hepatic injury. In pulmonary fibrosis, published reports suggest that collagen production in the injured lung is derived from fibrocytes recruited from the circulation in response to release of pulmonary CXCL12. Conversely...

BACKGROUND Despite intense research efforts, the aetiology and pathogenesis of idiopathic pulmonary fibrosis remain poorly understood. Gelsolin, an actin-binding protein that modulates cytoskeletal dynamics, was recently highlighted as a likely disease modifier through comparative expression profiling and target prioritisation. METHODS To decipher the possible role of gelsolin in pulmonary in...

Objectives: Idiopathic pulmonary fibrosis (IPF) (J84.1 by the International Statistical Classification of Diseases and Related Health Problems, 10th revision – ICD-10) is a rare disease of unknown cause. Among many risk factors, occupational exposure to metal dust is enumerated. Because of metal industry concentration in the Silesian Voivodeship, Poland, an attempt of spatial and temporal varia...

In 1980, the question “Is there a fibrotic gene?” was posed in an editorial [1] commenting on a report about pulmonary fibrosis in monozygotic twins. Over the next 37 years, the identification of kindreds of families with pulmonary fibrosis and advances in DNA sequencing provided answers to this question. An early example of a gene associated with pulmonary fibrosis involved a case of familial ...

Lung cancer is a frequent complication in pulmonary fibrosis. Overexpression of p53 proteins has been demonstrated by immunostaining in bronchoepithelial cells in patients with idiopathic pulmonary fibrosis. However, it is still unclear whether this overexpressed p53 protein is wild-type or mutant. It was hypothesized that pulmonary fibrosis may be a precancerous lesion with deoxyribonucleic ac...

BACKGROUND Recent evidence has demonstrated the role of angiogenesis in the pathogenesis of pulmonary fibrosis. Endostatin, a proteolytic fragment of collagen XVIII, is a potent inhibitor of angiogenesis. The aim of our study was to assess whether endostatin has beneficial effects on bleomycin (BLM)-induced pulmonary fibrosis in rats. METHODS The rats were randomly divided into five experimen...

Several reports indicate that melatonin alleviates bleomycin (BLM)-induced pulmonary fibrosis in rodent animals. Nevertheless, the exact mechanism remains obscure. The present study investigated the effects of melatonin on endoplasmic reticulum (ER) stress and epithelial-mesenchymal transition (EMT) during BLM-induced lung fibrosis. For the induction of pulmonary fibrosis, mice were intratrache...

OBJECTIVE Although lung transplantation is viewed as an acceptable option for patients with end-stage idiopathic pulmonary fibrosis, the survival benefit of this approach is still debated. This study examined whether there was a survival benefit of lung transplantation in a cohort of patients referred to our transplant center with a diagnosis of idiopathic pulmonary fibrosis according to Americ...

It was hypothesized that neutrophil elastase released from activated neutrophils may play an important role in the pathogenesis of pulmonary fibrosis. In the present study, we measured the neutrophil elastase:alpha-1-proteinase inhibitor complex (E-PI) in serum and bronchoalveolar lavage fluid (BALF) in 26 patients with pulmonary fibrosis and evaluated the correlation between E-PI levels and se...

OBJECTIVES Idiopathic pulmonary fibrosis (IPF) (J84.1 by the International Statistical Classification of Diseases and Related Health Problems, 10th revision - ICD-10) is a rare disease of unknown cause. Among many risk factors, occupational exposure to metal dust is enumerated. Because of metal industry concentration in the Silesian Voivodeship, Poland, an attempt of spatial and temporal variab...