Alveolar macrophages (AMs) from patients with interstitial lung diseases, such as sarcoidosis and idiopathic pulmonary fibrosis, suppress the phytohaemagglutinin (PHA) stimulation of autologous peripheral lymphocytes. The aim of this study was to determine whether the suppressive effect of alveolar macrophages of patients with interstitial lung disease is due, not only to the secretion of solub...

Pulmonary ossification is an idiopathic disorder that presents with the formation of mature bone in the pulmonary parenchyma. This is a very rare entity that occurs in conjunction with busulfan therapy as well as with a number of diseases including chronic bronchitis, cystic fibrosis, congestive heart failure, myositis ossificans, and idiopathic interstitial fibrosis. It is usually seen in olde...

Traditionally, a subset of patients diagnosed as having idiopathic pulmonary fibrosis had positive results on cellular biopsies (prominent lymphoplasmacytic inflammation), bronchoalveolar lavage lymphocytosis, a clinical response to steroids, and a better long-term prognosis. On review of the lung histopathology, the lesion was characterized by varying degrees of inflammation and fibrosis. This...

objective(s): pulmonary fibrosis (pf) is the most common outcome of a collection of diverse lung disorders known as interstitial lung diseases. it is proposed that alterations in the levels of fibrogenic mediators and the profibrotic/antifibrotic imbalance play a substantial role in the progression of pf in animal models and possibly in humans. caffeic acid phenethyl ester (cape), an active com...

Background Pathologic Classification of Idiopathic Pulmonary Fibrosis UIP DIP/Respiratory Bronchiolitis Interstitial Lung Disease (RBILD) AIP NSIP Clinical Features of the Idiopathic Interstitial Pneumonias UIP DIP/RBILD AIP N SIP Grading of “Cellularity” and Fibrosis Relationship of UIP and DIP Relationship of AIP and UIP Significance of NSIP—A Specific Disease or a Histologic Manifestation of...

BACKGROUND Cyclooxygenase-2, a key regulatory enzyme in the synthesis of the antifibrotic agent prostaglandin E2, is downregulated in lung tissue from patients with idiopathic pulmonary fibrosis. OBJECTIVE To investigate the association between COX2.3050 (G --> C), COX2.8473 (C --> T) and COX2.926 (G --> C) single nucleotide polymorphisms (SNP) and the susceptibility to idiopathic pulmonary f...

Sirt6 which is implicated in the control of aging, cancer, and metabolism, has been shown to have anti-fibrosis function in heart and liver. However, whether Sirt6 inhibits idiopathic pulmonary fibrosis remains elusive. Epithelial to mesenchymal transition has been found to be involved in the pathogenesis of idiopathic pulmonary fibrosis. In the present study, forced expression of Sirt6 signifi...

The development of pulmonary fibrosis is the end point of a wide range of respiratory diseases including organic and inorganic dust exposure, pulmonary infection, acute lung injury, radiation, the idiopathic interstitial pneumonias (IIP), and connective tissue diseases. The most common fibrotic lung disorder is idiopathic pulmonary fibrosis (IPF), an IIP with the histological appearance of usua...

Emphysema is characterized by the permanent abnormal enlargement of airspaces distal to the terminal bronchioles, accompanied by destruction of their walls. The characteristics of emphysema do not, by definition, include thickening of the alveolar septa and fibrosis. However, coincidental idiopathic pulmonary fibrosis (IPF) and emphysema was firstly reported in 1990 by Wiggins et al (Wiggins J,...

BACKGROUND Pirfenidone was recently approved for treatment of idiopathic pulmonary fibrosis. However, the therapeutic dose of pirfenidone is very high, causing side effects that limit its doses and therapeutic effectiveness. Understanding the molecular mechanisms of action of pirfenidone could improve its safety and efficacy. Because activated fibroblasts are critical effector cells associated ...