of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2003; 168: 543–548. 8 Raghu G, Brown KK, Costabel U, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med 2008; 178: 948–955. 9 Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis...

Pulmonary fibrosis is a diverse group of lung disorders defined by varying degrees and inflammation in the pulmonary parenchyma. While it may be caused known disease, e.g., autoimmune or connective tissue disorder, drugs, hypersensitivity to inhaled organic antigens, sarcoidosis, also occurs idiopathic. When we examine pathogenesis fibrosis, see that cellular aging plays major role. Lung fibrob...

Most of the cells found in lung parenchyma in patients with idiopathic pulmonary fibrosis are activated T lymphocytes and macrophages. The serum levels of three markers of cell mediated immunity were measured in 20 patients with idiopathic pulmonary fibrosis, in 20 normal subjects and in 12 patients with sarcoidosis to evaluate their clinical and prognostic significance in idiopathic pulmonary ...

This article focuses on recent advances in the identification of genes and genetic polymorphisms that have been implicated in the development of human interstitial lung diseases. It focuses on the inherited mendelian diseases in which pulmonary fibrosis is part of the clinical phenotype and the genetics of familial idiopathic pulmonary fibrosis and other rare inherited interstitial lung disease...

OBJECTIVE Respiratory pressure-volume curves fitted to exponential equations have been used to assess disease severity and prognosis in spontaneously breathing patients with idiopathic pulmonary fibrosis. Sigmoidal equations have been used to fit pressure-volume curves for mechanically ventilated patients but not for idiopathic pulmonary fibrosis patients. We compared a sigmoidal model and an e...

Khalilzadeh S1, Baghaei N2, Bolorsaz MR1, Masjedi MR3 1. Associate professor, Department of pediatrics, Education, research and treatment center of tuberculosis and long diseases, Shahid Beheshti University of medical sciences 2. Assistant professor, Department of pediatrics, Education, research and treatment center of tuberculosis and long diseases, Shahid Beheshti University of medical s...

A 25 year old male and his 46 year old aunt presented with shortness of breath and a dramatic response to steroids. The other two patients are sisters with more advanced disease. One of these responded partially to steroids, while the other died within 4 months of treatment. The genetic basis and pathogenesis are discussed.

BACKGROUND Idiopathic pulmonary fibrosis, the most common form of idiopathic interstitial pneumonia, is characterized by progressive, irreversible scarring of the lung parenchyma. Idiopathic pulmonary fibrosis has a poor prognosis, and there are no medical therapies available that have been shown to improve survival. It is usually sporadic, but there is evidence of familial clustering of pulmon...

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Proximity extension assay proves feasible for multiplex analysis of proteins in bronchoalveolar lavage. Small exploratory study found multiple inflammatory that differed between patients with sarcoidosis and idiopathic pulmonary fibrosis.https://bit.ly/3nW14nF