n. a drug with antifibrotic and anti-inflammatory properties that is used to treat *idiopathic pulmonary fibrosis. It is thought to reduce fibroblast proliferation, inhibit *transforming growth factor-?-stimulated collagen production, and reduce the production of fibrogenic mediators. Pirfenidone has also been shown to reduce production of inflammatory mediators, such as *tumour necrosis factor and interleukin 1 (IL-1). Side effects include gastrointestinal upsets.
(IPF) a serious interstitial lung disease, formerly called cryptogenic fibrosing alveolitis (see alveolitis). It is characterized by progressive fibrous scarring of the lung and increased numbers of inflammatory cells in the alveoli and surrounding tissues. The disease is usually diagnosed on clinical grounds on a basis of worsening breathlessness, inspiratory crackles at the lung bases on auscultation, clubbing of the fingers or toes, bilateral radiographic shadowing predominantly in the lower zones of the chest X-ray, subpleural *honeycomb change on CT scanning of the chest, and restrictive lung function on spirometry. It is also called usual interstitial pneumonia (UIP; see interstitial pneumonia), a term used by lung pathologists for the most common cellular pattern seen on biopsy. Treatment includes *pirfenidone, corticosteroids, and immunosuppressants.... idiopathic pulmonary fibrosis
Health Encyclopedia