id·i·o·path·ic pul·mo·nar·y fi·bro·sis (IPF),[MIM*178500]
an acute to chronic inflammatory process or interstitial fibrosis of the lung of unknown etiology. seen in association with collagen-vascular diseases.
Etymology: L, fibra + alviolus, small hollow; Gk, itis, inflammation
a severe form of alveolitis characterized by dyspnea and hypoxia. It occurs in advanced rheumatoid arthritis and other autoimmune diseases. X-ray films show thickening of the alveolar septa and diffuse pulmonary infiltrates. See also alveolitis.
fibrosing alveolitisPulmonology A progressive chronic inflammatory disease of adults–45 to 65, which later develops into diffuse interstitial fibrosis and honeycomb lung Prevalence 4/10,000; accounts for ±15% of honeycomb lung disease Clinical Dry cough, dyspnea, clubbed fingers; FA progresses to respiratory failure and may produce cor pulmonale PFTs Restrictive changes Complications ↑ lung CA, often peripheral adenoCA
fibrosing alveolitisA serious lung disorder involving extensive fine scarring of, and numerous cells within, the air sacs (alveoli) of the lungs. The condition can be caused by exposure to asbestos dust, mineral dusts, fungal spores and powdered bird droppings; rheumatic disorders such as RHEUMATOID ARTHRITIS, SJÖGREN'S SYNDROME and systemic sclerosis; some of the anticancer drugs such as bleomycin and cyclophosphamide; and the heart drug amiodarone. The condition may require lung transplantation.
Liebow,Averill A., Austrian-U.S. pulmonary pathologist, 1911-1978.
usual interstitial pneumonia of Liebow - a progressive inflammatory condition of the lung. Synonym(s): fibrosing alveolitis; Hamman-Rich syndrome; idiopathic interstitial fibrosis