idiopathic pulmonary fibrosis


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fibrosis

 [fi-bro´sis]
formation of fibrous tissue; see also fibroid degeneration. adj., adj fibrot´ic.
congenital hepatic fibrosis a developmental disorder of the liver, marked by formation of irregular broad bands of fibrous tissue containing multiple cysts formed by disordered terminal bile ducts, resulting in vascular constriction and portal hypertension.
cystic fibrosis (cystic fibrosis of pancreas) see cystic fibrosis.
diffuse idiopathic interstitial fibrosis (diffuse interstitial pulmonary fibrosis) idiopathic pulmonary fibrosis.
endomyocardial fibrosis an idiopathic type of myocardiopathy that is endemic in various parts of Africa and rarely in other areas, characterized by cardiomegaly, marked thickening of the endocardium with dense white fibrous tissue that may extend to involve the inner myocardium, and by congestive heart failure.
idiopathic pulmonary fibrosis chronic inflammatory progressive fibrosis of the pulmonary alveolar walls, with steadily progressive dyspnea, resulting in death from oxygen lack or right heart failure. Most cases are of unknown origin, although some are thought to result from pneumoconiosis, hypersensitivity pneumonitis, scleroderma, and other diseases.
mediastinal fibrosis development of hard white fibrous tissue in the upper portion of the mediastinum, sometimes obstructing the air passages and large blood vessels; called also fibrosing or fibrous mediastinitis.
periureteral fibrosis retroperitoneal fibrosis.
pleural fibrosis fibrosis of the visceral pleura so that part or all of a lung becomes covered with a plaque or a thick layer of nonexpansible fibrous tissue. The more extensive form is called fibrothorax.
postfibrinous fibrosis that occurring in tissues in which fibrin has been deposited.
proliferative fibrosis that in which the fibrous elements continue to proliferate after the original causative factor has ceased to operate.
pulmonary fibrosis idiopathic pulmonary fibrosis.
retroperitoneal fibrosis deposition of fibrous tissue in the retroperitoneal space, producing vague abdominal discomfort, and often causing blockage of the ureters, with resultant hydronephrosis and impaired renal function, which may result in renal failure. Called also Ormond disease.
fibrosis u´teri a morbid condition characterized by overgrowth of the smooth muscle and increase in the collagenous fibrous tissue of the uterus, producing a thickened, coarse, tough myometrium.

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.

idiopathic pulmonary fibrosis

Etymology: Gk, idios, own, pathos, disease; L, pulmoneous, the lungs, fibra, fiber
a disorder of unknown cause characterized by fibrosis of the lungs. It may follow an earlier inflammation or disease, such as tuberculosis or pneumoconiosis.

idiopathic pulmonary fibrosis

Idiopathic interstitial fibrosis of lung Pulmonology An idiopathic condition characterized by scarring and fibrosis of alveolar septae more common in middle-aged men, possibly related to collagen vascular disease, with positive 'rheumatoid' serology Clinical Aggressive–rapid onset of dyspnea, orthopnea, hemoptysis, cyanosis, clubbing of fingers and toes, pulmonary HTN, bibasilar rales, non-productive cough, death in 3-6 yrs Imaging Diffuse reticulonodular infiltrates. See Diffuse interstitial fibrosis, Lymphoid interstitial fibrosis, Usual interstitial fibrosis.

id·i·o·path·ic pul·mo·nar·y fi·bro·sis

(IPF) (id'ē-ō-path'ik pul'mŏ-nar-ē fī-brō'sis)
Subacute form also called Hamman-Rich syndrome (q.v.); an acute to chronic inflammatory process of the lungs, the healing stage of diffuse alveolar damage or acute interstitial pneumonia, either idiopathic or associated with collagen-vascular diseases.
Synonym(s): cryptogenic fibrosing alveolitis.
References in periodicals archive ?
This data driven report contains over 25 links to online copies of actual idiopathic pulmonary fibrosis deals and contract documents as submitted to the Securities Exchange Commission by companies and their partners, where available.
When the cause of the fibrosis is unknown (and certain pathologic or radiographic criteria are met), the disease is called idiopathic pulmonary fibrosis, or IPF.
Indeed, as previously reported (13), HHV-8 DNA was detected with significantly higher frequency in lung tissue samples of patients affected by idiopathic pulmonary fibrosis.
InterMune currently markets ACTIMMUNE(R) (Interferon gamma-1b) Injection in the United States for the treatment of chronic granulomatous disease (CGD) and severe, malignant osteopetrosis and is in Phase III clinical trials for the treatment of idiopathic pulmonary fibrosis (IPF) and multidrug-resistant tuberculosis (MDR TB).
The exact number of people who develop idiopathic pulmonary fibrosis each year is not known.
About I diopathic P ulmonary F ibrosis Idiopathic pulmonary fibrosis (IPF) is an irreversible and ultimately fatal disease characterized by progressive loss of lung function due to fibrosis (scarring) in the lungs, which hinders the ability of the lungs to absorb oxygen.
We read with pleasure Dr Leslie's thoughtful article describing a potential role for peripheral tractional lung injury in idiopathic pulmonary fibrosis (IPF).
We will distinguish this from the more narrowly defined disorder of idiopathic pulmonary fibrosis (IPF), the most common subset of the IIPs.
InterMune agreed in October to pay $37 million to settle charges it promoted Actimmune to treat idiopathic pulmonary fibrosis, a condition involving scarring of lung tissue.
Neither ACE inhibitors nor statins appear to improve survival in patients with idiopathic pulmonary fibrosis, reported Hassan F.
DUBLIN -- Dublin - Research and Markets has announced the addition of the "Global Idiopathic Pulmonary Fibrosis Drug Pipeline Capsule - 2012 Update" report to their offering.
today presented the results of pooled analyses of data from three multinational, randomised phase III trials (ASCEND[Study 016] and CAPACITY[Studies 004 and 006]) that provide evidence of a durable pirfenidone treatment benefit on multiple measures of disease status in patients with idiopathic pulmonary fibrosis (IPF) who continue through up to 72 weeks of treatment.

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