cryptogenic fibrosing alveolitis


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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.

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.

cryptogenic fibrosing alveolitis

A progressive lung disease involving thickening and fibrosis of the walls of the ALVEOLI and large mononuclear cells in the alveolar spaces. As the name implies, the cause is unknown but the condition is believed to be the end stage of a disorder brought about by one of a range of possible factors including exposure to various occupational dusts or volatile solvents, virus infections or genetic influences.
References in periodicals archive ?
In the interview with the Newcastle Chronicle, Caroline said: "Our mother was suffering from a very, very rare lung disease, Cryptogenic Fibrosing Alveolitis.
4) Active eosinophilic airway inflammation, increased skin sensitivity to common allergens, increased serum IgE and eosinophilia in peripheral blood have been demonstrated in idiopathic UIP and atopy has been suggested to be an important determinant of susceptibility to cryptogenic fibrosing alveolitis.
They noted these findings in cryptogenic fibrosing alveolitis, sarcoidosis, farmer's lung disease, histiocytosis, and also bronchiolitis obliterans organizing pneumonia (BOOP).
35) The condition has also been described in association with cryptogenic fibrosing alveolitis.