Diseases, Conditions, Syndromes

Unlocking the secrets of lung fibrosis with a new mouse model

A RIKEN-developed mouse model of an enigmatic lung disease promises to unlock new biological insights and catalyze the development of treatments for millions affected globally. The research is published in Nature Communications.

Idiopathic pulmonary fibrosis (IPF) (or cryptogenic fibrosing alveolitis (CFA) / or Idiopathic Fibrosing Interstitial Pneumonia) is a chronic, progressive form of lung disease characterized by fibrosis of the supporting framework (interstitium) of the lungs. By definition, the term is used only when the cause of the pulmonary fibrosis is unknown ("idiopathic").

Microscopically, lung tissue from patients shows a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). UIP is therefore the pathologic counterpart of IPF.

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