Idiopathic pulmonary fibrosis

Rare card · No. 31037

Idiopathic pulmonary fibrosis (IPF), previously called cryptogenic fibrosing alveolitis, is a rare, progressive illness of the respiratory system. It is characterized by the thickening and stiffening of the lung tissue that surrounds the air sacs, and is associated with the formation of scar tissue. It is a type of chronic pulmonary fibrosis characterized by a progressive and irreversible decline in lung function. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other symptoms may include feeling tired, and digit clubbing (abnormally enlarged, dome-shaped fingers and toenails). Complications may include pulmonary hypertension, heart failure, pneumonia or pulmonary embolism. The cause is unknown, hence the term idiopathic. Risk factors include cigarette smoking, gastroesophageal reflux disease, certain viral infections, and a genetic predisposition. The underlying mechanism involves scarring of the lungs. As it is such a severe disease, a full differential...

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