Chest Radiology > Pathology > Interstitial Disease

Interstitial Pulmonary Fibrosis

Interstitial pulmonary fibrosis has many causes.  The six most common causes of diffuse interstitial pulmonary fibrosis are idiopathic (IPF, >50% of cases), collagen vascular disease, cytotoxic agents and nitrofurantoin, pneumoconioses, radiation, and sarcoidosis.  Clinically the patient with IPF will present with progressive exertional dyspnea and a nonproductive cough.  Radiographically, IPF is associated with hazy "ground glass" opacification early and volume loss with linear opacities bilaterally, and honeycomb lung in the late stages.  IPF carries a poor prognosis with death due to pulmonary failure usually occurring within 3-6 years of the diagnosis unless lung transplant is performed. 

 

Interstitial Pulmonary Fibrosis