Idiopathic Pulmonary Fibrosis Treatment Update

Idiopathic pulmonary fibrosis (or cryptogenic fibrosing alveolitis (CFA) is a chronic, progressive form of lung disease characterized by fibrosis of the interstitium of the lungs.Despite extensive inveastigation, the cause of IPF remains unknown. The condition involves abnormal and excessive deposition of collagen (fibrosis) in the pulmonary interstitium  with minimal associated inflammation. 

IPF affects both genders and is usually encountered in patients greater than 50 years of age. However there is a notable reported case of a young adult has been diagnosed with IPF.There are many different statements about average survival time following first diagnosis. Symptoms are gradual in onset. The most common are progressive dyspnea,but also include dry cough, clubbing and rales.

Plain chest x-rays reveal decreased lung volumes, typically with prominent reticular interstitial markings near the lung bases. Honeycombing, a pattern of lung fibrosis characterized by multiple cystic spaces located at the bases of the lungs, is frequently seen in advanced cases. In less severe cases, these changes may not be evident on a plain chest film.

High-resolution CT scans of the chest demonstrate fibrotic changes in both lungs, with a predilection for the bases and the periphery. The most charactersitic radiologic feature of IPF is honeycombing, often described as traction bronchiectasis. There may be ground glass opacities of the lungs but these changes are relatively minor in comparison with the fibrotic changes.

Spirometry classically reveals a reduction in the vital capacity with either a proportionate reduction in airflows, or increased airflows for the observed vital capacity. The latter finding reflects the increased lung stiffness associated with pulmonary fibrosis, which leads to increased lung elastic recoil..

by

Akshaya Srikanth

Pharm.D Internee

Hyderabad, India