Idiopathic pulmonary fibrosis.

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is being more frequently diagnosed in India, due to its increased awareness, better availability of computed tomography (CT) and fiberoptic bronchoscopy. IPF has the histological appearance of usual interstitial pneumonia (UIP) on surgical lung biopsy. Recent research has given a new insight into the etiology of the disease. Clinical criteria have been specified for presumptive diagnosis of IPF and distinguishing IPF from other conditions. The conventional therapy has been steroids and immunosuppressive agents. But only a minority of patients respond to such a therapy. Immunomodulators (interferon Y1b), antioxidants (Acetyl cysteine) and antifibrotic agents (like pirfenidone) are being studied as novel therapies in this, otherwise, fatal condition. Lung transplantation is the only hope for those patients who show progressive deterioration on medical treatment. Living-donor lobar lung transplantation has been developed as a procedure for patients considered too ill to await cadaveric lung transplantation.