Abstract

Idiopathic pulmonary fibrosis (IPF) is the commonest form of idiopathic interstitial pneumonias with very poor prognosis. Currently, diagnostic and treatment approaches to this disease have been revised. Confirmation of the diagnosis requires careful exclusion of other known causes of interstitial lung diseases and the presence of usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRTC) and/or on lung biopsy. Also, multidisciplinary discussion involving experts with experience in the diagnosis of interstitial lung diseases is recommended. Given recent knowledge on pathogenesis of IPF antifibrotic drugs are recommended for the therapy of this disease. A clinical case that demonstrates the multidisciplinary approach to diagnosis of IPF is reported in this article.

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