Primary Usual Interstitial Pneumonia: Idiopathic Pulmonary Fibrosis – Morphology of Lungs with Review of Literature

A BSTRACT Usual interstitial pneumonia (UIP) is the commonly observed pattern of interstitial pneumonia and is characterized by heterogenous fibrosis of lung parenchyma with architectural distortion, fibroblastic focci and honeycombing. It is highly specific histopathology diagnosis in the present state. Establishing UIP pattern is very important for overall prognosis of patient and management of the disease. Idiopathic Pulmonary Fibrosis (IPF) represents the primary form of UIP with unknown etiologies. Revised 2018 guidelines by ATS/ERS/JRS/ALAT for IPF suggested on detail histologic examination of lung biopsies to evaluate for features that may suggest an etiology for the fibrotic lung disease (other than IPF). IPF contributes to a higher mortality rate. Treatment with antibiotics, symptomatic management, pulmonary rehabilitation, and early referral to lung transplantation is the key in IPF. UIP pattern with underlying aetiology and other fibrosis pattern have different therapeutic approach and presumably favorable prognosis.