Neumonías intersticiales idiopáticas

Abstract

Idiopathic interstitial fibrosis has nonspecific, insidious clinical symptoms; it is the most common interstitial disease and has the worse known prognosis, with a survival without treatment of two to five years. In 2018, scientific societies updated the diagnostic criteria; the presence of a usual interstitial pneumonia pattern on an HRCT scan and/or a lung biopsy are decisive for establishing a diagnosis. Among idiopathic interstitial pneumonias, the most common is pulmonary fibrosis, but there are other less common entities such as nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated diffuse interstitial lung disease (RB-DILD), and desquamative interstitial pneumonia (DIP); the latter two are related to tobacco use. There are also those which present in acute or subacute forms, such as cryptogenic organizing pneumonia (COP) and acute interstitial pneumonia (AIP). Lastly, there are the least common forms, such as lymphoid interstitial pneumonia (LIP) and idiopathic pleuroparenchymal fibroelastosis (IPPFE).