Actualizaciones clínicas en enfermedades pulmonares intersticiales: estrategias diagnósticas y terapéuticas avanzadas

Interstitial lung diseases (ILDs) are a heterogeneous group of disorders that affect the lung interstitium, characterized by inflammation and/or fibrosis. Historically, they were based on histopathological characteristics of lung biopsies, but with the advancement of high-resolution computed tomography (HRCT), the approach has shifted, allowing for the identification of disease patterns that suggest different etiologies. The classification of ILDs has evolved, and they are currently classified according to etiology, histopathological and/or radiological patterns, and evolutionary course. Idiopathic interstitial pneumonias (IIPs) are a group of ILDs of unknown origin, with idiopathic pulmonary fibrosis (IPF) being the most common within the IIPs. The concept of progressive fibrosing interstitial lung disease (PF-ILD) has been developed, describing a phenotype of ILD with progression despite treatment. Diagnosis is based on a detailed clinical history, physical examination, pulmonary function tests, autoimmune serology, and imaging techniques such as HRCT. Treatment varies according to etiology and may include immunomodulatory or anti-inflammatory therapy, disease-modifying therapy with antifibrotic agents and, in advanced cases, lung transplantation. Medical care should be holistic, including pulmonary rehabilitation, infection prevention, and oxygen therapy. This article aims to provide a clinical update on interstitial lung diseases and comment on advances in diagnostic and therapeutic strategies.