Update in diagnosis and treatment of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a rare disease characterized by chronic, fibrosing, progressive, and irreversible lung involvement of unknown etiology and poor prognosis. It is considered a complex disease resulting from the interaction between genetic susceptibility, cellular and molecular aging, and repeated environmental exposures. This review is part of a position paper on the diagnosis and treatment of IPF, developed by the clinical-radiological-pathological study group on diffuse interstitial lung diseases of the Catalan Society of Pulmonology. Based on published clinical evidence, it highlights key aspects of the diagnostic process: a systematic approach that includes invasive procedures (such as lung biopsy) in selected cases, the central role of multidisciplinary discussion, and the importance of genetic testing. Moreover, holistic therapeutic management of IPF is essential, combining antifibrotic therapy with supportive and non-pharmacological interventions such as supplementary oxygen, pulmonary rehabilitation, symptom control strategies, patient education, and palliative care.