Idiopathic pulmonary fibrosis: pathogenesis and management — Sgalla 2018
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease that affects the lungs' parenchyma and is marked by an abnormal buildup of fibrotic tissue. It has a high mortality rate and a dismal prognosis. This review will outline the significant advancements made in our knowledge of the pathophysiology of IPF, the therapeutic choices available to patients, and the unresolved problems with diagnosis and treatment.
Much has been learned over the past 20 years regarding the pathogenic mechanisms driving the onset and progression of lung scarring in IPF. Multiple molecular events of disordered repair occurring in genetically predisposed older persons have been linked to sustained alveolar epithelial micro-injury and activation. Patients can still remain unclassified when the current diagnostic criteria are strictly applied, necessitating the identification of a Usual Interstitial Pattern either on a high-resolution computed tomography scan or lung biopsy, despite the fact that multidisciplinary team discussion has shown to increase diagnostic accuracy.
Outstanding progress has been made in the treatment of these patients, as nintedanib and pirfenidone have repeatedly demonstrated their ability to slow the rate at which the fibrotic process advances. However, there are still a lot of unknowns when it comes to using these medications correctly, including the initial drug choice, the best time to start treatment, and the benefit-risk balance of a combination treatment plan. In the meantime, the supportive care of these patients and their caregivers should be appropriately prioritized, and more efforts should be made toward the prompt identification and management of relevant comorbidities. Several novel compounds are being developed in the perspective of a more targeted therapeutic approach.
The investigation of the role of gene variants, epigenetic changes, and other molecular biomarkers reflecting disease activity and behavior will hopefully enable earlier and more confident diagnosis, improve disease phenotyping, and support the development of novel agents for personalized treatment of IPF, building on the advances in the understanding of IPF pathobiology.
Sgalla G, Iovene B, Calvello M, Ori M, Varone F, Richeldi L. Idiopathic pulmonary fibrosis: pathogenesis and management. Respir Res. 2018 Feb 22;19(1):32. doi: 10.1186/s12931-018-0730-2. PMID: 29471816; PMCID: PMC5824456.
