Idiopathic pulmonary fibrosis beyond the lung: understanding disease mechanisms to improve diagnosis and management — luppi 2021

The projected median survival period for idiopathic pulmonary fibrosis (IPF), a chronic and progressive disease, is 3-5 years from diagnosis. The main risk factors for this illness, aging and exposure to cigarette smoke, are thought to be linked to both pulmonary and extrapulmonary comorbidities, according to epidemiological research (defined as the occurrence of two or more disorders in a single individual). Senescence and aging may interact with environmental factors to contribute to the pathogenesis of IPF through a number of processes, damaging the lung epithelium and making myofibroblasts more resistant to apoptosis, which in turn leads to the buildup of extracellular matrix and pulmonary fibrosis. Short telomere syndromes serve as a paradigm for premature aging syndromes and are frequently linked to pulmonary fibrosis. Patients with IPF may experience pulmonary and extrapulmonary features such as emphysema, pulmonary hypertension, lung cancer, coronary artery disease, gastro-oesophageal reflux, diabetes mellitus, and many other chronic diseases as a result of the pathophysiological features brought on by aging and senescence. These features may have a significant negative impact on a variety of outcome parameters. Therefore, it is important to think about all possible therapy options for IPF, including early identification and treatment of comorbidities. Careful diagnosis and treatment of comorbidities may represent an amazing possibility to improve quality of life and survival.

Luppi F, Kalluri M, Faverio P, Kreuter M, Ferrara G. Idiopathic pulmonary fibrosis beyond the lung: understanding disease mechanisms to improve diagnosis and management. Respir Res. 2021 Apr 17;22(1):109. doi: 10.1186/s12931-021-01711-1. PMID: 33865386; PMCID: PMC8052779.