Abstract
Idiopathic pulmonary fibrosis (IPF) is the most common and most lethal type of idiopathic interstitial pneumonia. It is a chronic, aging-associated lung disease characterized by fibrotic foci and inflammatory infiltrates, with no cure and very limited therapeutic options. Although its etiology is unknown, several pathogenic pathways have been described that could explain this process, involving aging, environmental factors, genomic instability, loss of proteostasis, telomere attrition, epigenetic changes, mitochondrial dysfunction, cell senescence, and altered intercellular communication. One of the main prognostic factors for the development of IPF in broad epidemiological studies is age. The incidence increases with age, making this a disease that predominantly affects the elderly population, being exceptional under 45 years of age. However, the degree to which each of these mechanisms is involved in the etiology of the uncontrolled fibrogenesis that defines IPF is still unknown. Clarifying these questions is crucial to the development of points of intervention in the pathogenesis of the disease. This review briefly summarizes what is known about each possible etiological factor, and the questions that most urgently need to be addressed.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease usually affecting the elderly, and its appearance is exceptional below the age of 45 years [1]
Epidemiological studies indicate that lung aging is associated with higher susceptibility to common chronic respiratory diseases such as chronic obstructive pulmonary disease (COPD) and COPD and IPF are distinct disease entities, they share some similarities. Both are punctuated by episodes of exacerbations, and histopathologic studies in animal models have shown increased collagen deposition and progressive pulmonary fibrosis in the lungs of aging rats [7], [8]
Aberrant DNA methylation can silence or activate gene expression patterns that drive the fibrosis process [31]. Another mechanism that favors the fibrotic process in IPF is changes in histones, for example, reduced histone H3 and H4 acetylation, which are associated with decreased cyclooxygenase-2 (COX-2) expression and prostaglandin E2 (PGE-2—a strong antifibrotic mediator) production by fibroblasts [32]
Summary
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease usually affecting the elderly, and its appearance is exceptional below the age of 45 years [1]. Its incidence and prevalence increase with age, and most cases are diagnosed in patients older than 60 years [2]. Aging is a complex multifactorial process characterized by a progressive loss of physiological integrity, accumulating deleterious changes of tissues, and cell damage, which are responsible for increased vulnerability and risk of diseases and death [4]. Aging is associated with increased susceptibility to a wide range of chronic diseases, including lung diseases such as IPF. We know that even in the absence of disease, both structural and functional age-related changes appear in the lungs. How and to what extent they participate in the development of IPF is uncertain
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