What Do We Need to Know About Rising Rates of Idiopathic Pulmonary Fibrosis? A Narrative Review and Update

Abstract

The most common type of idiopathic interstitial pneumonia is idiopathic pulmonary fibrosis (IPF), an irreversible, progressive disorder that has lately come into question for possible associations with COVID-19. With few geographical exceptions, IPF is a rare disease but its prevalence has been increasing markedly since before the pandemic. Environmental exposures are frequently implicated in IPF although genetic factors play a role as well. In IPF, healthy lung tissue is progressively replaced with an abnormal extracellular matrix that impedes normal alveolar function while, at the same time, natural repair mechanisms become dysregulated. While chronic viral infections are known risk factors for IPF, acute infections are not and the link to COVID-19 has not been established. Macrophagy may be a frontline defense against any number of inflammatory pulmonary diseases, and the inflammatory cascade that may occur in patients with COVID-19 may disrupt the activity of monocytes and macrophages in clearing up fibrosis and remodeling lung tissue. It is unclear if COVID-19 infection is a risk factor for IPF, but the two can occur in the same patient with complicating effects. In light of its increasing prevalence, further study of IPF and its diagnosis and treatment is warranted.

Single‐cell combined with transcriptome sequencing to explore the molecular mechanism of cell communication in idiopathic pulmonary fibrosis

“…Global statistics indicate that five million individuals suffering from IPF worldwide 23 . With exacerbating factors like environmental pollution and novel coronavirus infection, 24 the incidence rate is on the rise 25 . Despite considerable research efforts dedicated to IPF, treatment options remain severely limited, with no medications demonstrating significant efficacy in extending patient survival 26 .…”

Genome-wide SNP-sex interaction analysis of susceptibility to idiopathic pulmonary fibrosis

“…In the USA and Europe, IPF is estimated to have a disease prevalence of 0.63 to 7.6 per 100,000 people 2 . The number of people diagnosed with IPF is increasing and males are more likely to be diagnosed than women 3,4 . However, the reason why the disease is more prevalent in males is not understood.…”

Pirfenidone and Nintedanib in Pulmonary Fibrosis: Lights and Shadows

“…by a progressive distortion of airway architecture, leading to reduced lung volume and gradual loss of lung function, which can eventually result in respiratory failure [2][3][4][5][6][7][8][9].…”

“…Interstitial lung diseases (ILDs) comprise nearly a fifth of all lung diseases. ILDs are a heterogeneous group of disorders classified into distinct entities, each with specific clinical, radiological, and pathological characteristics [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 ]. Most ILDs are characterized by a progressive distortion of airway architecture, leading to reduced lung volume and gradual loss of lung function, which can eventually result in respiratory failure [ 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ].…”

“…The world’s highest prevalence occurs in South Korea with 4.51 cases per 10,000 persons, whereas in Europe, according to the most recent evidence in the literature, the estimated prevalence of IPF is estimated to be between 0.33 and 2.51 per 10,000 inhabitants [ 3 , 10 , 11 , 12 , 13 , 14 , 15 , 16 ]. The prevalence is estimated to be higher in men than in women, with a median age of 60 years old [ 4 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 ].…”