ADVANCES IN THE DIAGNOSIS AND TREATMENT OF IDIOPATHIC PULMONARY FIBROSIS - A LITERATURE REVIEW
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease of unknown cause, characterized by irreversible fibrosis of the lung parenchyma, which leads to progressive deterioration of respiratory function, hypoxemia, and eventually respiratory failure and death. The average survival from the time of diagnosis is about 3-5 years, making IPF one of the most fatal chronic respiratory diseases. The disease most often affects men over the age of 60, often smokers or former smokers, and its symptoms - such as chronic dry cough, exertional dyspnea and crackles over the bases of the lungs - are nonspecific, making early diagnosis significantly more difficult. Although advances have been made in the diagnosis and treatment of IPF in recent years, there is still a lack of effective methods to completely stop or reverse the fibrosis process. Currently available therapies - pirfenidone and nintedanib - have been shown to slow the progression of the disease, but do not reverse it. Lung transplantation remains the only causal treatment option, although it is reserved for a limited group of patients. Recent years have seen an intensification of research into new drugs targeting specific molecular mechanisms involved in the pathogenesis of IPF, such as TGF-β, PDGF, VEGF, FGF or inflammatory and epigenetic pathways. In parallel, techniques are being developed to more accurately differentiate disease phenotypes and personalize therapy. The purpose of this paper is to review the current state of knowledge on IPF, with particular emphasis on recent advances in pathogenesis, diagnosis, pharmacological and non-pharmacological treatment, as well as prospects for future research aimed at improving patients' quality of life and prognosis.
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