Idiopathic pulmonary fibrosis (IPF) represents a fibrotic interstitial lung disease characterized by uncertain etiology and poor prognosis. Over the years, the path to effective treatments has been marked by a series of advances and setbacks. The introduction of approved antifibrotic drugs, pirfenidone and nintedanib, marked a pivotal moment in the management of IPF. However, despite these advances, these drugs are not curative, although they can slow the natural progression of the disease. The history of drug therapy for IPF goes together with the increased understanding of the pathogenic mechanisms underlying the disease. Based on that, current research efforts continue to explore new therapies, possible personalized treatment strategies, drug combinations, and potential biomarkers for diagnosis and prognosis. In this review, we outline the route that led to the discover of the first effective therapies, ongoing clinical trials, and future directions in the search for more effective treatments.
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http://dx.doi.org/10.5826/mrm.2024.982 | DOI Listing |
Sci Rep
January 2025
Harbin Medical University, Harbin, Heilongjiang Province, China.
Interstitial lung disease (ILD) is known to be a major complication of systemic sclerosis (SSc) and a leading cause of death in SSc patients. As the most common type of ILD, the pathogenesis of idiopathic pulmonary fibrosis (IPF) has not been fully elucidated. In this study, weighted correlation network analysis (WGCNA), protein‒protein interaction, Kaplan-Meier curve, univariate Cox analysis and machine learning methods were used on datasets from the Gene Expression Omnibus database.
View Article and Find Full Text PDFEBioMedicine
January 2025
Department of Respiratory and Clinical Care Medicine, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200233, China. Electronic address:
Background: Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial pneumonia with restrictive ventilation. Recently, the structural and functional defects of small airways have received attention in the early pathogenesis of IPF. This study aimed to elucidate the characteristics of small airway epithelial dysfunction in patients with IPF and explore novel therapeutic interventions to impede IPF progression by targeting the dysfunctional small airways.
View Article and Find Full Text PDFLung
January 2025
Department of Respiratory Medicine, Affiliated Huzhou Hospital, Zhejiang University School of Medicine, Huzhou, People's Republic of China.
Purpose: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic lung disorder characterized by dry cough, fatigue, and exacerbated dyspnea. The prognosis of IPF is notably unfavorable, becoming extremely poor when the disease advances acutely. Effective therapeutic intervention is essential to mitigate disease progression; hence, early diagnosis and treatment are paramount.
View Article and Find Full Text PDFLung
January 2025
Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, No.1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.
Purposes: Immunoglobulin G4-related disease (IgG4-RD) and plasma cell-type idiopathic multicentric Castleman disease (PC-iMCD) have many overlapping features. Their differential diagnosis is challenging and crucial for clinical management due to their different prognoses and treatments. However, reports that compare these conditions are scarce, especially for patients with lung involvement.
View Article and Find Full Text PDFEur J Hum Genet
January 2025
Research Unit, Hospital Universitario Nuestra Señora de Candelaria, Instituto de Investigación Sanitaria de Canarias (IISC), Santa Cruz de Tenerife, Spain.
Idiopathic pulmonary fibrosis (IPF) is a progressive, late-onset disease marked by lung scarring and irreversible loss of lung function. Genetic factors significantly contribute to both familial and sporadic cases, yet there are scarce evidence-based studies highlighting the benefits of integrating genetics into the management of IPF patients. In this study, we performed whole-exome sequencing and telomere length (TL) measurements on IPF patients and their relatives.
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