Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal lung disease of which the origin and development mechanisms remain unknown. The few available pharmacological treatments can only slow the progression of the disease. The development of curative treatments is hampered by the absence of experimental models that can mimic the specific pathophysiological mechanisms of IPF. The aim of this mini-review is to provide an overview of the most commonly used experimental animal models in the study of IPF and to underline the urgent need to seek out new, more satisfactory models.
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http://dx.doi.org/10.1016/j.rmr.2024.02.009 | DOI Listing |
Xenobiotica
January 2025
Department of Pharmacy, School of Health Sciences, National and Kapodistrian University of Athens, Athens, Greece.
Idiopathic Pulmonary Fibrosis (IPF) is a chronic respiratory disorder for which pirfenidone is the recommended first-line anti-fibrotic treatment. While pirfenidone has demonstrated efficacy in slowing the progression of IPF, its use is associated with several challenges and unresolved issues that impact patient outcomes. Pirfenidone administration can result in gastrointestinal side effects, photosensitivity reactions, and significant drug interactions, particularly in patients with hepatic impairment.
View Article and Find Full Text PDFExp Biol Med (Maywood)
December 2024
Department of Pediatric Surgery, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China.
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with a poor prognosis. Its non-specific clinical symptoms make accurate prediction of disease progression challenging. This study aimed to develop molecular-level prognostic models to personalize treatment strategies for IPF patients.
View Article and Find Full Text PDFJ Clin Transl Endocrinol
December 2024
Division of Endocrinology Diabetes and Metabolism, Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
Cystic fibrosis-related diabetes (CFRD) is the most common non-pulmonary comorbidity in people with cystic fibrosis (CF). Current guidelines recommend insulin therapy as the treatment of choice for people with CFRD. In the past, obesity and overweight were uncommon in individuals with CF.
View Article and Find Full Text PDFRespir Med Case Rep
November 2024
Infectious and Tropical Diseases Unit, Careggi University Hospital, Florence, Italy.
Cystic fibrosis (CF)-related central (CNS) and peripheral nervous system (PNS) disorders have not yet been fully described. We report the first case of post-infective neuromuscular hyperexcitability syndrome in a 23-year-old male patient with CF and pulmonary exacerbation. CNS radiological investigations were unremarkable and no autoantibodies were detected.
View Article and Find Full Text PDFFront Immunol
December 2024
Department of Biomedical Sciences, University of Sassari, Sassari, Italy.
Introduction: The critical role played by vascular dysfunction and ineffective angiogenesis in the pathophysiology of systemic sclerosis (SSc) suggests that circulating biomarkers reflecting these alterations may be useful in the clinical evaluation of this patient group. We sought to address this issue by conducting a systematic review and meta-analysis of studies investigating a such candidate biomarker, endostatin, an endogenous glycoprotein exerting anti-angiogenic effects, in SSc patients and healthy controls.
Methods: A literature search was conducted in the electronic databases Web of Science, PubMed, and Scopus from inception to 27 May 2024.
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