Document Type : Review article
Authors
1
Department of Biochemistry, Faculty of Pharmacy, Minia University, El-Minia 61519, Egypt.
2
Department of Medicinal Chemistry, Faculty of Pharmacy, Minia University, El-Minia 61519, Egypt.
Abstract
A comprehensive overview of idiopathic pulmonary fibrosis (IPF) focusing on its pathogenesis, diagnosis, treatment, and preclinical research models, particularly the bleomycin (BLM) model. IPF is a progressive, degenerative lung disease of unknown origin, primarily affecting older individuals, and characterized by irreversible lung fibrosis. The article discusses the critical role of inflammation, apoptosis, oxidative stress, and growth factors such as TGF-β1, PDGF, and TNF-α in IPF development. Despite advances in pharmacological treatments such as pirfenidone and nintedanib, IPF remains incurable, with lung transplantation being the only definitive solution for select patients. Furthermore, the bleomycin-induced pulmonary fibrosis model is highlighted for its relevance in preclinical research, providing insights into the disease’s molecular mechanisms. The article also examines the pharmacokinetics, mechanisms of action, and adverse effects of bleomycin, a chemotherapeutic agent used in the BLM model. Lastly, the potential of pyrimido-diazepine derivatives, particularly diazepino[4,3-c]quinoline- 6,8,10-trione, is explored for their therapeutic potential in managing IPF. Overall, this article emphasizes the importance of continued research to discover novel treatments with fewer side effects for IPF patients and the utility of animal models in understanding disease progression and testing new therapies.
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