Antoniu Sabina A
Pulmonary Disease-Medicine II Department, University of Medicine and Pharmacy Grigore T Popa, Pulmonary Disease University Hospital, 30 Dr I CihacStr, Iasi, 700115, Romania.
Multidiscip Respir Med. 2012 Nov 12;7(1):41. doi: 10.1186/2049-6958-7-41.
Idiopathic pulmonary fibrosis is a rare, life threatening disease characterized by an anarchic fibrogenesis, limited survival and few therapeutic options. Its pathogenesis is complex and involves the interaction among various pathways driven by proinflammatory/profibrogenetic mediators such as platelet -derived growth factor, vascular endothelial growth factor or basic fibroblast growth factor. Given their prominent pathogenic roles in this disease such growth factor might be suitable therapeutic targets.In fact, the existing preclinical and clinical data demonstrated that their therapeutic inhibition results in a delayed progression of the pulmonary fibrosis and in the improvement of the disease outcome. BIBF 1120 is a potent triple blocker of the receptors of these growth factors which is currently evaluated as a potential therapy in the idiopathic pulmonary fibrosis. This review discusses the existing data supporting its potential use in this disease.
特发性肺纤维化是一种罕见的、危及生命的疾病，其特征是无序的纤维生成、生存期有限且治疗选择较少。其发病机制复杂，涉及由促炎/促纤维化介质（如血小板衍生生长因子、血管内皮生长因子或碱性成纤维细胞生长因子）驱动的各种途径之间的相互作用。鉴于这些生长因子在该疾病中突出的致病作用，它们可能是合适的治疗靶点。事实上，现有的临床前和临床数据表明，对它们进行治疗性抑制可导致肺纤维化进展延迟并改善疾病结局。BIBF 1120是这些生长因子受体的强效三联阻滞剂，目前正作为特发性肺纤维化的一种潜在治疗方法进行评估。本综述讨论了支持其在该疾病中潜在应用的现有数据。
