BOSANTAN IN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。特发性肺纤维化（IPF），也被称为隐源性纤维化肺泡炎，是一种独特的临床疾病，属于间质性肺疾病谱系。IPF是一种进行性疾病，其特征是在外科肺活检中表现为通常间质性肺炎（UIP）的组织学模式。没有任何疗法被证明可以改善IPF患者的生存或生活质量。目前的治疗仍然基于先前的假设，即IPF是一种炎症过程，同时伴有肺纤维化重塑。因此，它涉及抗炎治疗，包括皮质类固醇（例如，强的松，泼尼松），免疫抑制/细胞毒性药物（例如，硫唑嘌呤，环磷酰胺）或两者的组合。然而，由于目前治疗方法的边际效益和严重的副作用，以及对IPF发病机制的新认识，迫切需要新的治疗方法。抗纤维化治疗旨在减少基质沉积或增加胶原分解，包括秋水仙碱、d -青霉胺、干扰素和吡非尼酮在内的许多药物目前正在研究中。肺移植已成为一些IPF患者的可行选择。临床和实验研究表明，波生坦是安全的，耐受性良好，可以延缓IPF的进展，目前尚无有效的治疗方法。目前的BUILD-1研究调查了波生坦的适应症范围的可能扩展，波生坦目前被批准用于治疗中度至重度PAH，以治疗IPF患者的新类别。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is a distinct clinical disorder belonging to the spectrum of interstitial lung disease. IPF is a progressive disease characterized by the presence of a histological pattern of usual interstitial pneumonia (UIP) on surgical lung biopsy. No therapies have been shown to improve the survival or quality of life for patients with IPF. Current treatment is still based on the former presumption that IPF is an inflammatory process with concurrent remodeling of the lung by fibrosis. Consequently, it involves anti-inflammatory therapy, including corticosteroids (e.g., prednisone, prednisolone), immunosuppressive/cytotoxic agents (e.g., azathioprine, cyclophosphamide) or a combination of both. However, because of the marginal benefit and serious side effects of the current therapies, along with newer insights into the pathogenesis of IPF, novel therapeutic approaches are highly needed. Antifibrotic therapy is aimed at decreasing matrix deposition or increasing collagen breakdown and a number of agents including colchicine, D-penicillamine, interferon y, and pirfenidone, are currently under investigation. Lung transplantation has emerged as a viable option for some patients with IPF. Clinical and experimental studies suggest that bosentan would be safe and well tolerated and could delay the progression of IPF, a condition for which no established efficacious treatment is availale. The present BUILD-1 study investigates a posssible extension of the indication range of bosentan, which is currently approved for the treatment of moderate to severe PAH, to a new category of patients suffering from IPF.