BOSANTAN IN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSIS

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目及 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 特发性肺纤维化（IPF），也称为隐源性纤维化肺泡炎，是一种属于间质性肺病谱的独特临床疾病。 IPF是一种进行性疾病，其特征为手术肺活检时存在普通型间质性肺炎（UIP）的组织学模式。 尚无治疗显示可改善IPF患者的生存期或生活质量。 目前的治疗仍然基于先前的假设，即IPF是一种炎症过程，同时伴有纤维化导致的肺重塑。 因此，它涉及抗炎治疗，包括皮质类固醇（例如，泼尼松，泼尼松龙），免疫抑制剂/细胞毒性剂（例如，硫唑嘌呤、环磷酰胺）或两者的组合。 然而，由于当前治疗的边际获益和严重副作用，沿着对IPF发病机制的新见解，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，目前正在研究多种药物，包括秋水仙碱、D-青霉胺、干扰素γ和吡非尼酮。 肺移植已成为某些IPF患者的可行选择。 临床和实验研究表明，波生坦安全且耐受性良好，可延缓IPF的进展，IPF是一种尚无有效治疗的疾病。 本BUILD-1研究探讨了波生坦适应症范围（目前已获批用于治疗中度至重度PAH）扩展至新类别IPF患者的可能性。

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.