ORAL CYCLOPHOSPHAMIDE VS ORAL PLACEBO IN SYSTEM SCLEROSIS PULMONARY AVEOLITIS

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。这项研究旨在评估环磷酰胺(一种强大的抗炎和免疫抑制药物，商品名为环磷酰胺)在预防硬皮病造成的肺损伤方面是否比安慰剂(一种惰性的，没有有效治疗方法)更好。这条铁路将每天每公斤环磷酰胺2毫克(片剂形式约为100-150毫克)与看起来像环磷酰胺但具有惰性的安慰剂进行比较，并考虑到剂量调整的毒性。在之前几个中心的研究中，据报道，在开始使用环磷酰胺后，肺功能得到了改善。在最近的一项研究中，3/4的环磷酰胺患者和1/4的未治疗患者的肺功能改善了10%以上。这项研究旨在以科学的方式检查环磷酰胺是否确实改善了早期肺部受累的硬皮病患者的肺功能。

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. This study has been designed to evaluate whether cyclophosphamide (a strong anti-inflammatory and immunosuppressant medication, trade named Cytoxan) is better than placebo (an inert, no effective treatment) in preventing lung damage from scleroderma from getting worse. This rail will compare 2 milligrams per kilogram per day of cyclophosphamide (approximately 100-150 mg in tablet form) with a placebo that looks like cyclophosphamide but is inert, with allowances for dose adjustments for toxicity. In previous studies from several centers, improvement in lung function was reported to have occurred after starting cyclophosphamide. In one recent study, lung function improved by greater than 10% in 3/4 of the cyclophosphamide patients and in 1/4 of the non-treated patients. This study is designed toexamine in a scientific fashion whether cyclophosphamide does actually improve lung function in people with scleroderma with early lung involvement.