CLINICAL EFFICACY OF REMICADE IN CBD

该子项目是利用 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 中心，不一定是研究者的机构。 慢性铍中毒（CBD）是一种由吸入铍颗粒引起的免疫系统疾病。 暴露和疾病发生在使用铍和铍合金的工作场所，导致2-6%的工人患病。 泼尼松是CBD最常用的治疗方法。 停药通常会导致疾病症状复发。 重新开始类固醇治疗通常会改善患者的病情，然而，持续使用泼尼松会导致各种副作用。 即使使用类固醇治疗，许多患者仍然继续恶化。 治疗可能会减缓疾病的临床进展，但通常不会停止这种进展。 在一些患者中，CBD对泼尼松或其他治疗没有反应，如果在中期或晚期诊断，可能是致命的。 因此，有必要测试有前途的新疗法治疗这种疾病。 这项研究旨在测试一种名为英夫利西单抗的药物在CBD中的临床有效性。 这种药物可以减少一种称为TNF-α的物质，这种物质与肺部更严重的疾病和炎症有关。 接受英夫利西单抗可能有助于症状，并可能改善临床测试，如呼吸测试。 基线和随访测试将寻找呼吸测试（肺功能测试），肺部氧气交换（运动测试），胸部X射线和肺部炎症的改善。

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. Chronic Beryllium Disease (CBD) is an immune system disorder caused by the inhalation of beryllium particles. Exposures and disease occurs in workplaces in which beryllium and beryllium alloys are used, producing disease in 2-6% of workers. Prednisone is the most commonly prescribed treatment for CBD. Cessation of the drug usually leads to relapse of the disease symptoms. Restarting steroid treatment usually improves the patient's condition, however, continual use of prednisone can cause a variety of side effects. Even with steroid treatment, many patients still continue to worsen. The treatment may slow the clinical progression of disease, but often does not halt that progression. In some patients CBD does not respond to prednisone or other therapy and can be fatal if diagnosed at intermediate or late stages. As such, there is a need to test promising new therapies for this disorder. This research study is designed to test the clinical effectiveness of a drug called infliximab in CBD. This drug may reduce a substance called TNF-alpha, which is associated with more severe disease and inflammation in the lung. Receiving infliximab may help with symptoms and may improve clinical testing, such as breathing tests. Baseline and follow up testing will look for improvements in breathing tests (Pulmonary Function Testing), exchange of oxygen in the lungs (exercise test), chest x-ray, and lung inflammation.