NATURAL HISTORY OF PRIMARY BILIARY CIRRHOSIS AFTER TREATMENT WITH METHOTREXATE

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 熊去氧胆酸(UDCA)是目前唯一被批准用于治疗PBC的药物。它已被证明可以延长肝脏移植的时间，并可能延长存活时间。UDCA通过减少称为胆盐的有毒化合物的破坏性影响来做到这一点，当胆管受损时，这种化合物会在肝脏中积聚。然而，UDCA对免疫系统没有影响，因此对导致疾病的胆管的免疫损伤也没有影响。因此，免疫抑制药物(降低免疫系统的药物)，如甲氨蝶呤和他克莫司，已作为PBC的治疗方法在临床试验中进行了评估。尽管这些研究表明这些药物在处方剂量下是安全的，但它们潜在的长期副作用尚不清楚。 这项研究的目的是找出甲氨蝶呤或他克莫司是否会对参加该机构PBC临床试验评估这些药物的患者造成延迟的、以前未被认识到的有害或有益的影响。受试者将在 根据严重程度，每隔3、6或12个月。这是一项观察性研究。

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. Ursodeoxycholic acid (UDCA) is currently the only approved drug for the treatment of PBC. It has been shown to prolong the time to liver transplant and may prolong survival. UDCA does this by reducing the damaging effects of toxic compounds called bile salts that can build up in the liver when there is damage to the ducts. However, UDCA has no effect on the immune system and thus the immune injury to the bile ducts responsible for the disease. Immunosuppressive medications (drugs that lower the immune system), such as methotrexate and tacrolimus, therefore, have been evaluated in clinical trials as treatment for PBC. Even though such studies have shown these drugs to be safe at the dosages prescribed, their potential long-term side effects are unknown. The purpose of this study is to find out if methotrexate or tacrolimus may cause delayed, previously unrecognized effects, either harmful or beneficial, to patients who participated in clinical trials evaluating these medications in PBC at this institution. Subjects will be seen at 3, 6, or 12 month intervals depending upon the severity. This is an observational study.