Kim W R, Poterucha J J, Jorgensen R A, Batts K P, Homburger H A, Dickson E R, Krom R A, Wiesner R H, Lindor K D
Division of Gastroenterology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Hepatology. 1997 Jul;26(1):22-6. doi: 10.1002/hep.510260103.
Approximately 5% to 10% of patients with features otherwise consistent with primary biliary cirrhosis (PBC) lack antimitochondrial antibodies (AMA). Most of these patients have other autoantibodies, a syndrome recently named "autoimmune cholangitis." We report our experience in patients with AMA-negative PBC treated with ursodeoxycholic acid (UDCA) and/or liver transplantation (OLT). The study of response to UDCA was performed as follows. While recruiting patients for a previously reported multicenter trial, we identified 8 patients with AMA-negative PBC. The patients were given UDCA and followed up at regular intervals. The characteristics of AMA-negative patients at presentation were similar to those of AMA-positive patients with PBC. The clinical outcomes and sequential liver biochemistries of UDCA treatment were also comparable with those of AMA-positive patients. The study of outcome of OLT was performed as follows. We identified OLT recipients at the Mayo Clinic who had clinical, radiological, and histological features compatible with PBC. Their pretransplant AMA status was determined, and each AMA-negative patient was paired with 2 AMA-positive patients. Of 85 OLT recipients with a diagnosis of PBC, 6 (7.1%) were AMA negative, including 1 who had undergone UDCA therapy. After a median of 36 months of follow-up, graft and patient survival rates and subsequent histological changes (disease recurrence and steroid-resistant or late rejections) were comparable in AMA-negative and -positive PBC patients. In summary, in our experience of 13 AMA-negative PBC patients (including 9 who met the criteria for a diagnosis of autoimmune cholangitis), treatment with UDCA or OLT resulted in similar outcomes to those found in AMA-positive patients. We conclude that AMA status does not affect the response in PBC patients to treatment with UDCA or OLT.
约5%至10%具有原发性胆汁性肝硬化（PBC）其他特征的患者缺乏抗线粒体抗体（AMA）。这些患者大多有其他自身抗体，这种综合征最近被命名为“自身免疫性胆管炎”。我们报告了用熊去氧胆酸（UDCA）和/或肝移植（OLT）治疗AMA阴性PBC患者的经验。对UDCA反应的研究如下进行。在为之前报道的一项多中心试验招募患者时，我们确定了8例AMA阴性的PBC患者。给予这些患者UDCA并定期随访。AMA阴性患者就诊时的特征与AMA阳性的PBC患者相似。UDCA治疗的临床结果和连续肝脏生化指标也与AMA阳性患者相当。对OLT结果的研究如下进行。我们在梅奥诊所确定了具有与PBC相符的临床、放射学和组织学特征的OLT受者。确定了他们移植前的AMA状态，每例AMA阴性患者与2例AMA阳性患者配对。在85例诊断为PBC的OLT受者中，6例（7.1%）为AMA阴性，其中1例曾接受UDCA治疗。中位随访36个月后，AMA阴性和阳性的PBC患者在移植物和患者生存率以及随后的组织学变化（疾病复发和类固醇抵抗或晚期排斥反应）方面相当。总之，在我们对13例AMA阴性PBC患者（包括9例符合自身免疫性胆管炎诊断标准的患者）的经验中，UDCA或OLT治疗的结果与AMA阳性患者相似。我们得出结论，AMA状态不影响PBC患者对UDCA或OLT治疗的反应。
