Nakajima M, Shimizu H, Miyazaki A, Watanabe S, Kitami N, Sato N
Department of Gastroenterology, Juntendo University School of Medicine, Tokyo, Japan.
J Gastroenterol. 1999 Oct;34(5):607-12. doi: 10.1007/s005350050380.
Autoimmune cholangitis (AIC) has been proposed as a distinct disease entity from primary biliary cirrhosis (PBC), without antimitochondrial antibody (AMA) and anti-M2 antibody but with a high titer of antinuclear antibody (ANA) in the serum. However, negativity for AMA and anti-M2 antibody was determined by different methods in different studies. We hypothesized that anti-M2 antibody negativity in AIC resulted from methodological differences, including selection of the immunoglobulin subclass of the autoantibody. Twenty-three patients compatible with AIC whose serum tested negative for AMA and positive for ANA (> or = 1:80) were compared with 71 AMA-positive PBC patients. Laboratory findings, histology, and the pattern of anti-M2 antibody assessed by immunoblotting were compared. Alkaline phosphatase, total bilirubin, total cholesterol, and IgM values were lower in patients with AIC (P < 0.05, 0.01, respectively). Anti-smooth muscle antibody was detected more frequently in patients with AIC (P < 0.01). However, anti-M2 antibody was detected using immunoblotting not only in PBC but also in AIC cases. IgA class alone, IgM class alone, or both IgA and IgM classes of anti-M2 antibody were detected in 13%, 17%, and 22% of AIC patients, respectively, whereas they were not detected in PBC patients (P < 0.05, P < 0.01, P < 0.01). IgG class anti-M2 was detected in all patients with PBC, whereas it was detected in 48% of patients with AIC (P < 0.01). Histological evaluation showed that the early stages of disease were found more frequently in AIC (78%) than in PBC patients (39%) (P < 0.01). Anti-M2 antibody was detected by immunoblotting in all AIC patients. Hence, AIC is not a distinct disease from PBC. For diagnosing AIC and/or PBC, anti-M2 antibody should be examined by the immunoblotting assay to detect not only IgG but also IgA and IgM subclasses.
自身免疫性胆管炎(AIC)已被提出是一种与原发性胆汁性肝硬化(PBC)不同的疾病实体,其血清中无抗线粒体抗体(AMA)和抗M2抗体,但抗核抗体(ANA)滴度较高。然而,在不同研究中,AMA和抗M2抗体的阴性判定采用了不同方法。我们推测,AIC中抗M2抗体阴性是由方法学差异导致的,包括自身抗体免疫球蛋白亚类的选择。将23例符合AIC且血清AMA检测阴性、ANA阳性(≥1:80)的患者与71例AMA阳性的PBC患者进行比较。比较了实验室检查结果、组织学以及通过免疫印迹评估的抗M2抗体模式。AIC患者的碱性磷酸酶、总胆红素、总胆固醇和IgM值较低(分别为P < 0.05,P < 0.01)。AIC患者中抗平滑肌抗体的检测更为频繁(P < 0.01)。然而,通过免疫印迹不仅在PBC患者中,而且在AIC患者中也检测到了抗M2抗体。分别在13%、17%和22%的AIC患者中检测到单独的IgA类、单独的IgM类或IgA和IgM两类抗M2抗体,而在PBC患者中未检测到(P < 0.05,P < 0.01,P < 0.01)。所有PBC患者均检测到IgG类抗M2,而在48%的AIC患者中检测到(P < 0.01)。组织学评估显示,疾病早期在AIC患者中(78%)比在PBC患者中(39%)更常见(P < 0.01)。通过免疫印迹在所有AIC患者中均检测到抗M2抗体。因此,AIC并非与PBC不同的疾病。为诊断AIC和/或PBC,应通过免疫印迹法检测抗M2抗体,以不仅检测IgG,还检测IgA和IgM亚类。