论文部分内容阅读
AIM:To investigate possible associations of anti-nuclear envelope antibody(ANEA)with disease severity and survival in Greek primary biliary cirrhosis(PBC)patients.METHODS:Serum samples were collected at diagnosis from 147 PBC patients(85%female),who were followed-up for a median 89.5 mo(range 1-240).ANEA were detected with indirect immunofluorescence on 1% formaldehyde fixed Hep2 cells,and anti-gp210 antibodies were detected using an enzyme linked immunosorbent assay.Findings were correlated with clinical data,histology,and survival.RESULTS:ANEA were detected in 69/147(46.9%) patients and 31/147(21%)were also anti-gp210 positive.The ANEA positive patients were at a more advanced histological stage(Ⅰ-Ⅱ/Ⅲ-Ⅳ56.5%/43.5% vs 74.4%/25.6%,P=0.005)compared to the ANEA negative ones.They had a higher antimitochondrial antibodies(AMA)titer(≤1:160/>1:160 50.7%/49.3%vs 71.8%/28.2%,P=0.001)and a lower survival time(91.7 ±50.7 mo vs 101.8±55 mo,P=0.043).Moreover,they had more advanced fibrosis,portal inflammation,interface hepatitis,and proliferation of bile ductules(P =0.008,P=0.008,P=0.019,and P=0.027,respectively).They also died more frequently of hepatic failure and/or hepatocellular carcinoma(P=0.016).ANEA positive,anti-gp210 positive patients had a difference in stage(Ⅰ-Ⅱ/Ⅲ-Ⅳ54.8%/45.2%vs 74.4%/25.6%,P= 0.006),AMA titer(≤1:160/>1:160 51.6%/48.4%vs 71.8%/28.2%,P=0.009),survival(91.1±52.9 mo vs 101.8±55 mo,P=0.009),and Mayo risk score(5.5 ±1.9 vs 5.04±1.3,P=0.04)compared to the ANEA negative patients.ANEA positive,anti-gp210 negative patients had a difference in AMA titer(≤1:160/>1:160 50%/50%vs 71.8%/28.2%,P=0.002),stage(Ⅰ-Ⅱ/Ⅲ -Ⅳ57.9%/42.1%vs 74.4%/25.6%,P=0.033),fibrosis(P=0.009),portal inflammation(P=0.018),interface hepatitis(P=0.032),and proliferation of bile ductules(P=0.031).Anti-gp210 positive patients had a worse Mayo risk score(5.5±1.9 vs 4.9±1.7,P=0.038)than the anti-gp210 negative ones.CONCLUSION:The presence of ANEA and anti-gp210 identifies a subgroup of PBC patients with advanced disease severity and poor prognosis.
AIM: To investigate Possible associations of anti-nuclear envelope antibody (ANEA) with disease severity and survival in Greek primary biliary cirrhosis (PBC) patients. METHODS: Serum samples were collected at diagnosis from 147 PBC patients (85% female), who were followed by up for a median of 89.5 mo (range 1-240). ANEA were detected with indirect immunofluorescence on 1% formaldehyde fixed Hep2 cells, and anti-gp210 antibodies were detected using an enzyme linked immunosorbent assay. Findings were correlated with clinical data, histology, and survival .RESULTS: ANEA were detected in 69/147 (46.9%) patients and 31/147 (21%) were also anti-gp210 positive.The ANEA positive patients were at a more advanced histological stage (Ⅰ-Ⅱ / Compared to the ANEA negative ones. They had a higher antimitochondrial antibodies (AMA) titer (? 1: 160 /> 1: 160 50.7% / 35.5% / 43.5% vs 74.4% / 25.6% 49.3% vs 71.8% / 28.2%, P = 0.001) and a lower survival time (91.7 ± 50.7 mo vs 101.8 ± 55 mo, P = 0.043) .Moreover, they had more advanced fib rosis, portal inflammation, interface hepatitis, and proliferation of bile ductules (P = 0.008, P = 0.008, P = 0.019, and P = 0.027, respectively) 0.016), AEA titer (≤1: 160 / A16), ANEA positive, anti-gp210 positive patients had a difference in stage (Ⅰ-Ⅱ / Ⅲ-Ⅳ 54.8% / 45.2% vs 74.4% / 25.6%, P = (91.1 ± 52.9 mo vs 101.8 ± 55 mo, P = 0.009), and Mayo risk score (5.5 ± 1.9 vs 5.04 ± 1.3 vs. 51.8% / 28.2%, P = 0.009) , P = 0.04) compared to the ANEA negative patients. ANEA positive, anti-gp210 negative patients had a difference in AMA titer (≦ 1: 160 /> 1: 160 50% / 50% vs 71.8% / 28.2% 0.002), portal inflammation (P = 0.018), portal hepatitis (P = 0.008), stage hepatitis (P = 0.009) 0.032), and proliferation of bile ductules (P = 0.031). Anti-gp210 positive patients had a worse Mayo risk score (5.5 ± 1.9 vs 4.9 ± 1.7, P = 0.038) than the anti-gp210 negative ones. CONCLUSION: The presence of ANEA and anti-g p210 identifies a subgroup of PBC patients with advanced disease severity and poor prognosis.