摘要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-Ⅳ 56.5±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 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-Ⅳ 54.8±45.2% vs 74.4±25.6%, P = 0.006), AMA titer (≤ 1:160/> 1:160 51.6±48.4% vs 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 -Ⅳ 57.9±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 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.