►发现于原发性胆汁性胆管炎患者，以及其他自身免疫性肝病和系统性自身免疫性风湿疾病患者

►如临床怀疑为原发性胆汁性胆管炎，建议随访检测抗gp210抗体;该抗原包含在疾病特异性免疫测定试剂中（例如，扩展的自免肝抗体谱）（62-64）

►其他被识别的抗原包括p62核孔蛋白、LBR和Tpr；目前还没有针对这些自身抗体的特异性免疫检测商品化试剂盒（65-68）

*实用的炎症性肌病谱、系统性硬化症谱和自免肝抗体谱(扩展版)检测可能仅限于专业的临床实验室。

► Found in patients with PBC, as well as patients with other autoimmune liver diseases and SARD (61)

► If PBC is clinically suspected, it is recommended to perform a follow-up test for anti-gp210 antibodies; the antigen is included in disease specific immunoassays (i.e., extended liver profile*) (62–64)

► Other antigens recognized include p62 nucleoporin, LBR, and Tpr; specific immunoassays for these autoantibodies are currently not commercially available (65–68)

*Availability of the inflammatory myopathy profile, the SSc profile and the (extended) liver profile may be limited to specialty clinical laboratories.

61. Miyachi K, Hankins RW, Matsushima H, et al. Profile and clinical significance of anti-nuclear envelope antibodies found in patients with primary biliary cirrhosis: a multicenter study. J Autoimmun 2003;20:247–54.

62. Courvalin JC, Lassoued K, Bartnik E, et al. The 210-kD nuclear envelope polypeptide recognized by human autoantibodies in primary biliary cirrhosis is the major glycoprotein of the nuclear pore. J Clin Invest 1990;86:279–85.

63. Nickowitz RE, Worman HJ. Autoantibodies from patients with primary biliary cirrhosis recognize a restricted region within the cytoplasmic tail of nuclear pore membrane glycoprotein gp210. J Exp Med 1993;178:2237–42.

64. Bandin O, Courvalin JC, Poupon R, et al. Specificity and sensitivity of gp210 autoantibodies detected using an enzyme-linked immunosorbent assay and a synthetic polypeptide in the diagnosis of primary biliary cirrhosis. Hepatology 1996;23:1020–4.

65. Wesierska-Gadek J, Klima A, Komina O, et al. Characterization of autoantibodies against components of the nuclear pore complexes: high frequency of anti-p62 nucleoporin antibodies. Ann N Y Acad Sci 2007;1109:519–30.

66. Kraemer DM, Tony HP. Nuclear pore protein p62 autoantibodies in systemic lupus erythematosus. Open Rheumatol J 2010;4:24–7.

67. Courvalin JC, Lassoued K, Worman HJ, et al. Identification and characterization of autoantibodies against the nuclear envelope lamin B receptor from patients with primary biliary cirrhosis. J Exp Med 1990;172:961–7.

68. Ou Y, Enarson P, Rattner JB, et al. The nuclear pore complex protein TPR is a common autoantigen in sera that demonstrate nuclear envelope staining by indirect immunofluorescence. Clin Exp Immunol 2004;136:379–87.

61. Miyachi K, Hankins RW, Matsushima H, et al. Profile and clinical significance of anti-nuclear envelope antibodies found in patients with primary biliary cirrhosis: a multicenter study. J Autoimmun 2003;20:247–54.

62. Courvalin JC, Lassoued K, Bartnik E, et al. The 210-kD nuclear envelope polypeptide recognized by human autoantibodies in primary biliary cirrhosis is the major glycoprotein of the nuclear pore. J Clin Invest 1990;86:279–85.

63. Nickowitz RE, Worman HJ. Autoantibodies from patients with primary biliary cirrhosis recognize a restricted region within the cytoplasmic tail of nuclear pore membrane glycoprotein gp210. J Exp Med 1993;178:2237–42.

64. Bandin O, Courvalin JC, Poupon R, et al. Specificity and sensitivity of gp210 autoantibodies detected using an enzyme-linked immunosorbent assay and a synthetic polypeptide in the diagnosis of primary biliary cirrhosis. Hepatology 1996;23:1020–4.

65. Wesierska-Gadek J, Klima A, Komina O, et al. Characterization of autoantibodies against components of the nuclear pore complexes: high frequency of anti-p62 nucleoporin antibodies. Ann N Y Acad Sci 2007;1109:519–30.

66. Kraemer DM, Tony HP. Nuclear pore protein p62 autoantibodies in systemic lupus erythematosus. Open Rheumatol J 2010;4:24–7.

67. Courvalin JC, Lassoued K, Worman HJ, et al. Identification and characterization of autoantibodies against the nuclear envelope lamin B receptor from patients with primary biliary cirrhosis. J Exp Med 1990;172:961–7.

68. Ou Y, Enarson P, Rattner JB, et al. The nuclear pore complex protein TPR is a common autoantigen in sera that demonstrate nuclear envelope staining by indirect immunofluorescence. Clin Exp Immunol 2004;136:379–87.

►抗p62核孔蛋白抗体见于原发性胆汁性胆管炎和系统性红斑狼疮（23-24）

►抗LBR抗体见于原发性胆汁性胆管炎（25）

►抗Tpr抗体见于原发性胆汁性胆管炎、自身免疫性肝病、系统性红斑狼疮、系统性硬化症和干燥综合征（26）

注意：大多数报告都描述了直接结合特定抗原的自身抗体（例如，抗原特异性免疫检测），实际上并没有显示出与AC-12核型的明确关联；目前还没有针对这些自身抗体的特异性免疫检测商品化试剂。

► Anti‐p62 nucleoporin antibodies have been described in PBC and SLE (23, 24)

► Anti‐LBR antibodies have been described in PBC (25)

► Anti‐Tpr antibodies have been described in PBC, autoimmune liver disease, SLE, SSc and SjS (26)

Notes: Most reports describe autoantibodies directly binding to specific antigens (i.e., antigen‐specific immunoassays) and do not actually show clear correlations with the AC‐12 pattern as such; specific immunoassays for these autoantibodies are currently not commercially available.

23. Wesierska‐Gadek J, Klima A, Komina O, et al. Characterization of Autoantibodies against Components of the Nuclear Pore Complexes: High Frequency of Anti‐p62 Nucleoporin Antibodies. Ann NY Acad Sci 2007;1109:519–30.

24. Kraemer DM, Tony H‐P. Nuclear Pore Protein p62 Autoantibodies in Systemic Lupus Erythematosus. Open Rheumatol J 2010;4:24‐7.

25. Courvalin J‐C, Lassoued K, Worman HJ, et al. Identification and Characterization of Autoantibodies Against the Nuclear Envelope Lamin B Receptor from Patients with Primary Biliary Cirrhosis. J Exp Med 1990;172:961‐7.

26. Ou Y, Enarson P, Rattner JB, et al. The nuclear pore complex protein Tpr is a common autoantigen in sera that demonstrate nuclear envelope staining by indirect immunofluorescence. Clin Exp Immunol 2004;136:379–87.

23. Wesierska‐Gadek J, Klima A, Komina O, et al. Characterization of Autoantibodies against Components of the Nuclear Pore Complexes: High Frequency of Anti‐p62 Nucleoporin Antibodies. Ann NY Acad Sci 2007;1109:519–30.

24. Kraemer DM, Tony H‐P. Nuclear Pore Protein p62 Autoantibodies in Systemic Lupus Erythematosus. Open Rheumatol J 2010;4:24‐7.

25. Courvalin J‐C, Lassoued K, Worman HJ, et al. Identification and Characterization of Autoantibodies Against the Nuclear Envelope Lamin B Receptor from Patients with Primary Biliary Cirrhosis. J Exp Med 1990;172:961‐7.

26. Ou Y, Enarson P, Rattner JB, et al. The nuclear pore complex protein Tpr is a common autoantigen in sera that demonstrate nuclear envelope staining by indirect immunofluorescence. Clin Exp Immunol 2004;136:379–87.