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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
▶ 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. |
| First level information references |
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. |
| Second level information |
▶ 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. |
| Second level information references |
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. |
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