Autoantibodies against nuclear pore complexes are associated with more active and severe liver disease in primary biliary cirrhosis

Pietro Invernizzi, Mauro Podda, Pier Maria Battezzati, Andrea Crosignani, Massimo Zuin, Eva Hitchman, Marco Maggioni, Pier Luigi Meroni, Edward Penner, Józefa Wesierska-Gadek

Research output: Contribution to journalArticlepeer-review

123 Scopus citations


Background/Aims: Antibodies against nuclear pore complexes (NPCs) have been detected in primary biliary cirrhosis (PBC), but their clinical relevance is still unsettled. Methods: We tested sera from 171 consecutive PBC patients and 230 control subjects (149 with autoimmune or viral liver diseases, 28 with systemic lupus erythematosus, and 53 healthy) by immunoblotting for antibodies against purified human NPCs. Results: Antibodies to NPCs were detected in 27% of the patients with PBC, were highly specific (97%), and were not associated with antimitochondrial antibodies. Their prevalence was higher in symptomatic patients (36 vs. 16%, P < 0.01) and was associated (P < 0.001) with more severe disease, as assessed by the presence of cirrhosis or its complications (13% prevalence in patients without cirrhosis, 31% in uncomplicated, and 54% in complicated cirrhosis), or by the application of the Mayo prognostic model (12% in the lowest, 21% in the median, 47% in the highest score tertile). Positive patients had higher levels of serum bilirubin (2.2 ± 3.7 vs. 1.0 ± 1.1 mg/dl, P < 0.01) and more marked inflammatory infiltrates on liver biopsy (P < 0.05). Conclusions: Autoantibodies to NPCs are more prevalent in PBC patients than in controls and are strongly associated with more active and severe disease.

Original languageEnglish (US)
Pages (from-to)366-372
Number of pages7
JournalJournal of Hepatology
Issue number3
StatePublished - 2001
Externally publishedYes


  • Antinuclear antibodies
  • Autoantibodies
  • Primary biliary cirrhosis

ASJC Scopus subject areas

  • Gastroenterology


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