Primary biliary cirrhosis-related autoantibodies in a large cohort of Italian patients with systemic sclerosis

Ilaria Cavazzana, Angela Ceribelli, Mara Taraborelli, Micaela Fredi, Gary Norman, Angela Tincani, Minoru Satoh, Franco Franceschini

Research output: Contribution to journalArticlepeer-review


Objective. To analyze the prevalence, associations, and fine specificity of autoantibodies to primary biliary cirrhosis (PBC)-associated antigens (MIT3, Sp100, and gp210) in a cohort of Italian patients with systemic sclerosis (SSc). Methods. Sera samples from 201 patients with SSc were tested for antibodies to MIT3, gp210, and Sp100 by ELISA (the PBC screen). Anti-MIT3-positive sera were studied for IgG or IgA isotypes. All sera were analyzed by indirect immunofluorescence on HEp-2 cells and on rodent kidney/stomach/liver tissue sections in order to detect antinuclear and antimitochondrial antibodies (AMA). SSc was selected by American College of Rheumatology criteria and classified based on LeRoy's criteria. Results. Forty-three (21.4%) sera samples were positive for PBC screen antibodies. Anti-MIT3 antibodies were detected in 36 samples, anti-Sp100 in 5, and anti-gp210 in 1 sample. The other 3 PBC screen-positive samples showed no specificity for the single antigens. PBC screen-positive patients more frequently showed a limited cutaneous SSc subtype (p = 0.04), anticentromere antibodies (ACA; p = 0.0013), elevated alkaline phosphatase (ALP) (p <0.0001), PBC (p = 0.002), and AMA (p = 0.008). Teleangiectasia and calcinosis were less frequent in this group of patients. IgG+IgA anti-MIT3-positive patients had higher prevalence of AMA (p = 0.0035), diagnosis of PBC (p = 0.014), and increased ALP (p = 0.039), all considered biochemical markers of severe liver disease. Conclusion. PBC screen antibodies were detected in 20% of patients with SSc, strongly associated with ACA. ACA+/PBC screen+ patients had higher risk of developing PBC or elevation of ALP. The Journal of Rheumatology

Original languageEnglish
Pages (from-to)2180-2185
Number of pages6
JournalJournal of Rheumatology
Issue number10
Publication statusPublished - Oct 2011


  • Alkaline phosphatase
  • Anticentromere
  • Antimitochondrial antibodies
  • Primary biliary cirrhosis
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy


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