Anti-mutated citrullinated vimentin antibodies in antiphospholipid syndrome: diagnostic value and relationship with clinical features

Cristiano Alessandri, Nancy Agmon-Levin, Fabrizio Conti, Carlo Perricone, Elena Ortona, Monica Pendolino, Antonella Capozzi, Federica Delunardo, Riccardo Mancini, Simona Truglia, Francesca Romana Spinelli, Fulvia Ceccarelli, Maurizio Sorice, Yehuda Shoenfeld, Guido Valesini

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Abstract: Antiphospholipid antibodies (aPLs) are a heterogeneous group of autoantibodies essential for the diagnosis of antiphospholipid syndrome (APS) but do not predict clinical manifestations or disease progression. Hence, the co-presence of other antibodies may prove useful. Autoimmunity directed toward vimentin and other citrullinated peptides was established in rheumatoid arthritis (RA) and in other autoimmune conditions including systemic lupus erythematosus (SLE). We have previously described the presence of autoantibodies directed against vimentin/cardiolipin complex in patients with antiphospholipid syndrome (APS), but there are no data on the role of citrullinated vimentin in APS. Thus, we evaluated the prevalence and clinical significance of anti-MCV in APS patients. The study group consisted of 79 unselected outpatients with APS. Control groups included 25 patients with SLE, 30 patients with RA, and 20 healthy subjects age- and sex-matched. To detect anti-MCV, anti-vimentin, anti-vimentin/cardiolipin, and anti-CCP2 antibodies, commercial or homemade enzyme-linked immunosorbent assays (ELISA) were performed. Anti-MCV antibodies were found in a high percentage of APS patients (26.6%). A significant correlation between anti-MCV and anti-vimentin/cardiolipin serum levels was observed (p = 0.029). Moreover, vimentin reactivity was increased by its citrullination or conjugation with cardiolipin (p = 0.01 and p < 0.001, respectively). Interestingly, anti-MCV was found associated with the presence of arthritis (p = 0.011) and anti-vimentin/cardiolipin was highly specific for the presence of arterial or venous thrombosis in APS (p = 0.003 and p = 0.002, respectively). The detection of additional autoantibodies may contribute to clinical assessment of APS patients. Citrullination may occur in APS and play a role in the pathogenesis of this condition.[Figure not available: see fulltext.] Key Points: •Anti-MCV antibodies can be found in APS patients and are associated with the presence of arthritis. •Anti-vimentin/cardiolipin is strongly associated with the presence of thrombosis (both arterial and venous). •Citrullination occurs in APS, participate in disease pathogenesis, and influence clinical picture.

Original languageEnglish
Pages (from-to)524-531
Number of pages8
JournalImmunologic Research
Issue number2
Publication statusPublished - Apr 1 2017


  • Anti-CCP
  • Anti-MCV
  • aPL
  • APS

ASJC Scopus subject areas

  • Immunology


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