Prevalence and Thrombotic Risk Assessment of Anti-β2 Glycoprotein i Domain i Antibodies: A Systematic Review

Massimo Radin, Irene Cecchi, Dario Roccatello, Pier Luigi Meroni, Savino Sciascia

Research output: Contribution to journalReview articlepeer-review


Background To date, the exact prevalence of anti-β 2 glycoprotein I domain I (anti-β 2 GPI-DI) antibodies in patients with antiphospholipid syndrome (APS) and their role when assessing thrombosis risk is uncertain. Objectives To estimate the prevalence of anti-β 2 GPI-DI in patients with APS and to determine whether anti-β 2 GPI-DI-positive individuals are at greater risk of thrombosis, as compared with individuals without anti-β 2 GPI-DI, by systematically reviewing the literature. Methods A detailed literature search was applied a priori to Ovid MEDLINE In-Process and Other Non-Indexed Citation 1986 to present and to abstracts from the European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR)/Association for Rheumatology Health Professionals (ARHP) Annual Meetings (2011-2015). Results A total of 11 studies, including 1,585 patients, were analyzed. Patients were distributed as follow: 1,218 patients APS (45.4% anti-β 2 GPI-DI-positive; in more detail: 504 primary APS [55.4% anti-β 2 GPI-DI-positive], 192 secondary APS [43.2% anti-β 2 GPI-DI-positive], and 522 not specified), 318 with systemic lupus erythematosus (SLE; 26.7% anti-β 2 GPI-DI-positive), 49 asymptomatic carriers of antiphospholipid antibodies (aPL) (30.6% anti-β 2 GPI-DI-positive), and 1,859 healthy controls. When considering the five studies eligible for thrombotic risk assessment, four studies found a significant association of anti-β 2 GPI-DI-positivity with thrombotic events, whereas one study found no predictive correlation with thrombosis (overall odds ratio [OR] for pooled data: 1.99; 95% confidence interval [CI]: 1.52-2.6; p < 0.0001). Conclusion We report an overall estimated median prevalence of anti-β 2 GPI-DI antibodies of 44.3% in patients with APS and/or SLE and a significantly higher prevalence among patients with APS compared with SLE alone. Anti-β 2 GPI-DI antibodies might represent a promising tool when assessing thrombotic risk in patients with APS.

Original languageEnglish
Pages (from-to)466-474
Number of pages9
JournalSeminars in Thrombosis and Hemostasis
Issue number5
Publication statusPublished - Jul 1 2018


  • antiphospholipid antibodies
  • antiphospholipid syndrome
  • non-criteria aPL
  • thrombosis
  • β glycoprotein I domain

ASJC Scopus subject areas

  • Hematology
  • Cardiology and Cardiovascular Medicine


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