Semin Thromb Hemost
DOI: 10.1055/s-0037-1603936
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

Massimo Radin1, Irene Cecchi1, Dario Roccatello1, Pier Luigi Meroni2, Savino Sciascia1
  • 1Center of Research of Immunopathology and Rare Diseases - Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, and SCDU Nephrology and Dialysis, University of Turin and S. Giovanni Bosco Hospital, Turin, Italy
  • 2Department of Clinical Sciences and Community Health, University of Milan, Laboratory of Immuno-Rheumatology Research, Istituto Auxologico Italiano, Milan, Italy
Further Information

Publication History

Publication Date:
04 August 2017 (eFirst)


Background To date, the exact prevalence of anti-β2 glycoprotein I domain I (anti-β2GPI-DI) antibodies in patients with antiphospholipid syndrome (APS) and their role when assessing thrombosis risk is uncertain.

Objectives To estimate the prevalence of anti-β2GPI-DI in patients with APS and to determine whether anti-β2GPI-DI-positive individuals are at greater risk of thrombosis, as compared with individuals without anti-β2GPI-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-β2GPI-DI-positive; in more detail: 504 primary APS [55.4% anti-β2GPI-DI-positive], 192 secondary APS [43.2% anti-β2GPI-DI-positive], and 522 not specified), 318 with systemic lupus erythematosus (SLE; 26.7% anti-β2GPI-DI-positive), 49 asymptomatic carriers of antiphospholipid antibodies (aPL) (30.6% anti-β2GPI-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-β2GPI-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-β2GPI-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-β2GPI-DI antibodies might represent a promising tool when assessing thrombotic risk in patients with APS.

Author Contributions

M.R. and I.C. searched the literature, assisted with the organization of the manuscript, interpreted and collected data, and wrote and edited the review. P.L.M., D.R., and S.S. interpreted and collected data, helped to design the figures and panel, and wrote and edited the review.