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The Impact of Systemic Lupus Erythematosus on the Clinical Phenotype of Antiphospholipid Antibody Positive Patients: Results from AntiPhospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) Clinical Database and Repository

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Unlu O1, Erkan D2, Barbhaiya M2, Andrade D3, Nascimento I3, Rosa R3, Banzato A4, Pengo V4, Ugarte A5, Gerosa M6, Ji L7, Efthymiou M8, Branch DW9, de Jesus GR10, Tincani A11, Belmont HM12, Fortin PR13, Petri M14, Rodriguez E15, Pons-Estel GJ16, Knight JS17, Atsumi T18, Willis R19, Zuily S20, Tektonidou MG21; TheBehalf of APS ACTION. Arthritis Care Res (Hoboken). 2018 Apr 18. doi: 10.1002/acr.23584. [Epub ahead of print]


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1 Department of Medicine, Weill Cornell Medicine, New York, NY, USA.

2 Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, USA.

3 University of Sao Paulo, Sao Paulo, Brazil.

4 University Hospital Padova, Padova, Italy.

5 Hospital Universitario Cruces Bizkaia, Spain.

6 University of Milan, Milan, Italy.

7 Peking University First Hospital, Beijing, China.

8 University College London, London, UK.

9 University of Utah and Intermountain Healthcare, Salt Lake City, UT, USA.

10 State University of Rio de Janeiro, Rio de Janeiro, Brazil.

11 Department of Clinical and Experimental Science, University of Brescia, Brescia, Italy.

12 NYU School of Medicine Langone Medical Center, New York, NY, USA.

13 CHU de Quebec- Universite Laval, Quebec, Canada.

14 Johns Hopkins University, Baltimore, MD, USA.

15 Hospital Universitario, 12 de Octubre, Madrid, Spain.

16 Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain.

17 University of Michigan, Ann Arbor, MI, USA.

18 Hokkaido University Hospital, Sapporo, Japan.

19 Antiphospholipid Standardization Laboratory, University of Texas Medical Branch, Galveston, TX, USA.

20 Nancy University, Nancy, France.

21 First Department of Propaedeutic Internal Medicine, Joint Rheumatology Program, National and Kapodistrian University of Athens, Athens, Greece.


OBJECTIVE: Although systemic lupus erythematosus (SLE) is the most common autoimmune disease associated with antiphospholipid antibodies (aPL), limited data exist on the impact of SLE on the clinical phenotype of aPL-positive patients. The primary objective was to compare the clinical, laboratory, and treatment characteristics of aPL-positive patients with or without SLE.

METHODS: A secure web-based data capture system stores patient demographics, and aPL-related clinical and laboratory characteristics. Inclusion criteria include aPL positivity according to the Updated Sapporo Classification Criteria. Patients fulfilling the SLE Classification Criteria and those with no other autoimmune diseases were included in the analysis.

RESULTS: 672 aPL-positive patients were recruited from 24 international centers; 426 were without other autoimmune diseases and 197 with SLE. The aPL with SLE group had higher rates of thrombocytopenia, hemolytic anemia, low complements, and IgA anti-β2 glycoprotein-I antibodies (aβ₂GPI), whereas the aPL only group had higher rates of cognitive dysfunction and IgG aβ₂GPI. The frequency of arterial and venous thromboses (including recurrent) as well as the pregnancy morbidity were similar between the groups. The prevalence of cardiovascular disease risk factors at the registry entry did not differ between the two groups, except current smoking, which was more frequent in aPL with SLE group.

CONCLUSIONS: Although the frequencies of thrombosis and pregnancy morbidity are similar between aPL-positive patients with or without SLE, the diagnosis of SLE in persistently aPL-positive patients is associated with an increased frequency of thrombocytopenia, hemolytic anemia, low complements, and IgA aβ₂GPI positivity.