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Semin Arthritis Rheum. 2019 May 2. pii: S0049-0172(19)30090-3. doi: 10.1016/j.semarthrit.2019.04.009. [Epub ahead of print]

The adjusted global antiphospholipid syndrome score (aGAPSS) and the risk of recurrent thrombosis: Results from the APS ACTION cohort.

Author information

1
Center 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, S. Giovanni Bosco Hospital, Piazza del Donatore di Sangue 3, Turin 10124, Italy.
2
Center 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, S. Giovanni Bosco Hospital, Piazza del Donatore di Sangue 3, Turin 10124, Italy. Electronic address: savino.sciascia@unito.it.
3
Department of Medicine, Weill Cornell Medicine, New York, NY, United States.
4
University Hospital Padova, Padova, Italy.
5
First Department of Propaedeutic Internal Medicine, Joint Rheumatology Program, National and Kapodistrian University of Athens, Athens, Greece.
6
Autoimmune Diseases Research Unit, Department of Internal Medicine, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, UPV/EHU, Bizkaia, The Basque Country, Spain.
7
Laboratory of Immuno-Rheumatology Research, Istituto Auxologico Italiano, Milan, Italy.
8
Peking University First Hospital, Beijing, China.
9
NYU School of Medicine Langone Medical Center, New York, NY, United States.
10
University College London, London, United Kingdom.
11
State University of Rio de Janeiro, Rio de Janeiro, Brazil.
12
University of Utah and Intermountain Healthcare, Salt Lake City, UT, United States.
13
CHU de Quebec - Universite Laval, Quebec, Canada.
14
Department of Clinical and Experimental Science, University of Brescia, Brescia, Italy.
15
Johns Hopkins University School of Medicine, Baltimore, MD, United States.
16
Hospital Universitario 12 de Octubre, Madrid, Spain.
17
Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), Barcelona, Catalonia, Spain.
18
University of Michigan, Ann Arbor, MI, United States.
19
Hokkaido University Hospital, Sapporo, Japan.
20
Antiphospholipid Standardization Laboratory, University of Texas Medical Branch, Galveston, TX,United States.
21
Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC), Spain.
22
University of Sao Paulo, Sao Paulo, Brazil.
23
Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, United States.
24
Academic Department of Vascular Surgery, School of Cardiovascular Medicine & Sciences, King's College London, London, United Kingdom.

Abstract

OBJECTIVES:

To assess whether patients with antiphospholipid syndrome (APS) and history of recurrent thrombosis have higher levels of adjusted Global AntiphosPholipid Syndrome Score (aGAPSS) when compared to patients without recurrent thrombosis.

METHODS:

In this cross-sectional study of antiphospholipid antibody (aPL)-positive patients, we identified APS patients with a history of documented thrombosis from the AntiPhospholipid Syndrome Alliance For Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository ("Registry"). Data on aPL-related medical history and cardiovascular risk factors were retrospectively collected. The aGAPSS was calculated at Registry entry by adding the points corresponding to the risk factors: three for hyperlipidemia, one for arterial hypertension, five for positive anticardiolipin antibodies, four for positive anti-β2 glycoprotein-I antibodies and four for positive lupus anticoagulant test.

RESULTS:

The analysis included 379 APS patients who presented with arterial and/or venous thrombosis. Overall, significantly higher aGAPSS were seen in patients with recurrent thrombosis (arterial or venous) compared to those without recurrence (7.8 ± 3.3 vs. 6 ± 3.9, p<0.05). When analyzed based on the site of the recurrence, patients with recurrent arterial, but not venous, thrombosis had higher aGAPSS (8.1 ± SD 2.9 vs. 6 ± 3.9; p<0.05).

CONCLUSIONS:

Based on analysis of our international large-scale Registry of aPL-positive patients, the aGAPSS might help risk stratifying patients based on the likelihood of developing recurrent thrombosis in APS.

KEYWORDS:

Antiphospholipid antibodies; Antiphospholipid syndrome; Cardiovascular; GAPSS; Risk stratification; Thrombosis

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