de Azevedo Lopes et al. Adv Rheumatol (2021) 61:64
https://doi.org/10.1186/s42358-021-00222-3
RESEARCH
Clinical and laboratory characteristics
of Brazilian versus non-Brazilian primary
antiphospholipid syndrome patients
in AntiPhospholipid Syndrome Alliance
for Clinical Trials and InternatiOnal Networking
(APS ACTION) clinical database and repository
Erivelton de Azevedo Lopes
1
, Gustavo Guimarães Moreira Balbi
1,2
, Maria G. Tektonidou
3
, Vittorio Pengo
4
,
Savino Sciascia
5
, Amaia Ugarte
6
, H. Michael Belmont
7
, Maria Gerosa
8
, Paul R. Fortin
9
, Chary Lopez-Pedrera
10
,
Lanlan Ji
11
, Hannah Cohen
12
, Guilherme Ramires de Jesús
13
, D. Ware Branch
14
, Cecilia Nalli
15
, Michelle Petri
16
,
Esther Rodriguez
17
, Nina Kello
18
, Roberto Ríos-Garcés
19
, Jason S. Knight
20
, Tatsuya Atsumi
21
, Rohan Willis
22
,
Maria Laura Bertolaccini
23
, Doruk Erkan
24
and Danieli Andrade
1*
on behalf of APS ACTION
Abstract
Background: Antiphospholipid syndrome (APS) is characterized by episodes of thrombosis, obstetric morbidity or
both, associated with persistently positive antiphospholipid antibodies (aPL). Studying the profile of a rare disease in
an admixed population is important as it can provide new insights for understanding an autoimmune disease. In this
sense of miscegenation, Brazil is characterized by one of the most heterogeneous populations in the world, which is
the result of five centuries of interethnic crosses of people from three continents. The objective of this study was to
compare the clinical and laboratory characteristics of Brazilian vs. non-Brazilian primary antiphospholipid syndrome
(PAPS) patients.
Methods: We classified PAPS patients into 2 groups: Brazilian PAPS patients (BPAPS) and PAPS patients from other
countries (non-BPAPS). They were compared regarding demographic characteristics, criteria and non-criteria APS
manifestations, antiphospholipid antibody (aPL) profile, and the adjusted Global Antiphospholipid Syndrome Score
(aGAPSS).
Results: We included 415 PAPS patients (88 [21%] BPAPS and 327 [79%] non-BPAPS). Brazilian patients were signifi-
cantly younger, more frequently female, sedentary, obese, non-white, and had a higher frequency of livedo (25%
vs. 10%, p < 0.001), cognitive dysfunction (21% vs. 8%, p = 0.001) and seizures (16% vs. 7%, p = 0.007), and a lower
frequency of thrombocytopenia (9% vs. 18%, p = 0.037). Additionally, they were more frequently positive for lupus
© The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which
permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the
original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or
other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line
to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory
regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this
licence, visit http://creativecommons.org/licenses/by/4.0/.
Open Access
Advances in Rheumatology
*Correspondence: danieli.andrade@hc.fm.usp.br
1
University of São Paulo, Av. Dr. Arnaldo 455, Third Floor, Room 3109, São
Paulo 01246903, Brazil
Full list of author information is available at the end of the article