PB0859 - Identification of Phenotypes in Vascular Antiphospholipid Syndrome Using Cluster Analysis: A Multicenter Study of 600 Patients

Monday, June 24, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Gabrielle de Mello Santos

MD
Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
Rio de Janeiro, Rio de Janeiro, Brazil

Co-Author(s)

PD

Paula David

MD
Department of Medicine 'B' & The Zabludowicz Center of Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel and Sackler Faculty of Medicine, Tel-Aviv University, Israel
Tel Hashomer, Tel Aviv, Israel
JO

Juan Luis Ontiveros-Austria

MD
Department of Hematology and Oncology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico
Mexico City, Distrito Federal, Mexico
YP

Yonatan Shneor Patt

MD
Department of Medicine 'B' & The Zabludowicz Center of Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel and Sackler Faculty of Medicine, Tel-Aviv University, Israel
Tel Hashomer, Tel Aviv, Israel
SR

Sergio Rodriguez-Rodriguez

MD
Department of Hematology and Oncology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico
Mexico City, Distrito Federal, Mexico
EO

Erica Okazaki

MD
Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
São Paulo, Sao Paulo, Brazil
MC

Marina P. Collela

Hematologist
Hemocentro UNICAMP, University of Campinas, Campinas, SP, Brazil
Campinas, Sao Paulo, Brazil
BS

Bianca Stefanello

MD
Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
São Paulo, Sao Paulo, Brazil
GG

Gabriela G Yamaguti-Hayakawa

MD
Hematology and Hemotherapy Center, Faculty of Medical Sciences of the University of Campinas (UNICAMP), Campinas, Brazil
Campinas, Sao Paulo, Brazil
CR

Cynthia Rothschild

MD
Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
São Paulo, Sao Paulo, Brazil
JA

Joyce Annichino-Bizzachi

Supervisor of the Hemostasis laboratory
Centro de Hematologia e Hemoterapia - Universidade de Campinas (UNICAMP), Campinas, SP, Brasil
Campinas, Sao Paulo, Brazil
VR

Vanderson Rocha

MD
Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
São Paulo, Sao Paulo, Brazil
Erich V. De Paula, MD, PhD

Associate Professor
University of Campinas (UNICAMP)
Campinas, Sao Paulo, Brazil
OS

Ophira Salomon

MD
Thrombosis Unit, Sheba Medical Center, Tel Hashomer, Israel
Tel Hashomer, Tel Aviv, Israel
RD

Roberta Demichelis-Gomez

MD
Department of Hematology and Oncology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico
Mexico City, Distrito Federal, Mexico
AB

Ana Barrera-Vargas

MD
Department of Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico
Mexico City, Distrito Federal, Mexico
HA

Howard Amital

MD
Department of Medicine 'B' & The Zabludowicz Center of Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel and Sackler Faculty of Medicine, Tel-Aviv University, Israel
Tel Hashomer, Tel Aviv, Israel
PV

Paula Villaça

Hematologist
Hospital das Clínicas da Faculdade de Medicina da USP, São Paulo, São Paulo, Brazil
São Paulo, Sao Paulo, Brazil
YS

Yehuda Shoenfeld

MD
Department of Medicine 'B' & The Zabludowicz Center of Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel and Sackler Faculty of Medicine, Tel-Aviv University, Israel
Tel Hashomer, Tel Aviv, Israel
FO

Fernanda Andrade Orsi

MD
Department of Hematology, Hemotherapy and Cellular Therapy, Hospital das Clínicas of University of São Paulo Medical School (HCFMUSP), São Paulo, Brazil
São Paulo, Sao Paulo, Brazil

Background: Vascular primary antiphospholipid syndrome (PAPS) is a heterogeneous disorder with various phenotypes associated or not with underlying comorbidities.

Aims: This study aimed to identify homogeneous groups of vascular PAPS based on specific combinations of clinical features to improve disease classification.

Methods: This was a retrospective multicenter cohort study. Differences between groups were assessed by univariable and multivariable analysis. Hierarchical clustering was used to identify subgroups with distinct characteristics.

Results: The total cohort consisted of 619 patients from Brazil (n=296), Israel (n=300), and Mexico (n=23). Hypertension (OR 2.94, 95%CI 1.70-5.08) and BMI (OR 1.06, 95%CI 1.01-1.17) were independently associated with recurrent thrombosis. Hypertension was also associated with arterial thrombosis (OR 2.22, 95%CI 1.46-3.4), while lupus anticoagulant (OR 1.88, 95% CI 1.14-3.09), obesity (OR 1.67, 95% CI 1.1-2.52) and thrombocytopenia (OR 1.66, 95% CI 1-2.76) were associated with venous thrombosis. We identified three clusters. One "immunological" subgroup (n=146) characterized by young adults (median 30yo; IQR 23-45), triple-positive (100%), predominantly venous thrombosis (69%) with unprovoked episodes (71%), more prone to thrombocytopenia (32%) and lower levels of complement C3 and C4. The "pro-thrombotic" subgroup (n=351) is characterized by adults (median 39yo; IQR 26-51), 98.9% non-triple-positive, with venous events (61%), a high prevalence of unusual site thrombosis (22%), family history of thrombosis (25%) and higher FVIII levels. The "cardiovascular" subgroup (n=83) consists of older patients (median 52yo; IQR 41-63), mostly arterial events (54%) and multiple thrombosis (60%), and strongly associated with hypertension (78%), diabetes (100%) and dyslipidemia (91%).

Conclusion(s): Three distinct patterns of vascular PAPS have been identified: immunological, pro-thrombotic, and cardiovascular. These may represent different etiologies with varying interplay between immunology, coagulation, and risk factors contributing to thrombosis in APS.