TY - JOUR
T1 - Cluster analysis for the identification of clinical phenotypes among antiphospholipid antibody-positive patients from the APS ACTION Registry
AU - APS ACTION Investigators
AU - Zuily, Stéphane
AU - Clerc-Urmès, Isabelle
AU - Bauman, Cédric
AU - Andrade, Danieli
AU - Sciascia, Savino
AU - Pengo, Vittorio
AU - Tektonidou, Maria G.
AU - Ugarte, Amaia
AU - Gerosa, Maria
AU - Michael Belmont, H.
AU - Zamorano, Maria Angeles Aguirre
AU - Fortin, Paul
AU - Ji, Lanlan
AU - Efthymiou, Maria
AU - Cohen, Hannah
AU - Branch, D. Ware
AU - Jesus, Guilherme Ramires de
AU - Nalli, Cecilia
AU - Petri, Michelle
AU - Rodriguez, Esther
AU - Cervera, Ricard
AU - Knight, Jason S.
AU - Atsumi, Tatsuya
AU - Willis, Rohan
AU - Bertolaccini, Maria Laura
AU - Vega, Joann
AU - Wahl, Denis
AU - Erkan, Doruk
AU - Pons-Estel, Guillermo
AU - Giannakopoulos, Bill
AU - Krilis, Steve
AU - de Jesus, Guilherme
AU - Levy, Roger
AU - Fortin, Paul F.
AU - Zhang, Zhouli
AU - Andreoli, Laura
AU - Tincani, Angela
AU - Chighizola, Cecilia B.
AU - Meroni, Pierluigi
AU - Banzato, Alessandra
AU - Davis, Stacy
AU - Amengual, Olga
AU - Uthman, Imad
AU - Limper, Maarten
AU - de Groot, Philip
AU - Irastorza, Guillermo Ruiz
AU - Rodríguez-Pintó, Ignasi
AU - Cuadrado, Maria Jose
AU - Lopez-Pedrera, Rosario
AU - Gonzalez, Emilio
N1 - Publisher Copyright:
© The Author(s) 2020.
PY - 2022/10
Y1 - 2022/10
N2 - Objective: This study aimed to use cluster analysis (CA) to identify different clinical phenotypes among antiphospholipid antibodies (aPL)-positive patients. Methods: The Alliance for Clinical Trials and International Networking (APS ACTION) Registry includes persistently positive aPL of any isotype based on the Sydney antiphospholipid syndrome (APS) classification criteria. We performed CA on the baseline characteristics collected retrospectively at the time of the registry entry of the first 500 patients included in the registry. A total of 30 clinical data points were included in the primary CA to cover the broad spectrum of aPL-positive patients. Results: A total of 497 patients from international centres were analysed, resulting in three main exclusive clusters: (a) female patients with no other autoimmune diseases but with venous thromboembolism (VTE) and triple-aPL positivity; (b) female patients with systemic lupus erythematosus, VTE, aPL nephropathy, thrombocytopaenia, haemolytic anaemia and a positive lupus anticoagulant test; and (c) older men with arterial thrombosis, heart valve disease, livedo, skin ulcers, neurological manifestations and cardiovascular disease (CVD) risk factors. Conclusions: Based on our hierarchical cluster analysis, we identified different clinical phenotypes of aPL-positive patients discriminated by aPL profile, lupus or CVD risk factors. Our results, while supporting the heterogeneity of aPL-positive patients, also provide a foundation to understand disease mechanisms, create new approaches for APS classification and ultimately develop new management approaches.
AB - Objective: This study aimed to use cluster analysis (CA) to identify different clinical phenotypes among antiphospholipid antibodies (aPL)-positive patients. Methods: The Alliance for Clinical Trials and International Networking (APS ACTION) Registry includes persistently positive aPL of any isotype based on the Sydney antiphospholipid syndrome (APS) classification criteria. We performed CA on the baseline characteristics collected retrospectively at the time of the registry entry of the first 500 patients included in the registry. A total of 30 clinical data points were included in the primary CA to cover the broad spectrum of aPL-positive patients. Results: A total of 497 patients from international centres were analysed, resulting in three main exclusive clusters: (a) female patients with no other autoimmune diseases but with venous thromboembolism (VTE) and triple-aPL positivity; (b) female patients with systemic lupus erythematosus, VTE, aPL nephropathy, thrombocytopaenia, haemolytic anaemia and a positive lupus anticoagulant test; and (c) older men with arterial thrombosis, heart valve disease, livedo, skin ulcers, neurological manifestations and cardiovascular disease (CVD) risk factors. Conclusions: Based on our hierarchical cluster analysis, we identified different clinical phenotypes of aPL-positive patients discriminated by aPL profile, lupus or CVD risk factors. Our results, while supporting the heterogeneity of aPL-positive patients, also provide a foundation to understand disease mechanisms, create new approaches for APS classification and ultimately develop new management approaches.
KW - APS ACTION
KW - Antiphospholipid syndrome
KW - cardiovascular risk factors
KW - systemic lupus erythematosus
KW - triple positivity
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U2 - 10.1177/0961203320940776
DO - 10.1177/0961203320940776
M3 - Article
C2 - 32703117
AN - SCOPUS:85088425386
SN - 0961-2033
VL - 29
SP - 1353
EP - 1363
JO - Lupus
JF - Lupus
IS - 11
ER -