Patterns of Recurrent Thrombosis in Primary Antiphospholipid Syndrome—Multicenter, Real-Life Long-Term Follow-Up

Stanley Niznik; Micha J. Rapoport; Orly Avnery; Aharon Lubetsky; Soad Haj Yahia; Martin H. Ellis; Nancy Agmon-Levin

doi:10.3389/fimmu.2022.843718

Patterns of Recurrent Thrombosis in Primary Antiphospholipid Syndrome—Multicenter, Real-Life Long-Term Follow-Up

Stanley Niznik
, Micha J. Rapoport
, Orly Avnery
, Aharon Lubetsky
, Soad Haj Yahia
, Martin H. Ellis
, Nancy Agmon-Levin^*

^*Corresponding author for this work

Sheba Medical Center at Tel Hashomer
Assaf Harofeh Medical Center
Meir Hospital Sapir Medical Center
Inst. of Thrombosis and Hemostasis

Research output: Contribution to journal › Article › peer-review

22 Scopus citations

Abstract

Background: Antiphospholipid syndrome (APS) is an acquired hypercoagulable condition associated with antiphospholipid antibody (aPL) presence. Data on re-thrombosis following APS-diagnosis are limited. Methods: This is a retrospective analysis of new thrombotic events among primary APS (pAPS) patients followed for up to 15 years in three medical centers in Israel. Results: Among 312 primary-APS patients, 143 (46%) had new thrombotic event classified to three patterns: (1) Arterial—associated with heart valve disease (OR 7.24, 95% C.I. 2.26–24.6), hypertension (OR 3, 95% C.I. 1.44–6.25), elevated anti-B2-GPI IgM (OR 1.04, 95% C.I. 0.996–1.08), arterial thrombosis at presentation (OR 1.74 95% C.I. 0.992–3.26), and older age (41 vs. 34 years, p < 0.001). (2) Venous—linked with venous thrombosis at presentation (OR 12.9, 95% C.I. 5.27–31.6, p < 0.001), heart valve disease (OR 9.81 95% C.I. 1.82–52.9, p = 0.018), aGAPSS (OR 1.15 95% C.I. 1.02–1.29), and younger age (31 vs. 36.5 years, p = 0.001); and (3) Combined pattern—associated with heart valve disease (OR 40.5 95% C.I. 7.7–212) and pulmonary embolism (OR 7.47 95% C.I. 1.96–28.5). A 4th variant “the Breakthrough pattern” defined by re-thrombosis despite prophylactic therapy was observed in 100/143 (70%) patients and linked with heart valve disease (OR 8. 95% C.I. 2.43–26.3), venous thrombosis at presentation (OR 2.61 95% C.I. 1.47–4.66), leg ulcers (OR 12.2, 95% C.I. 1.4–107), hypertension (OR 1.99, 95% C.I. 0.92–4.34), and higher aGAPSS (OR 1.08, 95% C.I. 0.99–1.18). Conclusion: In this real-life observation, re-thrombosis was common among pAPS patients including in those recommended to receive prophylactic therapy. Different patterns of recurrence were identified and linked with presenting symptoms, specific serological markers, APS manifestations, and comorbidities. Studies that will address interventions to prevent recurrences of APS-related events are needed.

Original language	English
Article number	843718
Journal	Frontiers in Immunology
Volume	13
DOIs	https://doi.org/10.3389/fimmu.2022.843718
State	Published - 19 Apr 2022

Funding

Funders
Agency for Clinical Innovation
Mount Sinai Hospital Department of Medicine Research Fund
Pfizer
Gilead Sciences
AbbVie
Leona M. and Harry B. Helmsley Charitable Trust
Meso Scale Diagnostics
Takeda Pharmaceutical Company
Women and Children's Health Research Institute
Sandoz
Western Sydney Local Health District
Ferring Pharmaceuticals

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

Keywords

AGAPSS
antiphospholipid syndrome
cardiovascular disease
recurrence
therapy
thrombosis - immunology

Access to Document

10.3389/fimmu.2022.843718

Cite this

@article{4c264b7d09f5499195308415caca7331,

title = "Patterns of Recurrent Thrombosis in Primary Antiphospholipid Syndrome—Multicenter, Real-Life Long-Term Follow-Up",

abstract = "Background: Antiphospholipid syndrome (APS) is an acquired hypercoagulable condition associated with antiphospholipid antibody (aPL) presence. Data on re-thrombosis following APS-diagnosis are limited. Methods: This is a retrospective analysis of new thrombotic events among primary APS (pAPS) patients followed for up to 15 years in three medical centers in Israel. Results: Among 312 primary-APS patients, 143 (46\%) had new thrombotic event classified to three patterns: (1) Arterial—associated with heart valve disease (OR 7.24, 95\% C.I. 2.26–24.6), hypertension (OR 3, 95\% C.I. 1.44–6.25), elevated anti-B2-GPI IgM (OR 1.04, 95\% C.I. 0.996–1.08), arterial thrombosis at presentation (OR 1.74 95\% C.I. 0.992–3.26), and older age (41 vs. 34 years, p < 0.001). (2) Venous—linked with venous thrombosis at presentation (OR 12.9, 95\% C.I. 5.27–31.6, p < 0.001), heart valve disease (OR 9.81 95\% C.I. 1.82–52.9, p = 0.018), aGAPSS (OR 1.15 95\% C.I. 1.02–1.29), and younger age (31 vs. 36.5 years, p = 0.001); and (3) Combined pattern—associated with heart valve disease (OR 40.5 95\% C.I. 7.7–212) and pulmonary embolism (OR 7.47 95\% C.I. 1.96–28.5). A 4th variant “the Breakthrough pattern” defined by re-thrombosis despite prophylactic therapy was observed in 100/143 (70\%) patients and linked with heart valve disease (OR 8. 95\% C.I. 2.43–26.3), venous thrombosis at presentation (OR 2.61 95\% C.I. 1.47–4.66), leg ulcers (OR 12.2, 95\% C.I. 1.4–107), hypertension (OR 1.99, 95\% C.I. 0.92–4.34), and higher aGAPSS (OR 1.08, 95\% C.I. 0.99–1.18). Conclusion: In this real-life observation, re-thrombosis was common among pAPS patients including in those recommended to receive prophylactic therapy. Different patterns of recurrence were identified and linked with presenting symptoms, specific serological markers, APS manifestations, and comorbidities. Studies that will address interventions to prevent recurrences of APS-related events are needed.",

keywords = "AGAPSS, antiphospholipid syndrome, cardiovascular disease, recurrence, therapy, thrombosis - immunology",

author = "Stanley Niznik and Rapoport, \{Micha J.\} and Orly Avnery and Aharon Lubetsky and \{Haj Yahia\}, Soad and Ellis, \{Martin H.\} and Nancy Agmon-Levin",

note = "Publisher Copyright: Copyright {\textcopyright} 2022 Niznik, Rapoport, Avnery, Lubetsky, Haj Yahia, Ellis and Agmon-Levin.",

year = "2022",

month = apr,

day = "19",

doi = "10.3389/fimmu.2022.843718",

language = "אנגלית",

volume = "13",

journal = "Frontiers in Immunology",

issn = "1664-3224",

publisher = "Frontiers Media SA",

}

TY - JOUR

T1 - Patterns of Recurrent Thrombosis in Primary Antiphospholipid Syndrome—Multicenter, Real-Life Long-Term Follow-Up

AU - Niznik, Stanley

AU - Rapoport, Micha J.

AU - Avnery, Orly

AU - Lubetsky, Aharon

AU - Haj Yahia, Soad

AU - Ellis, Martin H.

AU - Agmon-Levin, Nancy

PY - 2022/4/19

Y1 - 2022/4/19

N2 - Background: Antiphospholipid syndrome (APS) is an acquired hypercoagulable condition associated with antiphospholipid antibody (aPL) presence. Data on re-thrombosis following APS-diagnosis are limited. Methods: This is a retrospective analysis of new thrombotic events among primary APS (pAPS) patients followed for up to 15 years in three medical centers in Israel. Results: Among 312 primary-APS patients, 143 (46%) had new thrombotic event classified to three patterns: (1) Arterial—associated with heart valve disease (OR 7.24, 95% C.I. 2.26–24.6), hypertension (OR 3, 95% C.I. 1.44–6.25), elevated anti-B2-GPI IgM (OR 1.04, 95% C.I. 0.996–1.08), arterial thrombosis at presentation (OR 1.74 95% C.I. 0.992–3.26), and older age (41 vs. 34 years, p < 0.001). (2) Venous—linked with venous thrombosis at presentation (OR 12.9, 95% C.I. 5.27–31.6, p < 0.001), heart valve disease (OR 9.81 95% C.I. 1.82–52.9, p = 0.018), aGAPSS (OR 1.15 95% C.I. 1.02–1.29), and younger age (31 vs. 36.5 years, p = 0.001); and (3) Combined pattern—associated with heart valve disease (OR 40.5 95% C.I. 7.7–212) and pulmonary embolism (OR 7.47 95% C.I. 1.96–28.5). A 4th variant “the Breakthrough pattern” defined by re-thrombosis despite prophylactic therapy was observed in 100/143 (70%) patients and linked with heart valve disease (OR 8. 95% C.I. 2.43–26.3), venous thrombosis at presentation (OR 2.61 95% C.I. 1.47–4.66), leg ulcers (OR 12.2, 95% C.I. 1.4–107), hypertension (OR 1.99, 95% C.I. 0.92–4.34), and higher aGAPSS (OR 1.08, 95% C.I. 0.99–1.18). Conclusion: In this real-life observation, re-thrombosis was common among pAPS patients including in those recommended to receive prophylactic therapy. Different patterns of recurrence were identified and linked with presenting symptoms, specific serological markers, APS manifestations, and comorbidities. Studies that will address interventions to prevent recurrences of APS-related events are needed.

AB - Background: Antiphospholipid syndrome (APS) is an acquired hypercoagulable condition associated with antiphospholipid antibody (aPL) presence. Data on re-thrombosis following APS-diagnosis are limited. Methods: This is a retrospective analysis of new thrombotic events among primary APS (pAPS) patients followed for up to 15 years in three medical centers in Israel. Results: Among 312 primary-APS patients, 143 (46%) had new thrombotic event classified to three patterns: (1) Arterial—associated with heart valve disease (OR 7.24, 95% C.I. 2.26–24.6), hypertension (OR 3, 95% C.I. 1.44–6.25), elevated anti-B2-GPI IgM (OR 1.04, 95% C.I. 0.996–1.08), arterial thrombosis at presentation (OR 1.74 95% C.I. 0.992–3.26), and older age (41 vs. 34 years, p < 0.001). (2) Venous—linked with venous thrombosis at presentation (OR 12.9, 95% C.I. 5.27–31.6, p < 0.001), heart valve disease (OR 9.81 95% C.I. 1.82–52.9, p = 0.018), aGAPSS (OR 1.15 95% C.I. 1.02–1.29), and younger age (31 vs. 36.5 years, p = 0.001); and (3) Combined pattern—associated with heart valve disease (OR 40.5 95% C.I. 7.7–212) and pulmonary embolism (OR 7.47 95% C.I. 1.96–28.5). A 4th variant “the Breakthrough pattern” defined by re-thrombosis despite prophylactic therapy was observed in 100/143 (70%) patients and linked with heart valve disease (OR 8. 95% C.I. 2.43–26.3), venous thrombosis at presentation (OR 2.61 95% C.I. 1.47–4.66), leg ulcers (OR 12.2, 95% C.I. 1.4–107), hypertension (OR 1.99, 95% C.I. 0.92–4.34), and higher aGAPSS (OR 1.08, 95% C.I. 0.99–1.18). Conclusion: In this real-life observation, re-thrombosis was common among pAPS patients including in those recommended to receive prophylactic therapy. Different patterns of recurrence were identified and linked with presenting symptoms, specific serological markers, APS manifestations, and comorbidities. Studies that will address interventions to prevent recurrences of APS-related events are needed.

KW - AGAPSS

KW - antiphospholipid syndrome

KW - cardiovascular disease

KW - recurrence

KW - therapy

KW - thrombosis - immunology

UR - https://www.scopus.com/pages/publications/85129271398

U2 - 10.3389/fimmu.2022.843718

DO - 10.3389/fimmu.2022.843718

M3 - ???researchoutput.researchoutputtypes.contributiontojournal.article???

C2 - 35514968

AN - SCOPUS:85129271398

SN - 1664-3224

VL - 13

JO - Frontiers in Immunology

JF - Frontiers in Immunology

M1 - 843718

ER -

Patterns of Recurrent Thrombosis in Primary Antiphospholipid Syndrome—Multicenter, Real-Life Long-Term Follow-Up

Abstract

Funding

UN SDGs

Keywords

Access to Document

Other files and links

Fingerprint

Cite this