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
N1 - Publisher Copyright:
Copyright © 2022 Niznik, Rapoport, Avnery, Lubetsky, Haj Yahia, Ellis and Agmon-Levin.
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 - http://www.scopus.com/inward/record.url?scp=85129271398&partnerID=8YFLogxK
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 -
