Cited by 632Systemic Lupus Erythematosus ResearchBlood Coagulation and Thrombosis MechanismsVenous Thromboembolism Diagnosis and Management

The DOI matches its Crossref record; 41 of the 41 references it cites were found in a registry or an index; 3 of the 23 sources its claims cite were checked for support.

Clinical course of high‐risk patients diagnosed with antiphospholipid syndrome

Open access
PENGO, V., RUFFATTI, A., LEGNANI, C., GRESELE, P., BARCELLONA, D., ERBA, N., TESTA, S., MARONGIU, F., BISON, E., DENAS, G., BANZATO, A., PADAYATTIL JOSE, S., ILICETO, S.·2010·Journal of Thrombosis and Haemostasis

Abstract

Background The characteristics and the clinical course of antiphospholipid syndrome (APS) in high-risk patients that are positive for all three recommended tests that detect the presence of antiphospholipid (aPL) antibodies have not been described. Methods This retrospective analysis of prospectively collected data examined patients referred to Italian Thrombosis Centers that were diagnosed with definite APS and tested positive for aPL [lupus anticoagulant (LA), anti-cardiolipin (aCL), and anti-beta2-glycoprotein I (beta2GPI) antibodies]. Laboratory data were confirmed in a central reference laboratory. Results One hundred and sixty patients were enrolled in this cohort study. The qualifying events at diagnosis were venous thromboembolism (76 cases; 47.5%), arterial thromboembolism (69 cases; 43.1%) and pregnancy morbidity (11 cases; 9.7%). The remaining four patients (2.5%) suffered from catastrophic APS. The cumulative incidence of thromboembolic events in the follow-up period was 12.2% (95% CI, 9.6-14.8) after 1 year, 26.1% (95% CI, 22.3-29.9) after 5 years and 44.2% (95% CI, 38.6-49.8) after 10 years. This was significantly higher in those patients not taking oral anticoagulants as compared with those on treatment (HR=2.4 95% CI 1.3-4.1; P<0.003). Major bleeding associated with oral anticoagulant therapy was low (0.8% patient/years). Ten patients died (seven were cardiovascular deaths). Conclusions Patients with APS and triple positivity for aPL are at high risk of developing future thromboembolic events. Recurrence remains frequent despite the use of oral anticoagulants, which significantly reduces the risk of thromboembolism.

Overview

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Written from the paper’s full text.

TL;DR

In 160 patients with definite antiphospholipid syndrome (APS) and triple positivity for lupus anticoagulant, anti-cardiolipin, and anti-β2-glycoprotein I antibodies, thromboembolic recurrence was frequent, with a cumulative incidence of 44.2% at 10 years. Oral anticoagulant treatment was associated with a significantly lower risk of recurrent thromboembolism, while major bleeding on anticoagulation was low.

Key findings

  • During follow-up, 55 of 160 patients (34.4%) had a thromboembolic event (25 venous, 30 arterial); cumulative incidence was 12.2% (95% CI 9.6–14.8) at 1 year, 26.1% (95% CI 22.3–29.9) at 5 years, and 44.2% (95% CI 38.6–49.8) at 10 years.
  • Oral anticoagulant status was the only predictor of thromboembolic events in multivariate Cox analysis: events occurred in 36/123 anticoagulated patients versus 19/37 non-anticoagulated patients, with HR 2.4 (95% CI 1.3–4.1; P < 0.003) for not taking oral anticoagulants; cumulative incidence was significantly higher in untreated patients (P = 0.002).
  • Major bleeding was low: 8 major bleeding events, 7 in patients on oral anticoagulants (0.8% patient/years); there were 10 deaths (0.8% patients/years; 7 cardiovascular, 3 non-cardiovascular).
  • Recurrence did not depend on the qualifying event: 10-year cumulative incidence was 45% for venous, 47% for arterial, and 37% for pregnancy morbidity as the qualifying event (P = ns).

Methods

  • Retrospective analysis of prospectively collected data from Italian Thrombosis Centers, including 160 patients with definite APS and triple positivity for LA, aCL, and anti-β2GPI antibodies, confirmed in a central reference laboratory; inclusion required LA and anti-β2GPI positivity and aCL > 40 GPL and/or > 40 MPL units.
  • Observation period from earliest diagnosis (March 1989) to March 2009; outcomes included objectively diagnosed thromboembolic events, major bleeding, and death.
  • Kaplan-Meier survival analysis estimated cumulative incidence of thromboembolic events; log-rank tests compared groups; Cox regression identified predictors of the primary endpoint.

Limitations

  • The paper does not include a formal limitations section; evident limitations include the observational, non-randomized treatment comparison (123 patients received long-term oral anticoagulants versus 37 who did not), so treatment effects may be confounded.
  • The cohort was selected from Thrombosis Centers and restricted to triple-positive APS patients with aCL > 40 units, limiting generalizability to other APS laboratory profiles or lower-risk populations.
  • Follow-up attrition was substantial: only 23 patients (14.4%) completed 10 years; pregnancy morbidity was uncommon (11 cases), and the reported percentage differs between the abstract (9.7%) and Results/Table 1 (6.9%).

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Integrity signals

Registry

Crossref · 10.1111/j.1538-7836.2009.03674.x

Cites

41 of 41 references verified0 citation errors · 0 not checkable

Statistics

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Verification

Claims the paper makes

24 Own result · 11 No full text of the sources · 6 Own interpretation · 4 Own method · 3 Sources not found · 1 Supported so far · 1 Partly supported · 1 Couldn’t confirm · 1 Not checked yet · accuracy not yet measured

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  • The quote only partly states this claim. 3
  • It may state two separate claims as one. 17
  • Probably not a substantial claim. 6
24 of 24
  • The paper relies on this without support.

    No prior study has described the characteristics and clinical course of APS in high-risk patients positive for all three recommended aPL tests.

    “To our knowledge, no study has reported the characteristics and clinical course of a homogenous group of APS patients that are positive in all three laboratory diagnostic tests.”
    “The characteristics and the clinical course of antiphospholipid syndrome (APS) in high-risk patients that are positive for all three recommended tests that detect the presence of antiphospholipid (aPL) antibodies have not been described.”
  • The paper relies on this without support.

    This is the first large-scale study to examine the clinical course of a homogeneous cohort of APS patients with full positive laboratory profile.

    “To our knowledge, this is the first large-scale study looking at the clinical course of a homogenous cohort of APS patients with full positive laboratory profile.”
    “which to our knowledge is the first to examine patients that are positive for APS as determined by three separate tests”
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Metadata

Authors
PENGO, V., RUFFATTI, A., LEGNANI, C., GRESELE, P., BARCELLONA, D., ERBA, N., TESTA, S., MARONGIU, F., BISON, E., DENAS, G., BANZATO, A., PADAYATTIL JOSE, S., & ILICETO, S.
Affiliations
  • University of Padua (IT)
  • University of Bologna (IT)
  • University of Perugia (IT)
  • University of Cagliari (IT)
Corresponding author
PENGO, V.
Venue
Journal of Thrombosis and Haemostasis
Publisher
Elsevier BV
Published
2010-02
Volume
8
Issue
2
Pages
237-242
ISSN
1538-7836 (ISSN-L)
OpenAlex
W2119266650
Cited by
632
References
39
Citations by year
2026: 37 · 2025: 31 · 2024: 40 · 2023: 47 · 2022: 52 · 2021: 45 · 2020: 55 · 2019: 50 · 2018: 41 · 2017: 33 · 2016: 38 · 2015: 35 · 2014: 32 · 2013: 30 · 2012: 30
FWCI
12.84
Type
journal article
Language
en
Open access
Locations
  • Journal of Thrombosis and Haemostasis (publishedVersion)
  • PubMed (publishedVersion)
  • UNICA IRIS Institutional Research Information System (University of Cagliari) (submittedVersion)
  • Padua Research Archive (University of Padova) (submittedVersion)
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Hematology › Blood Coagulation and Thrombosis Mechanisms
  • Health Sciences › Medicine › Internal Medicine › Venous Thromboembolism Diagnosis and Management
Keywords
antiphospholipid syndrome, triple positivity, lupus anticoagulant, anticardiolipin antibodies, anti-beta2-glycoprotein I antibodies, venous thromboembolism, arterial thromboembolism, pregnancy morbidity, catastrophic antiphospholipid syndrome, oral anticoagulation, thromboembolic recurrence, major bleeding, cardiovascular mortality
MeSH terms
Administration, Oral, Adult, Aged, Anticoagulants, Catastrophic Illness, Female, Hemorrhage, Humans, Italy, Male, Middle Aged, Predictive Value of Tests, Pregnancy, Pregnancy Complications, Cardiovascular, Recurrence, Retrospective Studies, Risk Factors, Time Factors, Biomarkers, Incidence, Proportional Hazards Models, Lupus Coagulation Inhibitor, Antiphospholipid Syndrome, Treatment Outcome, Antibodies, Antiphospholipid, Antibodies, Anticardiolipin, Disease Progression, Risk Assessment, Kaplan-Meier Estimate, beta 2-Glycoprotein I, Venous Thromboembolism

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