Cited by 974Systemic Lupus Erythematosus ResearchVasculitis and related conditionsBlood Coagulation and Thrombosis Mechanisms

Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients

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Cervera, R, Serrano, R, Pons-Estel, G J, Ceberio-Hualde, L, Shoenfeld, Y, de Ramón, E, Buonaiuto, V, Jacobsen, S +27 more·2015·Annals of the Rheumatic Diseases

Abstract

Objectives To assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later.Methods In 1999, we started an observational study of 1000 APS patients from 13 European countries. All had medical histories documented when entered into the study and were followed prospectively during the ensuing 10 years.Results 53.1% of the patients had primary APS, 36.2% had APS associated with systemic lupus erythematosus and 10.7% APS associated with other diseases. Thrombotic events appeared in 166 (16.6%) patients during the first 5-year period and in 115 (14.4%) during the second 5-year period. The most common events were strokes, transient ischaemic attacks, deep vein thromboses and pulmonary embolism. 127 (15.5%) women became pregnant (188 pregnancies) and 72.9% of pregnancies succeeded in having one or more live births. The most common obstetric complication was early pregnancy loss (16.5% of the pregnancies). Intrauterine growth restriction (26.3% of the total live births) and prematurity (48.2%) were the most frequent fetal morbidities. 93 (9.3%) patients died and the most frequent causes of death were severe thrombosis (36.5%) and infections (26.9%). Nine (0.9%) cases of catastrophic APS occurred and 5 (55.6%) of them died. The survival probability at 10 years was 90.7%.Conclusions Patients with APS still develop significant morbidity and mortality despite current treatment. It is imperative to increase the efforts in determining optimal prognostic markers and therapeutic measures to prevent these complications.

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Crossref · 10.1136/annrheumdis-2013-204838

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  • The occurrence of thrombosis in patients with LA is associated with an excessive increase in the risk of death, leading to impaired relative survival compared to an age- and sex-matched reference population.

    “Cervera et al. and our group have recently demonstrated that the occurrence of thrombosis in patients with LA is associated with an excessive increase in the risk of death, which in turn leads to a significantly impaired relative survival of these patients in comparison to an age- and sex-matched reference population [5, 6].”

    From Cardiovascular risk factors are major determinants of thrombotic risk in patients with the lupus anticoagulant (2017) · cites this paper as [6]

    The Mortality and causes of death section (page unavailable) reports that severe thromboses caused 36.5% of deaths and that the APS cohort had an unadjusted standardized mortality ratio of 1.8 versus the general population. It does not establish that thrombosis specifically increased mortality among patients with lupus anticoagulant (LA), or report relative survival against an age- and sex-matched reference population. The Discussion (page unavailable) says no clinical or immunological parameter was identified as prognostic for mortality. (LLM, conf=0.88)

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  • The 10-year cumulative TE incidence of 24.3% compares well to the thrombotic risk observed in the WAPS study and the Euro-Phospholipid Project registry, but was lower than the value in the Piedmont cohort study and higher than that in the study by Pengo et al.

    “The 10-year cumulative TE incidence of 24.3% compares well to the thrombotic risk observed in the WAPS study [31] and the very large Euro-Phospholipid Project registry [6], but was lower than the value in the recently published Piedmont cohort study [32] and higher than that in the recently published study by Pengo et al. [30].”
  • The relative frequencies of arterial and venous events as well as the proportion of patients receiving anticoagulation while developing an event were very similar to the results of the Euro-Phospholipid study.

    “Further, the relative frequencies of arterial and venous events as well as the proportion of patients receiving anticoagulation while developing an event were very similar to the results of the Euro-Phospholipid study [6].”

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Authors
Cervera, R., Serrano, R., Pons-Estel, G. J., Ceberio-Hualde, L., Shoenfeld, Y., de Ramón, E., Buonaiuto, V, Jacobsen, S., Zeher, M. M., Tarr, T., Tincani, A., Taglietti, M., Theodossiades, G., Nomikou, E., Galeazzi, M., Bellisai, F., Meroni, P. L., Derksen, R. H. W. M., de Groot, P. G. D., Baleva, M., Mosca, M., Bombardieri, S., Houssiau, F., Gris, J.-C., Quéré, I., Hachulla, E., Vasconcelos, C., Fernández-Nebro, A., Haro, M., Amoura, Z., Miyara, M., Tektonidou, M., Espinosa, G., Bertolaccini, M. L., & Khamashta, M. A.
Affiliations
  • Hospital Clínic de Barcelona (ES)
  • Consorci Institut D'Investigacions Biomediques August Pi I Sunyer (ES)
  • St Thomas' Hospital (GB)
  • Tel Aviv University (IL)
  • Copenhagen University Hospital (DK)
  • University of Debrecen (HU)
  • Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT)
  • Hippocration General Hospital (GR)
  • Azienda Ospedaliera Universitaria Senese (IT)
  • University of Milan (IT)
  • University Medical Center Utrecht (NL)
  • Medical University of Sofia (BG)
  • University of Pisa (IT)
  • Cliniques Universitaires Saint-Luc (BE)
  • Centre Hospitalier Universitaire de Nîmes (FR)
  • Hôpital Claude Huriez (FR)
  • Universidade do Porto (PT)
  • Hospital Regional Universitario de Málaga (ES)
  • Pitié-Salpêtrière Hospital (FR)
  • National and Kapodistrian University of Athens (GR)
Corresponding author
Cervera, R
Venue
Annals of the Rheumatic Diseases
Publisher
Elsevier BV
Published
2015-06
Volume
74
Issue
6
Pages
1011-1018
ISSN
0003-4967 (ISSN-L)
OpenAlex
W2120492526
Cited by
974
References
54
Citations by year
2026: 47 · 2025: 75 · 2024: 91 · 2023: 92 · 2022: 81 · 2021: 83 · 2020: 75 · 2019: 71 · 2018: 58 · 2017: 61 · 2016: 56 · 2015: 33 · 2014: 31 · 2013: 28 · 2012: 23
FWCI
49.79
Type
journal article
Language
en
Open access
Locations
  • Annals of the Rheumatic Diseases (publishedVersion)
  • PubMed (publishedVersion)
  • Research at the University of Copenhagen (University of Copenhagen) (publishedVersion, other-oa)
  • HAL (submittedVersion)
  • CINECA IRIS Institutial research information system (University of Pisa) (submittedVersion)
  • University of Debrecen Electronic Archive (University of Debrecen) (submittedVersion, other-oa)
  • Digital Access to Libraries (submittedVersion)
  • Dipòsit Digital de la Universitat de Barcelona (Universitat de Barcelona) (submittedVersion, public-domain)
  • Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
  • Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
  • Research Portal (King's College London) (submittedVersion)
  • Use Siena air (University of Siena) (submittedVersion)
  • Utrecht University Repository (Utrecht University) (submittedVersion)
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
  • Health Sciences › Medicine › Hematology › Blood Coagulation and Thrombosis Mechanisms
Keywords
antiphospholipid syndrome, thrombosis, stroke, deep vein thrombosis, pulmonary embolism, miscarriage, fetal growth restriction, preterm birth, catastrophic antiphospholipid syndrome, mortality, morbidity, systemic lupus erythematosus, survival
MeSH terms
Abortion, Spontaneous, Adolescent, Adult, Aged, Aged, 80 and over, Ischemic Attack, Transient, Child, Child, Preschool, Epilepsy, Female, Fetal Growth Retardation, Humans, Infant, Infant, Newborn, Infections, Longitudinal Studies, Lupus Erythematosus, Systemic, Male, Middle Aged, Pregnancy, Pregnancy Outcome, Prospective Studies, Pulmonary Embolism, Thrombocytopenia, Thrombosis, Cohort Studies, Antiphospholipid Syndrome, Venous Thrombosis, Stroke, Premature Birth, Livedo Reticularis, Young Adult

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