Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients
Open accessAbstract
Objectives To identify the main causes of morbidity and mortality in patients with antiphospholipid syndrome (APS) during a 5-year period and to determine clinical and immunological parameters with prognostic significance.Methods The clinical and immunological features of a cohort of 1000 patients with APS from 13 European countries who had been followed up from 1999 to 2004 were analysed.Results 200 (20%) patients developed APS-related manifestations during the 5-year study period. Recurrent thrombotic events appeared in 166 (16.6%) patients and the most common were strokes (2.4% of the total cohort), transient ischaemic attacks (2.3%), deep vein thromboses (2.1%) and pulmonary embolism (2.1%). When the thrombotic events occurred, 90 patients were receiving oral anticoagulants and 49 were using aspirin. 31/420 (7.4%) patients receiving oral anticoagulants presented with haemorrhage. 3/121 (2.5%) women with only obstetric APS manifestations at the start of the study developed a new thrombotic event. A total of 77 women (9.4% of the female patients) had one or more pregnancies and 63 (81.8% of pregnant patients) had one or more live births. The most common fetal complications were early pregnancy loss (17.1% of pregnancies) and premature birth (35% of live births). 53 (5.3% of the total cohort) patients died. The most common causes of death were bacterial infection (21% of deaths), myocardial infarction (19%) and stroke (13%). No clinical or immunological predictor of thrombotic events, pregnancy morbidity or mortality was detected.Conclusion Patients with APS still develop significant morbidity and mortality despite current treatment (oral anticoagulants or antiaggregants, or both).
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Published evidence indicates a 20% recurrence rate of thrombosis in antiphospholipid syndrome patients on oral anticoagulants after five years.
“Published data support a 20% prevalence rate of thrombosis recurrence in APS patients treated with OAs after 5 years of follow-up [1].”
From Antithrombotic effects of hydroxychloroquine in primary antiphospholipid syndrome patients (2013) · cites this paper as [1]
The present cohort differed from the Cervera et al. cohort in several respects.
“This selected cohort of patients differed from the large cohort described by Cervera et al. [25,26] in several aspects.”
From Clinical course of high‐risk patients diagnosed with antiphospholipid syndrome (2010) · cites this paper as [26]
Fewer than half of Cervera et al.'s patients were LA positive.
“Less than half of the patients reported by Cervera et al. were LA positive.”
From Clinical course of high‐risk patients diagnosed with antiphospholipid syndrome (2010) · cites this paper as [26]
The Laboratory studies section says LA activity was tested but gives no positivity rate. The General characteristics section says the cohort’s immunological findings were reported elsewhere. Searches for LA prevalence and a percentage below 50% found no figure in our copy (page numbers unavailable). (LLM, conf=0.75)
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Cites
MIYAKIS, S., LOCKSHIN, M. D., ATSUMI, T., BRANCH, D. W., BREY, R. L., CERVERA, R., DERKSEN, R. H. W. M., DE GROOT, P. G., KOIKE, T., MERONI, P. L., et al. (2006) · Journal of Thrombosis and Haemostasis · doi:10.1111/j.1538-7836.2006.01753.x · Cited by 7,203 · see citing works
Self-citationCervera, R., Piette, J., Font, J., Khamashta, M. A., Shoenfeld, Y., Camps, M. T., Jacobsen, S., Lakos, G., Tincani, A., Kontopoulou‐Griva, I., et al. (2002) · Arthritis & Rheumatism · doi:10.1002/art.10187 · Cited by 2,156 · cited 6× · see citing works
“these patients when entered in the study have been previously reported.(2) Since then, this cohort of patients has been followed up by the same doctors during the ensuing 5 years.”
BackgroundMethodResultInfluentialThe management of thrombosis in the antiphospholipid-antibody syndrome
Khamashta, M. A., Cuadrado, M. J., Mujic, F., Taub, N. A., Hunt, B. J., & Hughes, G. R. V. (1995) · New England Journal of Medicine · doi:10.1056/nejm199504133321504 · Cited by 1,450 · see citing works
Self-citationBrandt, J. T., Triplett, D. A., Alving, B., & Scharrer, I. (1995) · Thrombosis and Haemostasis · doi:10.1055/s-0038-1649901 · Cited by 993 · see citing works
Cervera, R., Khamashta, M. A., Font, J., Sebastiani, G. D., GIL, A., Lavilla, P., Domenech, I., Aydintug, A., Jędryka-Góral, A., de Ramón, E., et al. (1993) · Medicine · doi:10.1097/00005792-199303000-00005 · Cited by 976 · see citing works
Self-citationAsherson, R. A., Cervera, R., de Groot, P. G., Erkan, D., Boffa, M., Pietté, J. C., Khamashta, M. A., Shoenfeld, Y., & Catastrophic Antiphospholipid Syndrome Registry Project Group (2003) · Lupus · doi:10.1191/0961203303lu394oa · Cited by 863 · see citing works
Self-citationCervera, R., Khamashta, M. A., Font, J., Sebastiani, G. D., Gil, A., Lavilla, P., Aydintug, A., Jędryka-Góral, A., de Ramón, E., Fernández‐Nebro, A., et al. (1999) · Medicine · doi:10.1097/00005792-199905000-00003 · Cited by 429 · see citing works
Self-citationEffect of antimalarials on thrombosis and survival in patients with systemic lupus erythematosus
Ruiz-Irastorza, G., Egurbide, M.-V., Pijoan, J.-I., Garmendia, M., Villar, I., Martinez-Berriotxoa, A., Erdozain, J.-G., & Aguirre, C. (2006) · Lupus · doi:10.1177/0961203306071872 · Cited by 411 · see citing works
Erkan, D., Harrison, M. J., Levy, R., Peterson, M., Petri, M., Sammaritano, L., Unalp‐Arida, A., Vilela, V., Yazici, Y., & Lockshin, M. D. (2007) · Arthritis & Rheumatism · doi:10.1002/art.22663 · Cited by 401 · see citing works
Catastrophic Antiphospholipid Syndrome
Asherson, R. A., Cervera, R., Pietté, J. C., Shoenfeld, Y., Espinosa, G., Petri, M., LIM, E., Lau, T., Gurjal, A., Jędryka-Góral, A., et al. (2001) · Medicine · doi:10.1097/00005792-200111000-00002 · Cited by 384 · see citing works
Self-citationBucciarelli, S., Espinosa, G., Cervera, R., Erkan, D., Gómez-Puerta, J. A., Ramos‐Casals, M., Font, J., & Asherson, R. A. (2006) · Arthritis & Rheumatism · doi:10.1002/art.22018 · Cited by 333 · see citing works
Self-citationDerksen, R. H. W. M., de Groot, P. G., Kater, L., & Nieuwenhuis, H. K. (1993) · Annals of the Rheumatic Diseases · doi:10.1136/ard.52.9.689 · Cited by 240 · see citing works
Self-citationInfectious origin of the antiphospholipid syndrome
Shoenfeld, Y., Blank, M., Cervera, R., Font, J., Raschi, E., & Meroni, P. L. (2005) · Annals of the Rheumatic Diseases · doi:10.1136/ard.2005.045443 · Cited by 206 · see citing works
Self-citationAllegri, F., Sanmarco, M., Cinquini, M., Taglietti, M., Balestrieri, G., Koike, T., Ichikawa, K., Meroni, P. L., Boffa, M. C., & Tincani, A. (2001) · Thrombosis and Haemostasis · doi:10.1055/s-0037-1616089 · Cited by 182 · see citing works
Self-citationBleeding and Recurrent Thrombosis in Definite Antiphospholipid Syndrome
Ruiz‐Irastorza, G., Khamashta, M. A., Hunt, B. J., Escudero‐Contreras, A., Cuadrado, M. J., & Hughes, G. R. V. (2002) · Archives of Internal Medicine · doi:10.1001/archinte.162.10.1164 · Cited by 176 · see citing works
Self-citationClinical study and follow-up of 100 patients withthe antiphospholipid syndrome
Muñoz-Rodríguez, F. J., Font, J., Cervera, R., Reverter, J. C., Tàssies, D., Espinosa, G., López-Soto, A., Carmona, F., Balasch, J., Ordinas, A., et al. (1999) · Seminars in Arthritis and Rheumatism · doi:10.1016/s0049-0172(99)80029-8 · Cited by 114 · see citing works
Tarr, T., Lakos, G., Bhattoa, H. P., Shoenfeld, Y., Szegedi, G., & Kiss, E. (2007) · Lupus · doi:10.1177/0961203306074767 · Cited by 88 · see citing works
Self-citationThe catastrophic antiphospholipid syndrome--Asherson's syndrome.
Pietté, J. C., Cervera, R., Levy, R. A., et al. (2003) · PubMed · Cited by 72 · see citing works
Self-citationTHE CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME
Asherson, R. A., & Cervera, R. (2004) · Elsevier eBooks · doi:10.1016/b978-012433901-9/50044-2 · Cited by 68 · see citing works
Self-citationDiagnosis of antiphospholipid syndrome
Bertolaccini, M. L., Khamashta, M. A., & Hughes, G. R. V. (2005) · Nature Clinical Practice Rheumatology · doi:10.1038/ncprheum0017 · Cited by 40 · see citing works
Self-citationKrause, I., Leibovici, L., Blank, M., & Shoenfeld, Y. (2007) · Lupus · doi:10.1177/0961203306075977 · Cited by 39 · see citing works
Self-citationWilson (1999) · doi:10.1002/1529-0131(199907)42:7
Flagged first, then by citations
Research works printed but not linked: 4
- American Rheumatism Association glossary committee. Signs and symptoms. Dictionary of the Rheumatic Diseases 1982;1:1-80.
- Brandt JT, Triplett DA, Alving B, Scharrer I. Criteria for the diagnosis of lupus anticoagulants: an update. Thromb Haemost 1995;74:1185-90.
- Derksen RHWM, de Groot PG, Kater L, Nieuwenhuis HK. Patients with antiphospholipid antibodies should receive long term anticoagulant treatment. Ann Rheum Dis 1993;52:689-92.
- Piette JC, Cervera R, Levy RA, Nasonov EL, Triplett DA, Shoenfeld Y. The catastrophic antiphospholipid syndrome -Ashersons syndrome. Ann Med Intern 2003;154:195-6.
Cited by
Sorted by citations
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., et al. (2015) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2013-204838 · Cited by 974 · see citing works
Self-citationDiagnosis and management of the antiphospholipid syndrome
Garcia, D., & Erkan, D. (2018) · New England Journal of Medicine · doi:10.1056/nejmra1705454 · Cited by 819 · see citing works
Clinical course of high‐risk patients diagnosed with antiphospholipid syndrome
PENGO, V., RUFFATTI, A., LEGNANI, C., GRESELE, P., BARCELLONA, D., ERBA, N., TESTA, S., MARONGIU, F., BISON, E., DENAS, G., et al. (2010) · Journal of Thrombosis and Haemostasis · doi:10.1111/j.1538-7836.2009.03674.x · Cited by 633 · see citing works
Ruiz-Irastorza, G., Cuadrado, M., Ruiz-Arruza, I., Brey, R., Crowther, M., Derksen, R., Erkan, D., Krilis, S., Machin, S., Pengo, V, et al. (2011) · Lupus · doi:10.1177/0961203310395803 · Cited by 506 · see citing works
Self-citationArnaud, L., Mathian, A., Ruffatti, A., Erkan, D., Tektonidou, M., Cervera, R., Forastiero, R., Pengo, V., Lambert, M., Martinez-Zamora, M. A., et al. (2014) · Autoimmunity Reviews · doi:10.1016/j.autrev.2013.10.014 · Cited by 215 · see citing works
Self-citationArnaud, L., Mathian, A., Devilliers, H., Ruffatti, A., Tektonidou, M., Forastiero, R., Pengo, V., Lambert, M., Lefevre, G., Martinez-Zamora, M. A., et al. (2015) · Autoimmunity Reviews · doi:10.1016/j.autrev.2014.10.019 · Cited by 146 · see citing works
Antithrombotic effects of hydroxychloroquine in primary antiphospholipid syndrome patients
Schmidt‐Tanguy, A., Voswinkel, J., Henrion, D., Subra, J. F., Loufrani, L., Rohmer, V., Ifrah, N., & Belizna, C. (2013) · Journal of Thrombosis and Haemostasis · doi:10.1111/jth.12363 · Cited by 132 · see citing works
Fischer-Betz, R., Specker, C., Brinks, R., & Schneider, M. (2012) · Lupus · doi:10.1177/0961203312451335 · Cited by 56 · see citing works
Vikerfors, A., Johansson, A.-B., Gustafsson, J. T., Jonsen, A., Leonard, D., Zickert, A., Nordmark, G., Sturfelt, G., Bengtsson, A., Ronnblom, L., et al. (2013) · Rheumatology · doi:10.1093/rheumatology/kes252 · Cited by 51 · see citing works
- Authors
- Cervera, R., Khamashta, M. A., Shoenfeld, Y., Camps, M. T., Jacobsen, S., Kiss, E., Zeher, M. M., Tincani, A., Kontopoulou-Griva, I., Galeazzi, M., Bellisai, F., Meroni, P. L., Derksen, R. H. W. M., de Groot, P. G., Gromnica-Ihle, E., Baleva, M., Mosca, M., Bombardieri, S., Houssiau, F., Gris, J.-C., Quéré, I., Hachulla, E., Vasconcelos, C., Roch, B., Fernández-Nebro, A., Piette, J.-C., Espinosa, G., Bucciarelli, S., Pisoni, C. N., Bertolaccini, M. L., Boffa, M.-C., & Hughes, G. R. V.
- Affiliations
- Hospital Clínic de Barcelona (ES)
- St Thomas' Hospital (GB)
- Sheba Medical Center (IL)
- Hospital Regional Universitario de Málaga (ES)
- Copenhagen University Hospital (DK)
- University of Debrecen (HU)
- Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT)
- University of Siena (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)
- Hospital de Santo António (PT)
- University Hospital Carl Gustav Carus (DE)
- Pitié-Salpêtrière Hospital (FR)
- Corresponding author
- Cervera, R
- ORCID
- Venue
- Annals of the Rheumatic Diseases
- Publisher
- Elsevier BV
- Published
- 2009-09
- Volume
- 68
- Issue
- 9
- Pages
- 1428-1432
- ISSN
- 0003-4967 (ISSN-L)
- OpenAlex
- W2616490803
- Cited by
- 286
- References
- 22
- Citations by year
- 2026: 7 · 2025: 7 · 2024: 14 · 2023: 10 · 2022: 13 · 2021: 10 · 2020: 10 · 2019: 10 · 2018: 24 · 2017: 14 · 2016: 20 · 2015: 10 · 2014: 22 · 2013: 28 · 2012: 28
- FWCI
- 13.63
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Annals of the Rheumatic Diseases (publishedVersion)
- PubMed (publishedVersion)
- University of Debrecen Electronic Archive (University of Debrecen) (submittedVersion, other-oa)
- DIAL (Catholic University of Leuven) (submittedVersion)
- Research Portal (King's College London) (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Research at the University of Copenhagen (University of Copenhagen) (submittedVersion)
- Use Siena air (University of Siena) (submittedVersion)
- Funders
- University of Texas Rio Grande Valley
- Istituto Auxologico Italiano
- 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, mortality, morbidity, stroke, deep vein thrombosis, pulmonary embolism, transient ischemic attack, oral anticoagulants, aspirin, hemorrhage, pregnancy complications, obstetric antiphospholipid syndrome, fetal death, preterm birth, bacterial infection, myocardial infarction, prognostic factors
- MeSH terms
- Adolescent, Adult, Aged, Aged, 80 and over, Anticoagulants, Child, Child, Preschool, Drug Utilization, Epidemiologic Methods, Europe, Female, Humans, Infant, Infant, Newborn, Male, Middle Aged, Prognosis, Thrombosis, Antiphospholipid Syndrome, Young Adult
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