Cited by 2,156Systemic Lupus Erythematosus ResearchVasculitis and related conditionsSystemic Sclerosis and Related Diseases

Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients

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Cervera, Ricard, Piette, Jean‐Charles, Font, Josep, Khamashta, Munther A., Shoenfeld, Yehuda, Camps, María Teresa, Jacobsen, Soren, Lakos, Gabriella +20 more·2002·Arthritis & Rheumatism

Abstract

OBJECTIVE: To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression. METHODS: The clinical and serologic features of APS (Sapporo preliminary criteria) in 1,000 patients from 13 European countries were analyzed using a computerized database. RESULTS: The cohort consisted of 820 female patients (82.0%) and 180 male patients (18.0%) with a mean +/- SD age of 42 +/- 14 years at study entry. "Primary" APS was present in 53.1% of the patients; APS was associated with systemic lupus erythematosus (SLE) in 36.2%, with lupus-like syndrome in 5.0%, and with other diseases in 5.9%. A variety of thrombotic manifestations affecting the majority of organs were recorded. A catastrophic APS occurred in 0.8% of the patients. Patients with APS associated with SLE had more episodes of arthritis and livedo reticularis, and more frequently exhibited thrombocytopenia and leukopenia. Female patients had a higher frequency of arthritis, livedo reticularis, and migraine. Male patients had a higher frequency of myocardial infarction, epilepsy, and arterial thrombosis in the lower legs and feet. In 28 patients (2.8%), disease onset occurred before age 15; these patients had more episodes of chorea and jugular vein thrombosis than the remaining patients. In 127 patients (12.7%), disease onset occurred after age 50; most of these patients were men. These patients had a higher frequency of stroke and angina pectoris, but a lower frequency of livedo reticularis, than the remaining patients. CONCLUSION: APS may affect any organ of the body and display a broad spectrum of manifestations. An association with SLE, the patient's sex, and the patient's age at disease onset can modify the disease expression and define specific subsets of APS.

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Crossref · 10.1002/art.10187

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Authors
Cervera, R., Piette, J., Font, J., Khamashta, M. A., Shoenfeld, Y., Camps, M. T., Jacobsen, S., Lakos, G., Tincani, A., Kontopoulou‐Griva, I., Galeazzi, M., 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., Quéré, I., Hachulla, E., Vasconcelos, C., Roch, B., Fernández‐Nebro, A., Boffa, M., Hughes, G. R. V., & Ingelmo, M.
Affiliations
  • Hospital Clínic de Barcelona (ES)
  • St Thomas' Hospital (GB)
  • Sheba Medical Center (IL)
  • Hospital Regional Universitario de Málaga (ES)
  • University of Debrecen (HU)
  • Hippocration General Hospital (GR)
  • Ospedale Santa Maria alle Scotte (IT)
  • University of Milan (IT)
  • University Medical Center Utrecht (NL)
  • Immanuel Krankenhaus (DE)
  • 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, Ricard
Venue
Arthritis & Rheumatism
Publisher
Wiley
Published
2002-04
Volume
46
Issue
4
Pages
1019-1027
ISSN
  • 0004-3591 (ISSN-L)
  • 1529-0131
OpenAlex
W2042958596
Cited by
2,156
References
42
Citations by year
2026: 60 · 2025: 82 · 2024: 110 · 2023: 93 · 2022: 106 · 2021: 130 · 2020: 92 · 2019: 110 · 2018: 99 · 2017: 95 · 2016: 84 · 2015: 94 · 2014: 96 · 2013: 82 · 2012: 85
FWCI
43.59
Type
journal article
Language
en
Open access
Locations
  • Arthritis & Rheumatism (publishedVersion)
  • PubMed (publishedVersion)
  • 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 (Université catholique de Louvain (UCL), l'Université de Namur (UNamur) and the Université Saint-Louis (USL-B)) (submittedVersion)
  • Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion)
  • Research Portal (King's College London) (submittedVersion)
  • Use Siena air (University of Siena) (submittedVersion)
Funders
  • Comissió Interdepartamental per a la Recerca i la Tecnologia of Catalonia, Spain ((FI/FIAP 1999/00684))
  • Fondo de Investigaciones Sanitarias of Spain ((FIS 1999/280))
  • IRCCS Istituto Auxologico Italiano of Italy (Ricerca Corrente 2000)
  • Institut Electricité-Santé of France
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
Keywords
antiphospholipid syndrome, systemic lupus erythematosus, thrombosis, catastrophic antiphospholipid syndrome, sex differences, age of onset, livedo reticularis, thrombocytopenia, leukopenia, stroke, myocardial infarction, arterial thrombosis, lupus-like syndrome, chorea, migraine, epilepsy, angina pectoris
MeSH terms
Adolescent, Adult, Aged, Aged, 80 and over, Autoantibodies, Child, Child, Preschool, Diagnosis, Differential, Female, Humans, Infant, Infant, Newborn, Male, Middle Aged, Cohort Studies, Prevalence, Antiphospholipid Syndrome, Age Distribution, Sex Distribution, Lupus Vasculitis, Central Nervous System

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