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

Ricard Cervera, Jean Charles Piette, Josep Font, Munther A. Khamashta, Yehuda Shoenfeld, María Teresa Camps, Soren Jacobsen, Gabriella Lakos, Angela Tincani, Irene Kontopoulou-Griva, Mauro Galeazzi, Pier Luigi Meroni, Ronald H W M Derksen, Philip G. De Groot, Erika Gromnica-Ihle, Marta Baleva, Marta Mosca, Stefano Bombardieri, Frédéric Houssiau, Jean Christophe GrisIsabelle Quéré, Eric Hachulla, Carlos Vasconcelos, Beate Roch, Antonio Fernández-Nebro, Marie Claire Boffa, Graham R V Hughes, Miguel Ingelmo

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)1019-1027
Number of pages9
JournalArthritis and Rheumatism
Issue number4
Publication statusPublished - 2002

ASJC Scopus subject areas

  • Immunology
  • Rheumatology


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