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

Abstract

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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Affiliated Institutions

• IRCCS Istituto Auxologico Italiano IT
• Immanuel Krankenhaus DE
• Cliniques Universitaires Saint-Luc BE
• Sheba Medical Center IL
• Utrecht University NL
• UCLouvain BE
• University Hospital Carl Gustav Carus DE
• Sorbonne Université FR
• Hippocration General Hospital GR
• Consorci Institut D'Investigacions Biomediques August Pi I Sunyer ES
• University of Debrecen HU
• Hôpital Claude Huriez FR
• Istituti di Ricovero e Cura a Carattere Scientifico IT
• Pitié-Salpêtrière Hospital FR
• Hvidovre Hospital DK
• University of Pisa IT
• St Thomas' Hospital GB
• Technische Universität Dresden DE
• Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia IT
• Medical University of Sofia BG
• Hospital de Santo António PT
• Centre Hospitalier Universitaire de Nîmes FR

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