Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients
Corresponding Author
Ricard Cervera
Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, and Institut d'Investigacions Biomè diques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain
Servei de Malalties Autoimmunes, Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, Villarroel 170, 08036- Barcelona, Catalonia, SpainSearch for more papers by this authorJean-Charles Piette
Hôpital La Pitié Salpêtrière, Paris, France
Search for more papers by this authorJosep Font
Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, and Institut d'Investigacions Biomè diques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain
Search for more papers by this authorMunther A. Khamashta
The Rayne Institute, St. Thomas' Hospital, London, UK
Search for more papers by this authorYehuda Shoenfeld
Chaim-Sheba Medical Center, Tel-Hashomer, Israel
Search for more papers by this authorMaría Teresa Camps
Hospital Regional “Carlos Haya,” Má laga, Spain
Search for more papers by this authorGabriella Lakos
University of Debrecen, Medical and Health Science Centre, Debrecen, Hungary
Search for more papers by this authorAngela Tincani
Spedali Civili, Azienda Ospedaliera, Brescia, Italy
Search for more papers by this authorPier Luigi Meroni
IRCCS Istituto Auxologico, Università di Milano, Milan, Italy
Search for more papers by this authorRonald H. W. M. Derksen
University Medical Center, Utrecht, The Netherlands
Search for more papers by this authorPhilip G. de Groot
University Medical Center, Utrecht, The Netherlands
Search for more papers by this authorFrédéric Houssiau
Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
Search for more papers by this authorJean-Christophe Gris
Centre Hospitalier Universitaire, Nîmes, France
Search for more papers by this authorBeate Roch
Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
Search for more papers by this authorAntonio Fernández-Nebro
Hospital Clínico Universitario, Málaga, Spain
Search for more papers by this authorGraham R. V. Hughes
The Rayne Institute, St. Thomas' Hospital, London, UK
Search for more papers by this authorMiguel Ingelmo
Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, and Institut d'Investigacions Biomè diques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain
Search for more papers by this authorCorresponding Author
Ricard Cervera
Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, and Institut d'Investigacions Biomè diques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain
Servei de Malalties Autoimmunes, Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, Villarroel 170, 08036- Barcelona, Catalonia, SpainSearch for more papers by this authorJean-Charles Piette
Hôpital La Pitié Salpêtrière, Paris, France
Search for more papers by this authorJosep Font
Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, and Institut d'Investigacions Biomè diques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain
Search for more papers by this authorMunther A. Khamashta
The Rayne Institute, St. Thomas' Hospital, London, UK
Search for more papers by this authorYehuda Shoenfeld
Chaim-Sheba Medical Center, Tel-Hashomer, Israel
Search for more papers by this authorMaría Teresa Camps
Hospital Regional “Carlos Haya,” Má laga, Spain
Search for more papers by this authorGabriella Lakos
University of Debrecen, Medical and Health Science Centre, Debrecen, Hungary
Search for more papers by this authorAngela Tincani
Spedali Civili, Azienda Ospedaliera, Brescia, Italy
Search for more papers by this authorPier Luigi Meroni
IRCCS Istituto Auxologico, Università di Milano, Milan, Italy
Search for more papers by this authorRonald H. W. M. Derksen
University Medical Center, Utrecht, The Netherlands
Search for more papers by this authorPhilip G. de Groot
University Medical Center, Utrecht, The Netherlands
Search for more papers by this authorFrédéric Houssiau
Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
Search for more papers by this authorJean-Christophe Gris
Centre Hospitalier Universitaire, Nîmes, France
Search for more papers by this authorBeate Roch
Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
Search for more papers by this authorAntonio Fernández-Nebro
Hospital Clínico Universitario, Málaga, Spain
Search for more papers by this authorGraham R. V. Hughes
The Rayne Institute, St. Thomas' Hospital, London, UK
Search for more papers by this authorMiguel Ingelmo
Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, and Institut d'Investigacions Biomè diques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain
Search for more papers by this authorAbstract
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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