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 Gris, Isabelle Quéré, Eric Hachulla, Carlos Vasconcelos, Beate Roch, Antonio Fernández-Nebro, Marie-Claire Boffa, Graham R V Hughes, Miguel Ingelmo, Euro-Phospholipid Project Group, 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 Gris, Isabelle Quéré, Eric Hachulla, Carlos Vasconcelos, Beate Roch, Antonio Fernández-Nebro, Marie-Claire Boffa, Graham R V Hughes, Miguel Ingelmo, Euro-Phospholipid Project Group
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.
Source: PubMed