TY - JOUR
T1 - Antiphospholipid syndrome
T2 - Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients
AU - Cervera, Ricard
AU - Piette, Jean Charles
AU - Font, Josep
AU - Khamashta, Munther A.
AU - Shoenfeld, Yehuda
AU - Camps, María Teresa
AU - Jacobsen, Soren
AU - Lakos, Gabriella
AU - Tincani, Angela
AU - Kontopoulou-Griva, Irene
AU - Galeazzi, Mauro
AU - Meroni, Pier Luigi
AU - Derksen, Ronald H W M
AU - De Groot, Philip G.
AU - Gromnica-Ihle, Erika
AU - Baleva, Marta
AU - Mosca, Marta
AU - Bombardieri, Stefano
AU - Houssiau, Frédéric
AU - Gris, Jean Christophe
AU - Quéré, Isabelle
AU - Hachulla, Eric
AU - Vasconcelos, Carlos
AU - Roch, Beate
AU - Fernández-Nebro, Antonio
AU - Boffa, Marie Claire
AU - Hughes, Graham R V
AU - Ingelmo, Miguel
PY - 2002
Y1 - 2002
N2 - 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.
AB - 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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U2 - 10.1002/art.10187
DO - 10.1002/art.10187
M3 - Article
C2 - 11953980
AN - SCOPUS:0036228886
VL - 46
SP - 1019
EP - 1027
JO - Arthritis care and research : the official journal of the Arthritis Health Professions Association
JF - Arthritis care and research : the official journal of the Arthritis Health Professions Association
SN - 0893-7524
IS - 4
ER -