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


CONCLUSIONS
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.
Cervera
Ricard
R
Institut Clínic d'Infeccions i Immunologia (ICII), Hospital Clínic, and Institut d'Investigacions Biomè diques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain. rcervera@clinic.ub.es
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Khamashta
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Shoenfeld
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MT
Jacobsen
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Lakos
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Tincani
Angela
A
Kontopoulou-Griva
Irene
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Galeazzi
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PL
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RH
de Groot
Philip G
PG
Gromnica-Ihle
Erika
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Baleva
Marta
M
Mosca
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Bombardieri
Stefano
S
Houssiau
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Gris
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JC
Quéré
Isabelle
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Hachulla
Eric
E
Vasconcelos
Carlos
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Beate
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Fernández-Nebro
Antonio
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Boffa
Marie-Claire
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Miguel
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D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D017678:Sex Distribution
D001323:Autoantibodies