title: Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients.
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.
  
  
  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.
authors.affiliation: 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
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Josep
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authors.forename: Munther A
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authors.lastname: Khamashta
authors.forename: Yehuda
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authors.lastname: Shoenfeld
authors.forename: María Teresa
authors.initials: MT
authors.lastname: Camps
authors.forename: Soren
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authors.lastname: Jacobsen
authors.forename: Gabriella
authors.initials: G
authors.lastname: Lakos
authors.forename: Angela
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authors.lastname: Tincani
authors.forename: Irene
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authors.lastname: Kontopoulou-Griva
authors.forename: Mauro
authors.initials: M
authors.lastname: Galeazzi
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Meroni
authors.forename: Ronald H W M
authors.initials: RH
authors.lastname: Derksen
authors.forename: Philip G
authors.initials: PG
authors.lastname: de Groot
authors.forename: Erika
authors.initials: E
authors.lastname: Gromnica-Ihle
authors.forename: Marta
authors.initials: M
authors.lastname: Baleva
authors.forename: Marta
authors.initials: M
authors.lastname: Mosca
authors.forename: Stefano
authors.initials: S
authors.lastname: Bombardieri
authors.forename: Frédéric
authors.initials: F
authors.lastname: Houssiau
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Gris
authors.forename: Isabelle
authors.initials: I
authors.lastname: Quéré
authors.forename: Eric
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authors.lastname: Hachulla
authors.forename: Carlos
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authors.lastname: Vasconcelos
authors.forename: Beate
authors.initials: B
authors.lastname: Roch
authors.forename: Antonio
authors.initials: A
authors.lastname: Fernández-Nebro
authors.forename: Marie-Claire
authors.initials: MC
authors.lastname: Boffa
authors.forename: Graham R V
authors.initials: GR
authors.lastname: Hughes
authors.forename: Miguel
authors.initials: M
authors.lastname: Ingelmo
mesh_terms: 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
chemical_list: D001323:Autoantibodies