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THU0325 Long-Term Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study
  1. F. Dall'ara1,
  2. M. Taraborelli2,
  3. R. Reggia1,
  4. M. Fredi2,
  5. L. Andreoli1,
  6. M. Gerosa3,
  7. A. Hoxha4,
  8. L. Massaro5,
  9. M. Tonello4,
  10. P. Cacoub6,
  11. N. Costedoat-Chalumeau7,
  12. F. Franceschini2,
  13. P.L. Meroni3,
  14. J.C. Piette6,
  15. A. Ruffatti4,
  16. G. Valesini5,
  17. E.N. Harris8,
  18. A. Tincani1
  1. 1Rheumatology and Clinical Immunology, Spedali Civili and University of Brescia
  2. 2Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia
  3. 3Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan, Milan, Italy, Milan
  4. 4Rheumatology Unit, Department of Medicine - DIMED, University of Padua, Padua, Italy, Padua
  5. 5Internal Medicine and Medical Specialties Department, Policlinico Umberto I, “La Sapienza” University of Rome, Rome, Italy, rome, Italy
  6. 6Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares, AP-HP, Hospital “Pitié-Salpêtrière”, “Pierre et Marie Curie Paris VI” University, Paris, France
  7. 7Internal Medicine Department, Cochin Hospital, “René-Descartes Paris V” University, Paris, France, Paris, France
  8. 8Vice Chancellor Emeritus, University of the West Indies, Kingston 7, Jamaica, West Indies, Jamaica


Background Data on the long-term outcome in primary antiphospholipid syndrome (PAPS) patients are still very limited.

Objectives To assess the long-term frequency of recurrences, organ damage, severe comorbidities, mortality and evolution in connective tissue disease (CTD) in PAPS patients.

Methods Medical records of PAPS patients followed in 6 centers for ≥15 years were retrospectively reviewed. Chi square for categorical and Student t test for continuous variables were used. P<0.05 was considered significant.

Results One hundred and fifteen patients (88% females) followed between 1983 and 2014 with a mean age at diagnosis of 33 (±10) years and mean follow-up of 19 (±3.5) years were studied. Fifty-one patients (44%) had at least a thrombotic event during follow-up for a total of 75 events and an annual incidence of 3.5%. Thromboses were more frequent in patients with previous thrombotic history (p:0.002,OR:4.8, 95%CI:1.6–14.7). There was a tendency towards anticoagulant treatment being not protective against recurrences (p:0.063). Six patients (5%) had a catastrophic event. Fifty-two women had 87 pregnancies, successful in 78% of cases. Twenty-nine percent of patients had functional damage. Damage was significantly associated with a thrombotic history (p:0.004,OR:13.9,95%CI:1.8–288.4) and to arterial events (p<0.001,OR:7.9,95%CI:2.7–24.3) especially stroke, but not to demographics, serology or treatment. Twenty-four major bleeding episodes were recorded in 18 patients, all on anticoagulants. Severe infections affected 6 patients (5%) with 1 fatality. A solid cancer was diagnosed in 8 patients (7%). One patient (1%) with a chronic bowel ischemia died for sepsis. Eleven percent of patients developed a CTD and 14% another autoimmune disease.

Conclusions Despite therapy, a high proportion of patients experienced new thrombotic events,while pregnancy outcome was significantly improved. Organ damage developed in a significant proportion of patients and was associated with arterial events. The risk of evolution in CTD has to be considered.

Disclosure of Interest None declared

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