Long Term Outcome of Primary Antiphospholipid Syndrome Patients: A Multicenter Study

Francesca Dall'Ara¹, Mara Taraborelli², Rossella Reggia¹, Micaela Fredi², Maria Gerosa³, Laura Massaro⁴, Ariela Hoxha⁵, Marta Tonello⁵, Patrice Cacoub⁶, Nathalie Costedoat-Chalumeau⁷, Franco Franceschini², Pier Luigi Meroni⁸, Jean Charles Piette⁶, Amelia Ruffatti⁵, Guido Valesini⁴ and Angela Tincani¹, ¹Rheumatology and Clinical Immunology, Spedali Civili and University of Brescia, Brescia, Italy, ²Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia, Italy, ³Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan, Milano, Italy, ⁴Internal Medicine and Medical Specialties Department, Policlinico Umberto I, La Sapienza University of Rome, Roma, Italy, ⁵Rheumatology Unit, Department of Medicine DIMED, University of Padua, Padova, Italy, ⁶Internal Medicine Department, University Hospital “Pitié-Salpêtrière”, “Pierre et Marie Curie Paris VI” University, Paris, France, ⁷Internal Medicine Department, Cochin Hospital, “René-Descartes Paris V” University, Paris, France, ⁸Division of Rheumatology, Division of Rheumatology, Istituto Ortopedico Gaetano Pini, Milan, Italy

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: antiphospholipid syndrome

Session Information

Date: Tuesday, November 10, 2015

Title: Antiphospholipid Syndrome: Clinical

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Data on the long-term outcome in primary antiphospholipid syndrome (PAPS) patients (pt) are still very limited.The objectives of this work was to assess the prevalence of recurrences, organ damage, severe comorbidities (infections, hemorrhages, cancers), mortality, evolution in connective tissue disease (CTD), in long-standing PAPS.

Methods:

Medical records of PAPS pt 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 sixteen pt (88% females) with PAPS followed between 1983 and 2014 with mean age at diagnosis of 33 (±10) and mean follow-up of 19 years (±3.5) were studied. Fifty-one pt (44%) had at least a thrombotic event during follow-up. Thromboses were more frequent in pt with previous thrombotic history (p:0.002,OR:4.8, 95% CI:1.6-14.7) and oral anticoagulant (OA) treatment was not protective against recurrences (p: not significant). Six pt (5%) had a catastrophic event. Fifty-two women had 87 pregnancies, that were successful in 78% of cases. Twenty-nine percent of pt had functional damage (permanent loss of function) in at least one system. Damage was significantly associated to 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), but not to any demographical, serological or therapeutical variable. An anatomical damage (documented ischemic lesion) was present in 55% of pt. Twenty-four major bleeding episodes were recorded in 18 pt all on OA. Severe infections (4 bacterial, 2 viral) affected 6 pt (5%). A cancer (solid in 100%) was diagnosed in 8 pt (7%) at a mean age of 51 years (±6). One patient (1%) with a chronic bowel ischemia died for sepsis. Fourteen pt (12%) developed a CTD (7 Systemic Lupus Erythematosus,2 Sjogren, 5 Undifferentiated CTD).Compared to diagnosis at the end of the follow up we observed: less pt with anti-cardiolipin IgG (p:0.014) but more with antinuclear antibodies (p:0.01) and C4 reduction (p:0.025); less using estroprogestinics (p<0.001), more with hypercholesterolemia (p:0.043), hypertension (p:0.004), cancer (p:0.02); more using steroids (p:0.04), hydroxychloroquine (p<0.001), immunosuppressants (p<0.01), anticoagulants (p:0.003), anti-hypertensive drugs (p<0.001).

Conclusion:

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

Disclosure: F. Dall'Ara, None; M. Taraborelli, None; R. Reggia, None; M. Fredi, None; M. Gerosa, None; L. Massaro, None; A. Hoxha, None; M. Tonello, None; P. Cacoub, None; N. Costedoat-Chalumeau, None; F. Franceschini, None; P. L. Meroni, None; J. C. Piette, None; A. Ruffatti, None; G. Valesini, None; A. Tincani, None.

To cite this abstract in AMA style:

Dall'Ara F, Taraborelli M, Reggia R, Fredi M, Gerosa M, Massaro L, Hoxha A, Tonello M, Cacoub P, Costedoat-Chalumeau N, Franceschini F, Meroni PL, Piette JC, Ruffatti A, Valesini G, Tincani A. Long Term Outcome of Primary Antiphospholipid Syndrome Patients: A Multicenter Study [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/long-term-outcome-of-primary-antiphospholipid-syndrome-patients-a-multicenter-study/. Accessed .

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