TY - JOUR
T1 - Longterm Outcome of Patients with Primary Antiphospholipid Syndrome
T2 - A Retrospective Multicenter Study
AU - Taraborelli, Mara
AU - Reggia, Rossella
AU - Dall'Ara, Francesca
AU - Fredi, Micaela
AU - Andreoli, Laura
AU - Gerosa, Maria
AU - Hoxha, Ariela
AU - Massaro, Laura
AU - Tonello, Marta
AU - Costedoat-Chalumeau, Nathalie
AU - Cacoub, Patrice
AU - Franceschini, Franco
AU - Meroni, Pier Luigi
AU - Piette, Jean Charles
AU - Ruffatti, Amelia
AU - Valesini, Guido
AU - Harris, Eon Nigel
AU - Tincani, Angela
PY - 2017/8
Y1 - 2017/8
N2 - OBJECTIVE: To assess the longterm frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities, and evolution toward connective tissue disease (CTD) in primary antiphospholipid syndrome (PAPS).METHODS: Medical records of patients with PAPS followed in 6 centers for ≥ 15 years were retrospectively reviewed.RESULTS: One hundred fifteen patients were studied: 88% women, followed between 1983 and 2014 with a mean (± SD) age at diagnosis of 33 (± 10) years. During a median followup of 18 years (range 15-30), 50 patients (44%) had at least a thrombotic event 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). A catastrophic antiphospholipid syndrome occurred in 6 patients (5%). The use of oral anticoagulants in patients with thrombotic onset did not appear to be protective against recurrences (p = 0.26). Fifty-two women had 87 pregnancies, successful in 78%. Twenty-nine percent of patients accrued functional damage. Damage was significantly associated with a thrombotic history (p = 0.004) and with arterial events (p < 0.001), especially stroke, but not with demographics, serology, or treatment. Twenty-four major bleeding episodes were recorded in 18 patients, all receiving anticoagulants. Severe infections affected 6 patients (5%), with 1 fatality. A solid cancer was diagnosed in 8 patients (7%). Altogether, 16 patients (14%) developed an autoimmune disease and 13 (11%) a full-blown picture of CTD.CONCLUSION: Despite therapy, a high proportion of patients experienced new thrombotic events and organ damage, while evolution toward CTD was infrequent.
AB - OBJECTIVE: To assess the longterm frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities, and evolution toward connective tissue disease (CTD) in primary antiphospholipid syndrome (PAPS).METHODS: Medical records of patients with PAPS followed in 6 centers for ≥ 15 years were retrospectively reviewed.RESULTS: One hundred fifteen patients were studied: 88% women, followed between 1983 and 2014 with a mean (± SD) age at diagnosis of 33 (± 10) years. During a median followup of 18 years (range 15-30), 50 patients (44%) had at least a thrombotic event 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). A catastrophic antiphospholipid syndrome occurred in 6 patients (5%). The use of oral anticoagulants in patients with thrombotic onset did not appear to be protective against recurrences (p = 0.26). Fifty-two women had 87 pregnancies, successful in 78%. Twenty-nine percent of patients accrued functional damage. Damage was significantly associated with a thrombotic history (p = 0.004) and with arterial events (p < 0.001), especially stroke, but not with demographics, serology, or treatment. Twenty-four major bleeding episodes were recorded in 18 patients, all receiving anticoagulants. Severe infections affected 6 patients (5%), with 1 fatality. A solid cancer was diagnosed in 8 patients (7%). Altogether, 16 patients (14%) developed an autoimmune disease and 13 (11%) a full-blown picture of CTD.CONCLUSION: Despite therapy, a high proportion of patients experienced new thrombotic events and organ damage, while evolution toward CTD was infrequent.
KW - Journal Article
U2 - 10.3899/jrheum.161364
DO - 10.3899/jrheum.161364
M3 - Article
C2 - 28572466
VL - 44
SP - 1165
EP - 1172
JO - Journal of Rheumatology
JF - Journal of Rheumatology
SN - 0315-162X
IS - 8
ER -