Clinical characteristics and thrombosis outcomes of paediatric antiphospholipid syndrome: analysis of 58 patients

Abstract

The study aims to analyse the clinical and immunological manifestations of paediatric antiphospholipid syndrome (APS) in patients, based on the 2006 revised classification criteria of definite APS. Fifty-eight paediatric patients with APS were enrolled and analysed retrospectively. A total of 37 female and 21 male patients with a mean age of 14 ± 3 years at disease onset were included. Fourteen (24%) cases were primary APS, and 40 (69%) cases were secondary to systemic lupus erythaematosus (SLE). Anti-nuclear antibody (ANA) positivity and hypocomplementemia were more common in secondary APS than in primary APS. The most common manifestations of thrombosis were deep vein thrombosis of the lower extremities (25 cases, 37%). Non-thrombotic manifestations were mainly immunologic thrombocytopenia, autoimmune haemolytic anaemia, skin lesions, arthritis, pulmonary hypertension, heart valve vegetations and spontaneous abortion. LA, ACL and anti-β2GPI were positive in 42 (95%), 28 (64%) and 34 (77%) cases, respectively. Over half (23 cases, 52%) of the patients were triple-positive for antiphospholipid (aPL) antibodies. Among patients with single-positive LA and anti-β2GPI, the proportion with venous thrombosis was 100% (5 cases) and 0% (0 cases), respectively. The arterial thrombosis proportions were 22% (5 cases), 21% (3 cases) and 14% (1 case) in the triple-, double- and single-aPL-positive groups, respectively (P > 0.05). Fifty-three (91%) cases were followed up for 3 to 140 months, with a median time of 32 months. Seven (13%) cases had recurrences or appearances of thrombosis during follow-up, all of which were double- or triple-aPL positive. APS in the paediatric patients is mostly secondary to SLE. ANA positivity and hypocomplementemia are more common in secondary APS, but there are no differences in the other clinical manifestations between the primary and secondary APS groups. Deep vein thrombosis is the most common thrombotic event. Positive LA may increase the risk of venous thrombosis. Multiple-aPL positivity does not increase the proportion of thrombosis. Long-term anticoagulant or antiplatelet therapy is needed to prevent thrombosis recurrence in double- or triple-positive aPL cases.

Identification of high thrombotic risk triple‐positive antiphospholipid syndrome patients is dependent on anti‐cardiolipin and anti‐β2glycoprotein I antibody detection assays

“…Recent studies showed that the risk of thrombotic events increases with the number of positive tests in APS patients and the creation of antibody profiles and test combinations increases the association with thrombosis . On the other hand, another study showed a strong association between single aPL positivity and thrombosis in pediatric APS patients . Although single positivity is not always significantly correlated with thrombosis, within the current guidelines all aPL have the same diagnostic value .…”

Spectrum of juvenile antiphospholipid syndrome in two siblings: Case reports and review of literature

“…Our reported siblings are among the youngest to present with APS manifestations, at 13 years of age. To our knowledge, there are no cases of familial APS reported in the large pediatric APS cohorts [3,12 ].…”

The Truth Behind Hematuria

“…Our children have anticardiolipin antibody syndrome with right atrial thrombus, intracardiac thrombosis is a rare complication of APS, the physiological mechanism is not clear. The hypercoagulable state of nephrotic syndrome can easily lead to thrombosis, systemic lupus erythematosus with positive anticoagulant activity or medium or high level of anticardiolipin antibody, the risk of thrombosis or thromboembolism in the heart increases [11] . 37% of SLE patients with anticardiolipin antibody syndrome were positive for β-2-glycoprotein 1 antibody [12] .…”