European evidence-based recommendations for diagnosis and treatment of paediatric antiphospholipid syndrome: the SHARE initiative

Abstract

Antiphospholipid syndrome (APS) is rare in children, and evidence-based guidelines are sparse. Consequently, management is mostly based on observational studies and physician's experience, and treatment regimens differ widely. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative was launched to develop diagnostic and management regimens for children and young adults with rheumatic diseases. Here, we developed evidence-based recommendations for diagnosis and treatment of paediatric APS. Evidence-based recommendations were developed using the European League Against Rheumatism standard operating procedure. Following a detailed systematic review of the literature, a committee of paediatric rheumatologists and representation of paediatric haematology with expertise in paediatric APS developed recommendations. The literature review yielded 1473 articles, of which 15 were valid and relevant. In total, four recommendations for diagnosis and eight for treatment of paediatric APS (including paediatric Catastrophic Antiphospholipid Syndrome) were accepted. Additionally, two recommendations for children born to mothers with APS were accepted. It was agreed that new classification criteria for paediatric APS are necessary, and APS in association with childhood-onset systemic lupus erythematosus should be identified by performing antiphospholipid antibody screening. Treatment recommendations included prevention of thrombotic events, and treatment recommendations for venous and/or arterial thrombotic events. Notably, due to the paucity of studies on paediatric APS, level of evidence and strength of the recommendations is relatively low. The SHARE initiative provides international, evidence-based recommendations for diagnosis and treatment for paediatric APS, facilitating improvement and uniformity of care.

Pediatric Motor Inflammatory Neuropathy: The Role of Antiphospholipid Antibodies

“…Furthermore, more recently, only few studies reported evidence of the beneficial use of IVIG to treat pediatric aPL-related chorea [9,10,[12][13][14][15]. Our clinical case confirmed the importance of the early use of IVIG treatment in CIDP children with aPL positivity.…”

European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative

“…1B, randomised controlled study; 2A, controlled study without randomisation; 2B, quasi-experimental study; and for treatment studies: 1A, meta-analysis of randomised controlled trial; B, based on level 2 or extrapolated from level 1; C, based on level 3 or extrapolated from level 1 or 2; D, based on level 4 or extrapolated from level 3 or 4 expert opinion 14. Agreement indicates % of experts agreeing on the recommendation during the final voting round of the consensus meeting; for diagnostic and observational studies: 1A, meta-analysis of cohort studies; L, level of evidence; S, strength of recommendation: A, based on level 1 evidence; 3, descriptive study; 4, expert opinion 16 17…”

European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides – the SHARE initiative

“…The European initiative SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) aims to optimize care for children and young adults with Paediatric Rheumatic Diseases (6)(7)(8)(9)(10)(11). For rare forms of childhood systemic vasculitis (PAN, ANCA-associated vasculitides (AAV), and TA), the objective was to design cross-cutting and disease-specific evidence-based recommendations for diagnosis and treatment.…”