Anti-Phospholipid Syndrome

Antiphospholipid Syndrome and the Lungs

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterised by recurrent thromboembolic events (arterial or venous) and/or…
emjreviews.com

Background The serological test included in the classification for antiphospholipid syndrome are: IgG, IgM antibodies to cardiolipin (aCL), β2-glycoprotein…
ard.bmj.com

Lupus Anticoagulant is the strongest predictor of recurrent clotting in APS.

Anticoagulation in APS is lifelong. Do not stop just because the anti-phospholipid antibodies turn negative on follow-up.


The NOACs (Non-vitamin K Oral Anticoagulants) include DabIgatran (binds thrombin) and Rivaroxaban/Apixaban/Edoxaban (bind factor Xa).
They appear effective and safe to replace warfarin in APS with only venous thrombosis, but not arterial thrombosis.
But lack of reversal agents for some complicates the picture
medpagetoday.com

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

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 (SH…
ard.bmj.com
Level of evidence Strength Agreement (%)
Diagnostic recommendations
1. The adult criteria for APS, while specific, may lack sensitivity for paediatric APS. 3 C 100
2. New classification criteria for paediatric APS are needed that would incorporate non-thrombotic manifestations in children, in addition to thrombosis. 4 D 100
3. The following tests should be performed when suspecting paediatric APS: lupus anticoagulant, anticardiolipin IgG and IgM and anti-β2-glycoprotein-I IgG and IgM. 2A/B B 100
4. aPL screening should be performed in all patients with cSLE. 3 C 100
Treatment recommendations
1. In patients with cSLE and aPL, antiplatelet agents could be considered for primary prevention of thrombosis in addition to hydroxychloroquine. 3 C 100
2. When a patient has suffered a venous thrombotic event, anticoagulation therapy is indicated when manifestations are related to aPL. 3 C 100
3. When a patient has suffered a venous thrombotic event associated with persistent aPL positivity, long-term anticoagulation therapy is indicated. 3 C 100
4. When a patient has suffered an arterial thrombotic event associated with persistent aPL positivity, adequate long-term anticoagulation therapy or combined anticoagulation and antiaggregation therapy is indicated. 3 C 100
5. When a patient has suffered a recurrent thrombotic event associated with persistent aPL positivity despite oral anticoagulation with a target INR 2.0–3.0, long-term anticoagulation therapy to a target INR 3.0–4.0 or alternative therapies such as extended therapeutic dose of low-molecular- weight heparin yielding a target anti-Xa is indicated. 3 C 100
Recommendations for children born to mothers with APS
1. Perinatal thrombosis associated with aPL is a very rare complication in infants born to mothers with positive aPL. Recurrence rates in infants with perinatal thrombosis are extremely low and there are no uniform guidelines for the therapeutic approach. In general, infants with perinatal arterial ischaemic stroke associated with aPL should not usually receive anticoagulation. 3 C 100
2. Children born to mothers with APS may exhibit a higher frequency of neurodevelopmental abnormalities; regular neurodevelopmental assessments during their long-term follow-up may be considered. 3 C 87
Recommendations for treatment of paediatric CAPS
1. In a patient with paediatric CAPS, immediate combination treatment with anticoagulants, corticosteroids, plasma exchange with or without intravenous immunoglobulins should be considered. 3 C 100
2. In a patient with paediatric CAPS, rituximab or other immunosuppressive therapy may also be considered as a treatment option. 3 C/D 100
3. In CAPS, there are too few data to support the routine use of antiaggregation therapy. 4 D 100

SPECT reveals perfusion problems in antiphospholipid syndrome

Image result for spect
MELBOURNE – Neuropsychiatric symptoms could be related to brain perfusion problems detected on SPECT imaging in patients who have multiple antiphospholipid antibodies and no history of…
mdedge.com