Disastrous antiphospholipid syndrome (CAPS) complicates Thrombotic microangiopathy in a child. Stroke. Prasad N, 2012. CC BY NC SA 3.0

Definition

Catastrophic APL Syndrome (CAPS) (Asherson's Syndrome) is a serious complication antiphospholipid syndrome (APLS). Many blood clots occur almost simultaneously and cause failure of multiple organs (multiorgan failure).

Criteria

1. Three or more organs involved (kidney: s-creatinine more than 50% increased (as evidence of kidney failure).
2. Development (fast) over less than a week
3. Histologically (tissue sample) carcass vasculitis with occlusion (blood clot) in at least one organ
4. Lupus anticoagulant or anti-cardiolipin Antibodies

Safe CAPS: all 4 points, 3 points: probable CAPS (reference: Ascherson R, 2003)

Diagnostic: suggestions here (in Danish) (reference: Aquiar CL 2013)

Treatment

• Intensive care unit
• Anticoagulation (Heparin and Marevan)
• Plasmapheresis (Plasma exchange)
• Dialysis
• Cyclophosphamide (Sendoxane)
• Immunoglobulins
• Frozen plasma
• Fibrinolysis
• Off label treatment (for special cases): eculizumab

Prognosis

Serious condition. Deaths reported at 48%

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