Catasthrofic Antifosfolipid Syndrome (CAPS) 4.75/5 (4)

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CAPS

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 (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%

Antifosfolipid syndrome BINDVEVESSYKDOMMER.no


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