Chokri Ben Lamine: Early Recognition and Immediate Treatment of Catastrophic APS
Chokri Ben Lamine, Hematologist at King Faisal Specialist Hospital and Research Center, shared a post on X:
“Catastrophic antiphospholipid syndrome (CAPS)
Rare, fulminant and fatal – recognize early!
Suspect CAPS when:
- 3 or more organs involved
- Manifestations develop within 1 week or less
- Small-vessel thrombosis on histology
- Antiphospholipid antibodies detected
Common targets: kidney, lung, brain, heart, skin and GI tract
Thrombocytopenia, hemolysis and DIC-like findings may occur
- Frequent triggers
- Infection
- Surgery or trauma
- Pregnancy or postpartum
- Estrogen or anticoagulation withdrawal
- Malignancy or SLE flare
Do-not-miss mimics
- TTP or aHUS
- DIC or sepsis
- HIT
- HELLP syndrome
- Malignant hypertension
- Vasculitis
Treat immediately – do not await full criteria
- Therapeutic unfractionated heparin
- High-dose glucocorticoids
- Plasma exchange with or without IV immunoglobulin
- Identify and treat the trigger
- Early ICU with hematology or rheumatology involvement
Refractory CAPS: consider rituximab or complement inhibition such as eculizumab in expert centers; evidence remains limited.
Repeat antiphospholipid antibodies 12 or more weeks later, but never delay emergency treatment for confirmatory testing.
ASH Education Program 2024, EULAR recommendations, BSH APS guideline 2024.”
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