ICU · haematology-coagulation

Catastrophic Antiphospholipid Syndrome (CAPS) in the ICU

Also known as Catastrophic antiphospholipid syndrome · CAPS · Asherson syndrome · Antiphospholipid syndrome catastrophic · Multi-organ microthrombosis · APS storm

Catastrophic antiphospholipid syndrome (CAPS) — the most severe form of antiphospholipid syndrome (APS) characterised by rapid multi-organ thrombosis (small-vessel) affecting three or more organ systems within one week, histopathological evidence of microthrombosis, and antiphospholipid antibodies (2003 laboratory criterion: lupus anticoagulant and/or anticardiolipin; anti-beta-2-glycoprotein I is frequently detected in the registry and belongs to current APS serology). Affects: kidneys (AKI from renal microthrombi), lungs (ARDS, pulmonary embolism, pulmonary haemorrhage), brain (stroke, encephalopathy, seizures), heart (myocardial infarction, cardiomyopathy, valvular lesions), skin (livedo reticularis, digital gangrene), liver, GI, and adrenal haemorrhage or infarction. Triggers: infection (most common), surgery, malignancy, pregnancy, withdrawal of anticoagulation. Management: (1) eliminate trigger (treat infection), (2) triple therapy — anticoagulation (heparin) + corticosteroids + plasma exchange ± IVIG, (3) rituximab or eculizumab for refractory cases, (4) supportive care (RRT, ventilation, vasopressors). Mortality 37% of episodes in the 500-patient registry; later analyses 25-30%.

Checked against its sources on 5 Sept 2026