(CAPS: acronym for Catastrophic AntiPhospholipid Syndrome, catastrophic antiphospholipid syndrome)

Also called "catastrophic antiphospholipid syndrome" because a microangiopathy is associated with the consequences of the antiphospholipid syndrome. Antiphospholipid antibodies are probably the result of the antigenic similarity between certain infectious agents and natural anticoagulants. The "catastrophic" variant is triggered by factors related to the host or changes in the environment. 

In children, this complication can occur:

-        de novo, in the context of infection (60 %) and cancer (17 %)

-        in the context of systemic lupus erythematosus (28 %)

-        in the perioperative period (5-10 %) especially if a change in antithrombotic treatment (vitamin K antagonists) justified by the antiphospholipid syndrome is associated with other contributing factors.

The average age is 11.5 ± 4.6 years, and there is a female predominance (70 %) in adults.

Clinical signs are those of a multiorganic microangiopathy: the most commonly affected organs are the kidneys (63 % including renal failure, proteinuria, hypertension), lungs (63 % including ARDS, alveolar haemorrhage), the heart (58 % including heart failure and cardiomyopathy), brain (47 % including encephalopathy, convulsions), bone marrow (pancytopenia, hemolysis), liver (41 %), peripheral vessels (52%, thrombosis), skin (11 % digital necrosis  and ulcers). 

Mortality is high: 26 %.

Treatment: anticoagulants, high dose corticosteroids; sometimes plasmapheresis or IV immunoglobulins , or cyclophosphamide or rituximab

Anesthetic implications: 

check hemostasis (risk of associated thrombocytopenia); according to affected organs ; avoid infection and hypothermia.

References : 

-          Batra Y, Rajeev S. 
Anesthetic implications of the catastrophic antiphospholipid syndrome. 
Pediatr Anesth 2006 ; 16 : 1090-3.

-         Berman H, Rodriguez-Pinto I, Cervera R, Gregory S, de Meis E et al. 
Pediatric cvatastrophic antiphospholipid syndrome : descriptive analysis of 45 patients from the « CAPS » registry ». 
Autoimmunity Reviews 2014 : 13 : 157-62.

-        Guntz J, Layios N, Damas P. 
Catastrophic antiphospholipid syndrome: case reports and review of the literature. 
Acta Anaesth Belg 2014; 65: 87-94.

Updated: November 2019