CAPS

Q: All of the following are required for the criteria of "catastrophic antiphospholipid syndrome" (CAPS) EXCEPT? (select one) 

A) History of antiphospholipid syndrome (APS) and/or antiphospholipid antibodies (aPL) 

B) Two or more new organ thromboses within a month 

C) Biopsy confirmation of a microthrombus 

D) Exclusion of other causes of multiple organ thromboses or microthromboses

Answer: B

It is important to know that CAPS is a very rare phenomenon, occurring only in 0.8 percent of patients of APS. This is important as other causes may look alike CAPS such as Heparin-Induced Thrombocytopenia (HIT) may deceive a clinician and may lead to wrong management. There are four strict criteria to establish CAPS 

●History of APS and/or antiphospholipid antibodies (aPL) 

●Three or more new organ thromboses within a week 

●Biopsy confirmation of a microthrombus 

●Exclusion of other causes of multiple organ thromboses or microthromboses

If not all four criteria can be established, it can be label as "probable CAPS".

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References:

1. Asherson, RA, Cervera, R, de Groot, PG, et al. Lupus 2003; 12:530 

2. Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol. 2014 Jan;3(1):9-17. doi: 10.12860/jnp.2014.03. Epub 2014 Jan 1. PMID: 24644537; PMCID: PMC3956908. 

3. Carmi O, Berla M, Shoenfeld Y, Levy Y. Diagnosis and management of catastrophic antiphospholipid syndrome. Expert Rev Hematol. 2017 Apr;10(4):365-374. doi: 10.1080/17474086.2017.1300522. Epub 2017 Mar 13. PMID: 28277850.