Description

Cryopyrin-Associated Periodic Syndrome (CAPS) is one of the autoinflammatory periodic fevers. The Eurofever Project and PRINTO developed a score for identifying affected patients.


Patient selection: periodic fever

 

Parameters:

(1) urticarial rash

(2) neurosensorial hearing loss

(3) conjunctivitis

(4) exudative pharyngitis

(5) abdominal pain

 

Parameter

Finding

Points

urticarial rash

yes

25

 

no

0

neurosensorial hearing loss

yes

25

 

no

0

conjunctivitis

yes

10

 

no

0

exudative pharyngitis

yes

0

 

no

25

abdominal pain

yes

0

 

no

15

 

total score =

= SUM(points for all 5 parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 100

• A score >= 52 indicates a patient who may have CAPS.

 

Performance:

• The area under the ROC curve was 0.99.

• The sensitivity ranges from 96 to 100% and specificity ranges from 92 to 93% (training and validation series).


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