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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