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. 2021 Jun 14;22(12):6360. doi: 10.3390/ijms22126360

Table 3.

New Eurofever/PRINTO Classification Criteria for CAPS.

Presence of pathogenic NLRP3 mutations and at least one among the following:
▪ Urticarial rash
▪ Red eye (conjunctivitis, uveitis, episcleritis)
▪ Neurosensorial hearing loss
OR Presence of VUS of NLRP3 and at least two among the following:
▪ Urticarial rash
▪ Red eye (conjunctivitis, uveitis, episcleritis)
▪ Neurosensorial hearing loss