Ozen et al reported criteria for refractory Familial Mediterranean Fever (FMF).
Patient selection: Familial Mediterranean Fever (FMF)
Criteria for FMF refractory to colchicine:
(1) >= 1 attack per month
(2) receiving maximally tolerated colchicine dose of therapy for >= 6 months
(3) patient compliant with therapy
Additional measures for response to therapy:
(1) FMF50 score (at least 50% improvement in 5 out of 6 criteria by 3-6 months with no worsening in any criteria)
(2) autoinflammatory disease activity index (AIDAI)
(3) autoinflammatory disease damage index (ADDI)
Additional outcomes of concern other than clinical attacks:
(1) ongoing subclinical inflammation with ongoing deposition of amyloid protein