From diagnosis to treatment response: predicting FMF50 response using PREDICT-crFMF and TURPAID scores in children with Familial Mediterranean Fever: a retrospective cohort study
摘要
This study assessed the predictive value of TURPAID and PREDICT-crFMF scores at diagnosis for FMF50 response at the sixth month in children with Familial Mediterranean Fever (FMF) and examined their associations with disease-activity indices and acute-phase reactants.
MethodsChildren newly diagnosed with FMF according to the Eurofever/PRINTO criteria and who received colchicine treatment for at least 6 months were included. Clinical and laboratory data were retrospectively obtained from electronic medical records. Treatment response was evaluated at the 6-month follow-up visit.
ResultsOverall, 168 children with FMF (50.6% female) were included. FMF50 response at 6 months was achieved in 64.8% of patients. PREDICT-crFMF and TURPAID scores were significantly higher in non-responders than in responders (p < 0.001 for both). Higher PREDICT-crFMF (aOR 1.240, 95% CI 1.113–1.382, p < 0.001) and TURPAID (aOR 2.009, 95% CI 1.384–2.916, p < 0.001) scores, and M694V homozygosity (aOR 3.390, 95% CI 1.700–6.760, p < 0.001) independently predicted FMF50 nonresponse. Both scores showed significant discrimination (AUC = 0.685 for PREDICT-crFMF; 0.670 for TURPAID; p < 0.001). Optimal cut-offs were ≥ 3 for PREDICT-crFMF (sensitivity 67.8%, specificity 69.7%) and > 1.5 for TURPAID (sensitivity 76.3%, specificity 54.1%). PREDICT-crFMF scores correlated with erythrocyte sedimentation rate (ESR), serum amyloid A, and disease activity indices, but not Mor score; TURPAID scores correlated with ESR and all evaluated disease activity indices.
ConclusionEarly assessment of PREDICT-crFMF and TURPAID scores at diagnosis may help identify FMF patients at risk for colchicine resistance.