Objective <p>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.</p> Methods <p>Children newly diagnosed with FMF according to the Eurofever/PRINTO criteria and who received colchicine treatment for at least 6&#xa0;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.</p> Results <p>Overall, 168 children with FMF (50.6% female) were included. FMF50 response at 6&#xa0;months was achieved in 64.8% of patients. PREDICT-crFMF and TURPAID scores were significantly higher in non-responders than in responders (<i>p</i> &lt; 0.001 for both). Higher PREDICT-crFMF (aOR 1.240, 95% CI 1.113–1.382, <i>p</i> &lt; 0.001) and TURPAID (aOR 2.009, 95% CI 1.384–2.916, <i>p</i> &lt; 0.001) scores, and M694V homozygosity (aOR 3.390, 95% CI 1.700–6.760, <i>p</i> &lt; 0.001) independently predicted FMF50 nonresponse. Both scores showed significant discrimination (AUC = 0.685 for PREDICT-crFMF; 0.670 for TURPAID; <i>p</i> &lt; 0.001). Optimal cut-offs were ≥ 3 for PREDICT-crFMF (sensitivity 67.8%, specificity 69.7%) and &gt; 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.</p> Conclusion <p>Early assessment of PREDICT-crFMF and TURPAID scores at diagnosis may help identify FMF patients at risk for colchicine resistance.</p> <p><Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> <p><b>Key Points</b></p> <p>• <i>This is the first study to evaluate PREDICT-crFMF and TURPAID scores together using FMF50 as the treatment-response outcome in children with FMF.</i></p> <p>• <i>Higher PREDICT-crFMF and higher TURPAID scores at diagnosis independently predicted failure to achieve FMF50 response at 6&#xa0;months.</i></p> <p>• <i>The significant associations of these scores with disease activity indices and inflammatory markers support their value in reflecting early inflammatory burden.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>

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From diagnosis to treatment response: predicting FMF50 response using PREDICT-crFMF and TURPAID scores in children with Familial Mediterranean Fever: a retrospective cohort study

  • Eda Nur Dizman,
  • Feray Kaya,
  • Elif Kucuk,
  • Lutfiye Koru,
  • Zelal Aydin,
  • Hatice Kubra Dursun,
  • Merve Ozen Balci,
  • Ufuk Furkan Ozdemir,
  • Serpil Meric Toprak,
  • Aynur Alizada,
  • Kubra Ozturk,
  • Fatih Haslak

摘要

Objective

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.

Methods

Children 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.

Results

Overall, 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.

Conclusion

Early assessment of PREDICT-crFMF and TURPAID scores at diagnosis may help identify FMF patients at risk for colchicine resistance.

Key Points

This is the first study to evaluate PREDICT-crFMF and TURPAID scores together using FMF50 as the treatment-response outcome in children with FMF.

Higher PREDICT-crFMF and higher TURPAID scores at diagnosis independently predicted failure to achieve FMF50 response at 6 months.

The significant associations of these scores with disease activity indices and inflammatory markers support their value in reflecting early inflammatory burden.