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A score for predicting colchicine resistance at the time of diagnosis in familial Mediterranean fever: data from the TURPAID registry.


ABSTRACT:

Objectives

Colchicine forms the mainstay of treatment in FMF. Approximately 5-10% of FMF patients are colchicine resistant and require anti-IL-1 drugs. We aimed to compare the characteristics of colchicine-resistant and colchicine-responsive patients and to develop a score for predicting colchicine resistance at the time of FMF diagnosis.

Methods

FMF patients (0-18 years) enrolled in the Turkish Paediatric Autoinflammatory Diseases (TURPAID) registry were included. The predictive score for colchicine resistance was developed by using univariate/multivariate regression and receiver operating characteristics analyses.

Results

A total of 3445 FMF patients [256 (7.4%) colchicine-resistant and 3189 colchicine-responsive) were included (female:male ratio 1.02; median age at diagnosis 67.4 months). Colchicine-resistant patients had longer, more frequent attacks and were younger at symptom onset and diagnosis (P < 0.05). Fever, erysipelas-like erythema, arthralgia, arthritis, myalgia, abdominal pain, diarrhoea, chest pain, comorbidities, parental consanguinity and homozygosity/compound heterozygosity for exon 10 MEFV mutations were significantly more prevalent among colchicine-resistant than colchicine-responsive patients (P < 0.05). Multivariate logistic regression analysis in the training cohort (n = 2684) showed that age at symptom onset, attack frequency, arthritis, chest pain and having two exon 10 mutations were the strongest predictors of colchicine resistance. The score including these items had a sensitivity of 81.3% and a specificity of 49.1%. In the validation cohort (n = 671), its sensitivity was 93.5% and specificity was 53.8%.

Conclusion

We developed a clinician-friendly and practical predictive score that could help us identify FMF patients with a greater risk of colchicine resistance and tailor disease management individually at the time of diagnosis.

SUBMITTER: Batu ED 

PROVIDER: S-EPMC10907807 | biostudies-literature | 2024 Mar

REPOSITORIES: biostudies-literature

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A score for predicting colchicine resistance at the time of diagnosis in familial Mediterranean fever: data from the TURPAID registry.

Batu Ezgi Deniz ED   Şener Seher S   Arslanoglu Aydin Elif E   Aliyev Emil E   Bagrul İlknur İ   Türkmen Şeyma Ş   Akgün Özlem Ö   Balık Zeynep Z   Tanatar Ayşe A   Bayındır Yağmur Y   Kızıldağ Zehra Z   Torun Rüya R   Günalp Aybüke A   Coşkuner Taner T   İşgüder Rana R   Aydın Tuncay T   Haşlak Fatih F   Kasap Cüceoğlu Müşerref M   Esen Esra E   Akçay Ulaş U   Başaran Özge Ö   Pac Kısaarslan Aysenur A   Akal Fuat F   Yüce Deniz D   Özdel Semanur S   Bülbül Mehmet M   Bilginer Yelda Y   Aktay Ayaz Nuray N   Sözeri Betül B   Kasapçopur Özgür Ö   Ünsal Erbil E   Özen Seza S  

Rheumatology (Oxford, England) 20240301 3


<h4>Objectives</h4>Colchicine forms the mainstay of treatment in FMF. Approximately 5-10% of FMF patients are colchicine resistant and require anti-IL-1 drugs. We aimed to compare the characteristics of colchicine-resistant and colchicine-responsive patients and to develop a score for predicting colchicine resistance at the time of FMF diagnosis.<h4>Methods</h4>FMF patients (0-18 years) enrolled in the Turkish Paediatric Autoinflammatory Diseases (TURPAID) registry were included. The predictive  ...[more]

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