Demirkaya, Erkan

Kalkan, Gokhan

Ozen, Seza

English

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Western University Scholarship@Western Paediatrics Publications Paediatrics Department 10-1-2012 Severity scoring system for paediatric FMF Gokhan Kalkan Gazi Üniversitesi Erkan Demirkaya Gulhane Military Medical Academy, erkan.demirkaya@lhsc.on.ca Seza Ozen Hacettepe Üniversitesi Follow this and additional works at: https://ir.lib.uwo.ca/paedpub Citation of this paper: Kalkan, Gokhan; Demirkaya, Erkan; and Ozen, Seza, "Severity scoring system for paediatric FMF" (2012). Paediatrics Publications. 1129. https://ir.lib.uwo.ca/paedpub/1129 NATURE REVIEWS | RHEUMATOLOGY www.nature.com/nrrheumNature Reviews Rheumatology published online 11 September 2012; doi:10.1038/nrrheum.2012.54-c1CORRESPONDENCEIn a recent commentary in Nature Reviews Rheumatology, Livneh1 discussed paediatric scoring systems for familial Mediterranean fever (FMF; Severity score system for paedi‑atric FMF, Nat. Rev. Rheumatol. 8, 309‑310; 2012) in relation to our recent manu script on the topic.2 We consider sharing our opinion with the scientific community as an ethical obligation and, therefore, would like to respond to the criticisms in the Livneh commentary.1One of the key messages of our study was the lack of consistency between the current severity scoring tools for paedi‑atric FMF. However, this finding would not neces sarily make studies of sever‑ity scoring systems questionable. In this regard, Ozen et al.3 examined the environ‑mental effects on the severity of childhood FMF by adjusting Mor4 and Pras5 scoring systems to children, and did not com pare the consistency of different scoring sys‑tems, but compared the results of each scoring system according to the country of residence. Ozen et al.3 also demonstrated disagreement between the two scoring systems, although they did not specifi‑cally mention such finding. For example, the percentage of patients with severe dis‑ease in Turkey was 78.2% based on modi‑fied scoring system by Mor and colleagues. How ever, the percentage of patients with severe disease was only 34.5% according to modified Pras scoring system.CORRESPONDENCESeverity scoring system for paediatric FMFGokhan Kalkan, Erkan Demirkaya and Seza OzenAnother area of criticism in the Livneh commentary was the modification of the colchicine dose, in that it might have biased our results. The clinician’s decision regard‑ing the dose of colchicine administered for adults is primarily related to disease sever‑ity. However, age and weight are the main determinants of colchicine dosing in chil‑dren. Therefore, it is evident that the amount of dosing might not be a direct indicator of disease severity for an infant as compared with an adult. Moreover, colchicine dose by itself as a measure of disease severity might not be the best choice in light of current knowledge of pharmacogenetics; increase in colchicine dose used can be due to the pharmaco genetic profile of an indivi dual, rather than an increase in disease severity. In addition, there is a subgroup of patients who do not respond to colchicine but do respond to other therapies (such as as interleukin‑receptor blockers). In this regard, severity scoring systems, irrespective of their modi‑fication status, will probably miss a patient with severe disease.Lastly, we are pleased to hear Dr Livneh’s support for an international consortium to establish a severity scoring system specifi‑cally for children with FMF. The distinct course of the disease in the paediatric age group decreases the utility of the scoring systems of Mor et al.4 and Pras et al.,5 which were originally developed for adults with FMF. There are many qualitative FMF researchers across the Mediterranean basin. We will be supportive of any initiative that aims to bring expert experiences together.Department of Paediatrics, Gazi University, Cocuk Yogun Bakim Unitesi 10.kat Yenimahalle, Ankara 06500, Turkey (G. Kalkan). Division of Paediatric Rheumatology, Gulhane Military Medical Faculty, FMF Arthritis Vasculitis and Orphan disease Research in Paediatric Rheumatology (FAVOR), 06018 Etlik, Ankara, Turkey (E. Demirkaya). Paediatric and Nephrology Unit, Hacettepe University School of Medicine, 06100 Sihhiye, Ankara, Turkey (S. Ozen). Correspondence to: E. Demirkaya  dottore_erkan@yahoo.com doi:10.1038/nrrheum.2012.54-c1Competing interestsThe authors declare no competing interests.1. Livneh, A. Diagnosis: Severity scoring system for paediatric FMF. Nat. Rev. Rheumatol. 8, 309–10 (2012).2. Kalkan, G. et al. Evaluation of the current disease severity scores in paediatric FMF: is it necessary to develop a new one? Rheumatology (Oxford) 51, 743–748 (2012).3. Ozen, S. et al. Disease severity in children and adolescents with familial Mediterranean fever: a comparative study to explore environmental effects on a monogenic disease. Ann. Rheum. Dis. 68, 246–248 (2009).4. Mor, A. et al. Evaluation of disease severity in familial Mediterranean fever. Semin. Arthritis Rheum. 35, 57–64 (2005).5. Pras, E. et al. Clinical differences between North African and Iraqi Jews with familial Mediterranean fever. Am. J. Med. Genet. 75, 216–219 (1998).© 2012 Macmillan Publishers Limited. All rights reserved

Severity scoring system for paediatric FMF

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Severity scoring system for paediatric FMF

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