Search

Background Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children that often results in persistent and disabling foot impairments (1). Foot and ankle problems are common but relatively neglected manifestations of JIA (2). Ultrasound examination is beneficial for detecting subclinical disease, especially in foot and ankle, which are otherwise difficult to examine in children (3). Therefore, in this study, we evaluated the involvement of foot and ankle in JIA patients using both ultrasonography and physical examination. Objectives The aim of the study was to investigate the foot and ankle involvement in JIA patients and determine the relationship between this involvement and disease activity, functional status, and quality of life. Methods We evaluated those children who attended the Pediatric Rheumatology Department between December 2014 and April 2015. Thirty two JIA patients, aged between 6–16 years, participated in this study. Along with the demographic characteristics, the Disease Activity Score-28 (DAS-28), Childhood Health Assessment Questionnaire (CHAQ), and Pediatric Quality of Life (PEDsQoL) scores were recorded for the measurement of disease activity, physical functional status, and quality of life, respectively. The involvement of foot and ankle was investigated by both physical examination (tenderness, swelling and deformities) and detailed gray-scale ultrasonographic evaluation. Results Of the total included JIA patients, 17 patients (53%) showed foot and ankle involvement. Foot or ankle tenderness was detected in eight patients, swelling in four patients, pes planus in four patients, calcaneal valgus in four patients, limited ankle joint range in two patients, and hallux valgus was detected in three patients. The ultrasonographic investigation revealed tenosynovitis in five patients, ankle joint effusion in four patients, synovial hypertrophy in one patient, bursitis in one patient, and plantar fasciitis in one patient. There was no difference between the group with foot and ankle involvement and the group with no such involvement in terms of demographical data or disease activity (p>0.05). A lower physical functional state was detected in the patients of the foot and ankle involvement group compared to the patients who had no such sign (p 0.05). Conclusions This study showed that foot and ankle disorders are not rare in children with JIA. Appropriate evaluation and management of the foot and ankle are important for better functional status of JIA patients. References Spraul G. Koenning G. A descriptive study of foot problems in children with juvenile rheumatoid arthritis (JRA). Arthritis Care Res 1994;144–50. Hendry GJ. Turner DE. McColl J. Lorgelly PK. Sturrock RD. Watt GF et al. Protocol for the Foot in Juvenile Idiopathic Arthritis trial (FiJIA): a randomised controlled trial of an integrated foot care programme for foot problems in JIA. J Foot Ankle Res 2009;2:21. Hendry GJ. Gardner-Medwin J. Steultjens MP. Woodburn J. Sturrock RD. Turner DE. Frequent discordance between clinical and musculoskeletal ultrasound examinations of foot disease in juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2012;64:4417. Disclosure of Interest None declared

Background Familial Mediterranean fever (FMF) is the most common auto inflammatory syndrome with recurrent inflammatory attacks of fever and serositis lasting 1-4 days. It may have ongoing subclinical inflammation during attack-free periods. Blood neutrophil to lymphocyte (N/L) ratio has been used as a simple marker of inflammation both in cardiac and non-cardiac disorders. Objectives In this study, we aimed to investigate the relationship between neutrophil to lymphocyte ratio and FMF. Methods There were 160 participants (68.4%) in the FMF patient group, and 74 participants (31.6%) in the control group. Ninety of patients (38.5%) were in attack-free period, and 70 (29.9%) were in attack period. Neutrophils and lymphocyte counts were recorded and N/L ratio was calculated. Results General group comparisons revealed a statistically significant difference between groups (p Conclusions In this study our results shows that N/L ratio is higher in patients at attack period compared with FMF patients at attack free period and control groups. N/L ratio may indicate FMF attack period. Since there was no significant difference between attack free patients and control groups, N/L ratio cannot be used as a subclinical inflammation marker. References Uluca U, Ece A, Sen V, Karabel D, Yel S, Gunes A, Tan I, Sabas M. Usefulness of mean platelet volume and neutrophil-to-lymphocyte ratio for evaluation of children with Familial Mediterranean fever. Med Sci Monit. 2014 Sep 5;20:1578-82. Celikbilek M, Dogan S, Akyol L, Borekci E, Zararsiz G, Kozan M, Gunaydin I. Neutrophil-Lymphocyte Ratio in Patients With Familial Mediterranean Fever. J Clin Lab Anal. 2014 Mar 28. Ahsen A, Ulu MS, Yuksel S, Demir K, Uysal M, Erdogan M, Acarturk G. As a new inflammatory marker for familial Mediterranean fever: neutrophil-to-lymphocyte ratio. Inflammation. 2013 Dec;36(6):1357-62. Disclosure of Interest None declared

Background Familial Mediterranean fever (FMF) is the most common auto inflammatory syndrome with recurrent inflammatory episodes. Colchicine is the main treatment of FMF. It is also effective in preventing secondary amyloidosis. There is increasing number of refractory cases which were successfully treated with biologics targeting IL-1 production in patients with FMF. However, currently there is no controlled study evaluating the safety and efficacy of IL-1 blockage in management of FMF patients who were unresponsive to colchicine. Objectives The aim of this report was to evaluate and discuss treatment of pediatric FMF patients with anti-interleukin1 (IL1) agents. Methods We retrospectively reviewed the medical records of FMF patients attended to our pediatric rheumatology department. Six patients diagnosed as colchicine resistant were participated in our series. Refractory FMF or unresponsive to colchicine was described as severe and frequent attacks and/or having high acute phase reactance levels despite having a maximum dose of colchicine (2mg/day). Disease course, adverse effects, duration of follow-up, treatment protocols, responses to the therapies were discussed. Results Six patients (5 male, 1 female) having refractory FMF were identified. The mean age of the patients was 14.3 years (range 10.6-19). The mean time of colchicine usage was 6 years (range 2-10). The median follow-up time from starting of colchicine to the beginning of biologic agents was 5.1 years (range 1-10). The mean time of anti-IL 1 agents usage was 0.99 years (range 0.15-1,5). MEFV gene analyses revealed homozygote M694V mutations in 5 patients and heterozygote M694V mutations in one patient. They were all treated with anakinra and/or canakinumab along with the colchicine management. The use of anti-IL1 drugs were beneficial to all patients with complete remission. Injection- side reaction developed in only one patient and lasted in three weeks. None of them had any severe adverse effects due to the therapy. Conclusions Anakinra and canakinumab seems to be effective in patients difficult to treat with colchicine as shown both in our series and in the literature. Therefor controlled trials are needed to evaluate the safety and long term efficacy of IL-1 targeting agents in resistant patients. References Lidar M, Scherrmann JM, Shinar Y, Chetrit A, Niel E, Gershoni-Baruch R,Langevitz P, Livneh A. Colchicine nonresponsiveness in familial Mediterraneanfever: clinical, genetic, pharmacokinetic, and socioeconomic characterization.Semin Arthritis Rheum. 2004 Feb;33(4):273-82. Meinzer U, Quartier P, Alexandra JF, Hentgen V, Retornaz F, Kone-Paut I.Interleukin-1 targeting drugs in familial Mediterranean fever: a case series and a review of the literature. Semin Arthritis Rheum. 2011 Oct;41(2):265-71. Ozen S, Bilginer Y, AktayAyaz N, Calguneri M. Anti-interleukin 1 treatmentfor patients with familial Mediterranean fever resistant to colchicine. JRheumatol. 2011 Mar;38(3):516-8. Roldan R, Ruiz AM, Miranda MD, Collantes E. Anakinra: new therapeutic approachin children with Familial Mediterranean Fever resistant to colchicine. Joint Bone Spine. 2008 Jul;75(4):504-5. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1694