Comparison of Different Pharmaceutical Preparations of Colchicine in Children with FMF: Is Colchicine Opocalcium a Good Alternative?

BACKGROUND: Colchicine is an anti-inflammatory agent used for preventing FMF attacks and amyloidosis. Remarkable numbers of patients are non-responsive or intolerant to domestic drug colchicum dispert. AIM: This study aimed to compare the efficacy and side effects of colchicum dispert and colchicine opocalcium in children with FMF. STUDY DESIGN AND METHODS: Twenty-nine children with FMF, who used colchicum dispert (CD) at least six months initially, and another consecutive 6 months of colchicine opocalcium (CO) were included. Sex and gender equity in research (SAGER) was considered. Clinical features, visual analog scale (VAS) for pain scores, exercise induced leg pain (EILP), and FMF severity scores with laboratory parameters were evaluated for both treatment periods. Bristol stool chart and number of stools per 24 hours were recorded for comparing gastrointestinal side effects. RESULTS: Major indication was non-responsiveness in 18 patients (62 %), and intolerance in 11 patients (38 %). Usage of CO (significantly higher dosage than CD) showed beneficial effects on number and duration of attacks, VAS for pain and EILP scores, also on FMF severity scores, statistically significantly (p<0.05 for each parameters). Bristol stool chart questionnaire scores decreased from 5.62 ± 1.56 to 4.15 ± 1.73 points, and scores of daily stool number decreased from 0.46 ± 0.894 points to 0.03 ± 0.118 (p<0.05). There were 12 patients who benefited from the switch without a change in dosage and the clinical features were better under CO treatment significantly. CONCLUSION: Pediatric FMF patients, who have active disease and/or gastrointestinal complaints during the use of CD, may benefit from CO

The feasibility of withdrawing canakinumab in paediatric colchicine-resistant familial Mediterranean fever patients.

Objective. To determine the feasibility of withdrawing canakinumab (CAN) in a large cohort of paediatric patients with colchicine-resistant familial Mediterranean fever (crFMF)

Cluster analysis of paediatric Behcet's disease: Data from The Pediatric Rheumatology Academy-Research Group

Objectives Behcet's disease (BD) is a systemic vasculitis affecting many organ systems, with the involvement of all-sized arteries and veins. The study aims to determine the main characteristics of paediatric BD patients and also analyse the clustering phenotypes. Methods Demographic data, clinical manifestations, laboratory features, treatment schedules, and disease outcomes were achieved from patients' charts retrospectively. A cluster analysis was performed according to the phenotype. Results A total of 225 (109 male/116 female) patients with BD were enrolled in the study. The median ages of disease onset and diagnosis were 131 (36-151) and 156 (36-192) months, respectively. According to cluster analysis, 132 (58.6%) patients belonged to the mucocutaneous-only cluster (C1), while 35 (15.6%) patients fitted to articular type (C2), 25 (11.1%) were in the ocular cluster (C3), 26 (11.6%) were in the vascular cluster (C4), and 7(3.1%) belonged to the gastrointestinal cluster (C5). Ocular and vascular clusters were more common in boys (p < .001), while girls usually presented with the mucocutaneous-only cluster. The disease activity at the diagnosis and the last control was higher in ocular, vascular, and gastrointestinal clusters. Conclusions These identified juvenile BD clusters express different phenotypes with different outcomes Our analysis may help clinicians to identify the disease subtypes accurately and to arrange personalized treatment