Pigmented villonodular synovitis (PVNS) is a rare benign disorder of a joint which affects its synovium, tendon sheaths and bursas. While most cases of PVNS are adult patients aged between 20-50 years, few afflicted children have also been reported. Clinical signs of PVNS are insidious and non-specific. Pain, swelling and stiffness are the major symptoms. Magnetic Resonance Imaging (MRI) is the best radiological method for diagnosis of PVNS, as the initial X-ray is normal in early phase of disease in most cases. Therefore, diagnosis is often delayed or confused with mechanical disorders, haemophilic arthropathy, tuberculosis, juvenile idiopathic arthritis (JIA), and other disorders. Four paediatric PVNS cases are being reported in this case series with the aim to highlight that PVNS should be considered in the differential diagnoses of chronic monoarthritis. Two of our cases were initially misdiagnosed as JIA and the remaining two as Familial Mediterranean fever (FMF). They did not respond to conventional anti-inflammatory treatment and eventually only benefited from surgery. These four cases emphasize that the radiologist and clinician should collaborate carefully while managing any child with monoarthritis to ensure that the diagnosis of PVNS is not missed.

Objectives: This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis.Patients and methods: Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.5 to 21 years) who were diagnosed with chronic non-bacterial osteomyelitis in three pediatric rheumatology centers were retrospectively analyzed. The demographic, clinical, and laboratory findings of the patients were recorded. Results: The median age at the time of diagnosis was 10.2 years. The median time from symptom onset to diagnosis was 6.5 months. The median follow-up was 18.5 months. The most frequent symptom at onset was arthralgia (75.0%). The most frequently involved bone was the femur (67.9%). Eight (63.6%) of 12 patients had at least one Mediterranean fever gene (MEFV) mutation, indicating a significantly higher prevalence than the Turkish population (14.8%). Five of these patients fulfilled the diagnostic criteria for familial Mediterranean fever (FMF). All patients received non-steroidal anti-inflammatory drugs. Other drugs were methotrexate (46.4%), sulfasalazine (39%), corticosteroids (25%), anti-tumor necrosis factor (anti-TNF) agents (32%), pamidronate (25%), and colchicine (21.4%). Six of eight patients with MEFV mutations were administered with colchicine, and all of them responded to treatment. Conclusion:Clinical evolution and imaging investigations should be carefully performed to prevent any delay in the diagnosis of patients with chronic non-bacterial osteomyelitis. Based on our study results, FMF coexistence is worth investigating in patients with chronic non-bacterial osteomyelitis, particularly in the Turkish population.

Kawasaki disease (KD) is an acute, systemic, febrile vasculitis that occurs during infancy and is the most common cause of childhood coronary artery disease. 1 Although, its etiology has not been definitively determined, recent studies have focused on the increased inflammatory cytokines in KD pathology. 2-4 The clinical features include an existence of fever persisting more than five days with mucocutaneous and lymphatic manifestations that are commonly self-limiting. However, the most serious complication is the coronary artery involvement that can be mortal. With the routine use of intravenous immunoglobulin (IVIG) treatment, the incidence of coronary artery lesions (CALs) has declined from 23% to 8%. 5 On the other hand, some patients are at risk for resistance to IVIG treatment and development of CALs. 6 In Turkey, KD was reported as the second most common type of vasculitis in a nationwide study by Ozen et al. 7 , despite the real prevalence being unknown. Previous studies about KD from Turkey reported higher prevalence of coronary arterial involvement than Japanese children based studies. 8-12 Many studies have previously been conducted about the risk factors of KD, and patients with atypical age presentation, elevated acute phase reactants and liver function tests were reported as high risk. 10-12 The Japanese-based risk scoring systems such as that by Kobayashi, Egami, and Sano was reported as inadequate for fully determining the risks for IVIG resistance and CALs in Western populations living in North

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