No abstract

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS ( p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was − 1.64 (− 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was −2.81 ([− 3.79] to [− 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.

No abstract

No abstract

No abstract

Chronic recurrent multifocal osteomyelitis (CRMO) is a clinical entity which occurs mainly in children and adolescents with recurrent episodes of pain occurring over several years. Cause and physiopathology of disease is still uncertain. We aim to assess clinical characteristics and treatment options, need and response to anti-inflammatory therapies in children diagnosed chronic recurrent multifocal osteomyelitis Demographic data and clinical features of seventeen children diagnosed with CRMO in 2 pediatric rheumatology centers in Turkey were reviewed retrospectively. The diagnosis was based on clinical findings, radiological images and histopathological and microbiological studies. A total of 17 patients were included in the study. The median age of diagnosis was 9.6±4.2 years. The mean follow-up time was 31.6 months (range 6-35 months). Most patients (n: 10) had a recurrent multifocal disease course (>6 months), 6 patients had a persistent course and a patient had only one episode of CRMO. MEFV gene mutations were detected in 4 patients whose clinical features reduced with colchicine therapy. All patients had received nonsteroidal anti-inflammatory drugs but only one had complete response. Thirteen children with NSAID failure subsequently received corticosteroids, sulfasalazine, methotrexate, Anti TNF α drugs, or a combination of these drugs. This study is the largest cohort of pediatric CRMO patients in our country. Clinical evolution and imaging investigations should be closely done to avoid delays in diagnosis. Ethnic differences create changes in the presentation of the disease and response to treatment

Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children. It is a group of heterogeneous disorders that have chronic arthritis as a common feature. It has a worldwide distribution and many studies have shown that subtype frequencies in JIA seem to be showing geographical distribution. The aim of this study was to define subtype frequencies, demographic features, and the rates of macrophage activation syndrome, uveitis and remission in Turkish JIA patients. The files of all JIA patients (378 cases) that were being followed in Pediatric Rheumatology Clinic of our institution, between May 2010 and February 2016 were reviewed. Two hundred and sixty-five patients were included into the study. Gender, JIA subtype, age at diagnosis, age at the initial symptoms, JIA medications, uveitis presence, JIA status at the time of enrollment were recorded from the files. There were 87 enthesitis related arthritis, 87 oligoarthritis (81 persistent, 6 extended), 36 rheumatoid factor (RF) negative polyarthritis, 35 systemic arthritis, 10 RF-positive polyarthritis, 5 psoriatic arthritis and 5 undifferentiated arthritis cases. Mean age at diagnosis was 9.9 ± 4.9 years and male/female ratio was 1.05. Uveitis was found in 4.5% of the cases. Biologics were used in 26% of the patients. At the time of enrollment, 69% of the patients were under remission while 31% of them were active. Systemic arthritis and persistent oligoarthritis cases were the groups that most commonly achieved remission, while patients with polyarticular involvement, namely RF positive polyarthritis, RF negative polyarthritis and extended oligoarthritis patients were the groups with high number of active patients. In conclusion, JIA is a heterogeneous group of disorder, and differences in subtype frequencies from country to country make it even more heterogeneous disease. Patients with polyarticular involvement may need early and aggressive treatment to control the disease activity.

No abstract