Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy.

OBJECTIVES: The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS). METHODS: Demographic, clinical, and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen pediatric rheumatology centers across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment. RESULTS: One hundred and sixty-two patients were included in the study. 45 of the MAS events were detected under the effect of anti-IL-1/-6 biologics, while the patients experiencing the remaining 155 events have not received biological treatment in the last three months. Platelet count [128 (72-232) vs 199 (130-371) 109/l], ferritin level on admission [1107 (676-2050) vs 2863 (1193-9562) ng/ml], C-reactive protein level [15.4 (2.9-56) vs 90 (32-160) mg/l], erythrocyte sedimentation rate [13 (3-36) vs 43.5 (13-77) mm/h] and fever duration [5 (4-7.5) vs 10 (7-14.3) days] were found lower in the group under the impact of anti-IL-1/-6 biologics. Among patients treated with biologics, 26.6% did not meet the published 2016 MAS classification criteria at presentation. The rates of hepatomegaly and splenomegaly were relatively lower in the canakinumab-treated group when compared with those receiving other biologicals or to patients, not on biologicals. CONCLUSION: Anti-IL-1/-6 therapies can mask the clinical and laboratory features of MAS, and proposed guidelines for MAS classification criteria may not be met.

The validity and reliability of the Turkish version of the Fear of Pain Questionnaire for Children-Short Form (FOPQC-SF) in children and adolescents with juvenile idiopathic arthritis.

OBJECTIVE: The aim of this study was to investigate the validity and reliability of the Turkish version of the Fear of Pain Questionnaire for Children-Short Form (FOPQC-SF) in children/adolescents with juvenile idiopathic arthritis (JIA). METHODS: To evaluate validity of FOPQC-SF, 70 children/adolescents with JIA were included. Data were collected using Pediatric Quality Of Life Inventory 3.0. Module Arthritis (PedsQL), Childhood Health Assessment Questionnaire (CHAQ) and Juvenile Arthritis Disease Activity Score (JADAS).To determine the reliability of the FOPQC-SF, test-retest was performed at one-week intervals on participants who had not made any changes to their pharmacological treatment and had not received any additional treatment. RESULTS: With factor restrictions, items of Turkish version of FOPQC-SF were found acceptable for a 2-factor structure (fear:4 items; avoidance:6 items)(RMSEA = 0.058, GFI = 0.890, X2 = 40.667 X2/df = 1.196). With no restrictions, items of Turkish version of FOPQC-SF were found to be excellent for a 3-factor structure (fear:3 items; avoidance:4 items; other:3 items) (RMSEA = 0.036, GFI = 0.909, X2 = 34.465, X2/df = 1.077).The Cronbach's alpha value of Turkish version of FOPQC-SF total was 0.865 (good). The intraclass correlation coefficient (ICC2,1) was 0.865 (very high). Fear and avoidance subscales and total score of Turkish version of FOPQC-SF had low to moderate correlation with CHAQ-disability index, CHAQ-pain, CHAQ-global evaluation, JADAS, PedsQL-child total, PedsQL-parent total (r:-0.283/-0.452)(p < 0.05). Other subscale of Turkish version of FOPQC-SF had low to moderate correlation with CHAQ-disability index, CHAQ-pain, PedsQL-parent total (r:0.286/0.318) (p < 0.05). CONCLUSION: The Turkish version of FOPQC-SF was found to be clinically valid and reliable in children and adolescents with JIA.

A clinical overview of paediatric sarcoidosis: Multicentre experience from Turkey.

OBJECTIVES: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS). METHODS: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis. RESULTS: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7). CONCLUSIONS: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications.

The safety of canakinumab in systemic juvenile idiopathic arthritis and autoinflammatory diseases in pediatric patients: a multicenter study.

OBJECTIVE: To evaluate the safety of canakinumab using real-world data in patients with systemic juvenile idiopathic arthritis (sJIA) and autoinflammatory diseases (AID). RESEARCH DESIGN AND METHODS: This was a cross-sectional observational, multicenter study. Patients diagnosed with AID and sJIA treated with canakinumab were included in the study. The participating 13 centers retrospectively collected their patients' data. RESULTS: A total of 335 patients were involved in the study. Among these patients, 280 were in the AID group and 55 were in the sJIA group. Canakinumab was administered at a median dose of 3 (2.5-4) mg/kg. The median total exposure time to canakinumab was 1.9 (0.8-3.2) years, corresponding to 759.5 patient-years. Seven hundred and seventy-nine total adverse events (AE) were identified. The total incidence of AE, and serious adverse events (SAE) throughout the study period was 1.02 per patient-years. The upper respiratory tract infection rate was 0.7 per patient-years, while the other infection rate was 0.13 per patient-years. While no death was observed in any patient, SAE were observed in 8 patients. Interstitial lung disease, anaphylaxis, or anaphylactoid reactions were not observed in any patient. CONCLUSIONS: Real-life data from a large cohort of patients suggests that canakinumab is as safe as claimed in clinical trials.