To determine the rate of achieving The Lupus Low Disease Activity State (LLDAS) in children with systemic lupus erythematosus (SLE) for tracing pertinent treatment modalities. A total of 122 juvenile-onset SLE (jSLE) patients from six pediatric rheumatology centers in Turkey were enrolled in the study. LLDAS-50 was defined as encountering LLDAS for at least 50% of the observation time. According to the achievement of LLDAS-50, clinical features, immunological profiles, and treatments of patients with jSLE have been revealed. LLDAS of any duration was achieved by 82% of the cohort. Although only 10.8% of the patients achieved remission, 68.9% reached LLDAS-50. A significant difference was found between patients who reached LLDAS-50 and those who did not, in terms of the time to reach low-dose corticosteroid treatment (p = 0.002), the presence of subacute cutaneous findings (p = 0.007), and the presence of proteinuria (p = 0.002). Both of the groups were under similar treatment approaches. However, the number of patients being treated with corticosteroids at the last visit was found to be significantly higher in patients who achieved LLDAS-50 (p<0.001). Targeting LLDAS in jSLE, even with long-term, low-dose corticosteroid use, seems to be an achievable goal in clinical practice.

Toevaluate the safety of canakinumab using real-world data in patientswith systemic juvenileidiopathic arthritis (sJIA),andautoinflammatory diseases (AID). Thiswas a cross-sectional observational, multicenter study. Patientsdiagnosed with AID, and sJIA treated with canakinumab were includedin the study. The participating 13 centers retrospectively collectedtheir patients' data. A total of335 patients were involved in the study. Among these patients, 280were in the AID group and 55 were in the sJIA group. Canakinumab wasadministered at a median dose of 3(2.5-4) mg/kg. The median totalexposure time to canakinumab was 1.9(0.8-3.2) years, corresponding to759.5 patient-years. 779 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 upperrespiratory tract infection rate was 0.7 per patient years, while theother infection rate was 0.13 per patient-years. While no death wasobserved in any patient, SAE were observed in 8 patients.Interstitial lungdisease, anaphylaxis,or anaphylactoid reactions, werenot observed in any patient. Real-lifedata from a large cohort of patients suggests that canakinumab is assafe as claimed in clinical trials.

Objective: Rheumatoid factor (RF)-positive polyarthritis is the least common type of juvenile idiopathic arthritis (JIA). Functional disability in RF-positive polyarthritis patients is much more severe than in patients with other subtypes; but data on this subtype alone is limited. This study aimed to analyze clinical features, long-term follow-up, treatment response, and remission status in a large multicenter cohort of RF-positive polyarthritis patients. Methods: This retrospective study included RF-positive polyarthritis patients that were followed up for ≥ 6 months between 2017 and 2022 by the Pediatric Rheumatology Academy (PeRA)-Research Group (RG). Data on patient demographics, clinical and laboratory characteristics were obtained from medical charts. JIA treatments and duration of treatment were also recorded. The patients were divided into 2 groups based on methotrexate (MTX) response, as follows: group 1: MTX responsive, group 2: MTX unresponsive. Clinical and laboratory findings were compared between the 2 groups. Results: The study included 56 (45 female and 11 male) patients. The median age at onset of RF-positive polyarthritis was 13.2 years [(interquartile range) (IQR): 9.0–15.0 years] and the median duration of follow-up was 41.5 months (IQR: 19.5–75.7 months). Symmetrical arthritis affecting the metacarpophalangeal and proximal interphalangeal joints of the hands was commonly observed. Subcutaneous MTX was the preferred initial treatment; however, it was ineffective in 39 (69.6%) of the patients. Of 25 patients followed for 24 months, 56% still had active disease at 24 months. Conclusion: During 2 years of treatment, 44% of RF-positive polyarthritis patients have inactive disease, and they should be considered as a distinct and important clinical entity requiring aggressive and early treatment.