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Background: Systemic juvenile idiopathic arthritis (sJIA) is marked with arthritis and several features of systemic inflammation, including fever, rashes, hepatosplenomegaly, lymphadenopathy, and serositis. Interleukin-1 receptor blockers have been used as one of the most effective biologics for the treatment of resistant types of disease. The main purpose of this study was to evaluate the safety and efficacy of PerkinRA in comparison with Kineret in patients with sJIA. Methods: This was a double-blind, randomized clinical trial conducted on 72 patients with sJIA. The patients were enrolled based on the 2018 International League of Associations for Rheumatology criteria and were treated at the outpatient clinic of Mofid Children's Hospital, Pediatric Medical Center, and four other centers between February 2020 and March 2021. Patients were randomly assigned in a 1:1 ratio to one of two treatment groups: one group received PerkinRA (manufactured by Persis Gene, n = 36), while the other group received Kineret (manufactured by SOBI, n = 36). The primary outcome measure was the proportion of patients achieving an American College of Rheumatology (ACR) 30, ACR 50, and ACR 70 response. Secondary outcomes included changes in vital signs, incidence of adverse events, clinical laboratory assessments, and findings from physical examinations. These parameters were evaluated at 1, 2, 4, 8, 12, 16, 20, and 24 weeks after the first injection, and the two treatment groups were compared. Results: A total of 35 patients in the PerkinRA group and 32 patients in the Kineret group were included in the final analysis. The results showed no clinically significant differences between the two treatment groups. Patients in both the PerkinRA and Kineret groups achieved similar proportions of ACR 30, ACR 50, and ACR 70 responses over the 6-month evaluation period. Conclusion: Based on the findings of this study, the two investigational medications, PerkinRA and Kineret, demonstrated comparable efficacy in the treatment of sJIA. The two treatment groups showed no significant differences in the primary and secondary outcomes assessed. Therefore, it can be concluded that PerkinRA and Kineret are noninferior to each other for the management of sJIA. [ABSTRACT FROM AUTHOR]

Background: Legg-Calvé-Perthes disease (LCPD) presents in childhood with idiopathic avascular necrosis of the femoral head(s), which can lead to disability and the need for corrective surgery at an early age. Bisphosphonates are a group of drugs that prevent osteoclastic bone resorption and may be helpful in the treatment of LCPD. So far, no trial has been conducted to investigate the effect of monthly intravenous pamidronate in the treatment of LCPD in children. Therefore, we assessed this issue in this study. Materials and Methods: This study was conducted as a clinical trial on children with LCPD who were referred to Mofid Children's Hospital (Iran-Tehran) during 2022 and 2023. Pamidronate was injected monthly and intravenously at a dose of 1 mg/kg/dose. Both groups were the same in terms of the type of treatment received (except pamidronate), the number of visits, and other follow-up parameters. The study was conducted for one year. The significance level was considered less than 0.05. Results: Forty-seven patients were included in the study. Twenty-one (60%) were in the pamidronate group, and 26 (40%) were in the control group. The mean age in the intervention group was 6.19 ± 1.69 years, and it was 6.15 ± 1.85 years in the control group (P-value=0.944). There was no statistically significant difference in children's gender (P-value = 0.731). Patients with pamidronate had a better overall score than the control group. The overall score in the pamidronate group was 2.37 more than the control group, but this difference was not statistically significant (P value=0.753). The scores of pain, lameness, walking, distance, squat, cross leg, and range of motion (ROM) in the pamidronate group were not significantly different from the control group (P value>0.05). Conclusion: Pamidronate can improve the clinical results of children with Legg-Calve-Perthes disease, and it is recommended that this drug be used along with the usual treatments for these patients. [ABSTRACT FROM AUTHOR]

Background: Hypermobility spectrum disorder (HSD), likely a multifactorial condition characterized by musculoskeletal pain, joint instability, and reduced bone mineral density (BMD), is the focus of this study. Objectives: The aim of this study was to assess and compare bone density in children diagnosed with HSD and those with benign hypermobility. Methods: This case-control study involved 73 pediatric patients diagnosed with hypermobility spectrum disorder and benign hypermobility, who were referred to the Rheumatology clinic of Mofid Hospital in Tehran between February and November 2022. Bone mineral density was measured using Z-scores from L1-L4 and the whole body less head via dual-energy X-ray absorptiometry (DEXA). Results: Significant differences were observed in the frequency distribution of spine BMD Z-scores between the two groups (P = 0.002). There was no significant correlation between spine and whole body BMD Z-scores with age and gender (P > 0.05). A notable correlation was found between spine and whole body BMD Z-scores (P < 0.001 and r = 0.64). Among the HSD group, 18 patients (81.8%) had normal BMD, while 4 patients (18.2%) exhibited low bone density, with no cases of osteoporosis. In contrast, osteoporosis was observed in 7.3% of patients in the benign hypermobility group. Conclusions: Given the high prevalence of osteoporosis in patients with benign hypermobility, clinicians should be vigilant for tendencies toward osteopenia when treating such patients. [ABSTRACT FROM AUTHOR]

Background: Renal involvement is the most damaging long-term complication of Immunoglobulin-A (IgA) vasculitis. In the lack of a definite predictive biomarker for renal involvement, antiphospholipid antibodies (aPL) have been proposed in recent years. Methods: In this prospective cohort of 48 pediatric patients who were admitted with IgA vasculitis from September 2015 to June 2017, two serum samples were taken 12 weeks apart to detect Anti-Phospholipid antibodies. All patients were followed-up for renal involvement for six months. Results: Renal involvement occurred in 14 out of 48 patients with IgA vasculitis (29.16%). APLs were positive in nine out of 14 patients with IgA vasculitis and renal involvement (64.28%), in contrast to only six out of 34 patients with IgA vasculitis without renal involvement (17.64%). The presence of aPL antibodies was statistically associated with renal involvement (P=0.002). Although, the relationship between both sex (P=0.025) and age (P=0.046) with aPL positivity was statistically significant, performing a modified logistic regression test, the odds ratio was significant between the groups with and without renal involvement only in term of age and aPL positivity). Conclusion: The presence of aPL antibodies was statistically associated with renal involvement. We found a significant relationship between the age and aPL positivity. Hence, we need multicenter, more extensive cohort studies to reach a better and more accurate conclusion on the relationship between serum aPLs and renal involvement in IgA vasculitis patients. [ABSTRACT FROM AUTHOR]

Introduction: Kawasaki disease (KD) is a systemic vasculitis that occurs mostly in children under five years old. Kawasaki affects the middle-size arteries, especially the coronary arteries. Therefore, without adequate treatment, it may cause coronary artery aneurysm in 25% of patients. The purpose of this study was to investigate the relationship between Kobayashi, Sano, and Egami criterions with coronary artery aneurysm in KD patients during the last ten years and to identify risk factors in patients with intravenous immunoglobulin (IVIG)-resistant and coronary artery aneurysms. Methodology: Medical records of 363 Kawasaki patients referred during 2008– 2017 were reviewed. Patients' demographic data and Kobayashi, Sano, and Egami scores of each patient were calculated. Based on echocardiographic findings, cases of coronary artery aneurysm were determined. Sensitivity, specificity, positive and negative predictive value, and the accuracy of each criterion were determined to predicting IVIG resistance and detect coronary artery aneurysm. Results: There was a slight relationship between IVIG-resistance in Kawasaki children and its prediction based on the Kobayashi risk score, but no relationship was found between the Egami and Sano criteria. Sixty-three patients (17.4%) had coronary artery lesions (CALs) on time of diagnosis. There were no statistically significant differences between gender and mean age of children with and without CALs. Also, there was no significant relationship between coronary artery aneurysm in Kawasaki children and its prediction based on the above three risk factors. The area under the ROC-curve of all three risk measures of Kobayashi, Egami, and Sano indicated that all three criteria were not useful in predicting CALs. Conclusion: Despite the low accuracy of the three above criteria to predictive of patients with IVIG resistance, it seems that the variables of age, duration of fever, and C-reactive protein (CRP) are more useful than other variables and may be utilized to evaluate patients by establishing a more appropriate cut-off point. [ABSTRACT FROM AUTHOR]

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide, various, and sometimes deceptive clinical and serological manifestations. Environmental factors such as ultraviolet radiation, viral infections, drugs, hormones, and chemicals could trigger SLE flares in genetically predisposed patients. Case report: We presented a 13-year-old girl with the first presentation of systemic lupus erythematosus triggered by a mosquito bite. She presented with a malar rash started after a mosquito bite on her left cheek. She had oral ulcers, photosensitivity, lymphopenia, proteinuria, and positive serologic tests for SLE. Renal biopsy revealed class II lupus nephritis. Conclusion: Environmental factors can trigger the onset of SLE in genetically susceptible cases. Besides microbial agents, UV radiation, hormones, drugs, emotional stresses, immunization, and chemicals are some of the published examples. We presented a case with a mosquito bite as the possible environmental trigger. [ABSTRACT FROM AUTHOR]

Juvenile idiopathic arthritis (JIA) refers to a group of disorders characterized by chronic arthritis. It is a chronic rheumatic disease and one of the major causes of disability in children. The diagnosis of JIA disease in children younger than 16 years with arthritis (swelling or effusion or two about decreased range of motion, warmth, pain or tenderness) for at least 6 weeks and rule out of other causes of arthritis with which they would be identifiable. The incidence and prevalence of JIA from 1 to 20 per 100,000 to 400 per 100,000 have been reported. There are several separated classification criteria for patients under 16 years of age who have chronic arthritis. The most practical and acceptable criteria are presented by American College of Rheumatology (ACR), European League against Rheumatism (EULAR), and the International League of Associations for Rheumatology (ILAR). Furthermore, spondyloarthritis in young children, including psoriatic arthritis and ankylosing spondylitis are being diagnosed with difficulty. Here in we discussed about chronic arthritis in children and the differences between these classification criteria. [ABSTRACT FROM AUTHOR]