Predictors of disease course in systemic juvenile idiopathic arthritis.

PURPOSE: To identify potential predictors of the disease course of systemic juvenile idiopathic arthritis (sJIA) at the time of diagnosis. METHODS: This retrospective observational study was conducted in patients diagnosed with sJIA in our hospital between April 2009 and October 2023. The relationship between the disease course of sJIA patients and demographic, clinical, laboratory findings and complications were analyzed. RESULTS: Of the 51 patients diagnosed with sJIA, 26 (51%) patients had monocyclic, 7 (13.7%) polycyclic and 18 (35.2%) persistent disease course. 3 (5.8%) patients had a persistent disease course with persistent arthritis developed flares with systemic manifestations during follow-up. The presence of arthritis, polyarticular involvement, and hip involvement at the time of diagnosis were associated with persistent disease course (p=0.009, p=0.003, p=0.003). Serositis and higher white blood cell and neutrophil counts at the time of diagnosis were associated with a monocyclic disease course (p=0.034, p=0.002, p=0.008). However, no significant correlation was found between macrophage activation syndrome (MAS) and disease course (p=1). CONCLUSIONS: Systemic JIA patients with polyarthritis and hip involvement at disease onset may develop a persistent course. Although MAS is an important complication of sJIA, its effect on the course of the disease was not found in this study.

When should the use of biological agents be considered in persistent oligoarticular juvenile idiopathic arthritis patients?

The purpose of this study was to compare the demographic and clinical characteristics of the groups with and without bDMARDs added to the treatment of persistent oligoarticular juvenile idiopathic arthritis (JIA) patients on methotrexate (MTX) and also to determine the predictors of adding bDMARDs to treatment. This study included 86 oligoarticular JIA patients on MTX. Patients were divided into two groups receiving MTX (n = 69) and MTX plus bDMARD (n = 17). Predictors of adding bDMARDs were investigated by comparing demographic, clinical features and laboratory findings. Gender, age at diagnosis, time elapsed from the onset of symptoms to diagnosis, and disease duration, the number and distribution of affected joint at the time of diagnosis were similar in both groups. The mean JADAS10 at the time of diagnosis were 18.8 ± 4.2 and 19.5 ± 6.4 in the MTX and MTX plus bDMARDs groups, respectively (p = 0.68). JADAS10 at 3rd and 6th month were significantly higher in patients on MTX plus bDMARDs (p = 0.001, p = 0.004, respectively). In multivariate analysis, the risk of adding bDMARD was shown to increase 1.24-fold (p = 0.004, 95% CI: 1.07-1.43) for each point increase on the JADAS 10 at 3rd months. The number (p = 0.64) or type (p = 0.18) of joint involvement at disease onset were not predictors of adding a bDMARD. CONCLUSION: JADAS10 indicating ongoing severe disease activity at 3rd and 6th months rather than baseline JADAS10 is associated with the addition of bDMARDs. WHAT IS KNOWN: • Oligoarticular JIA patients have the best outcomes among JIA categories and respond favorably to first-line therapies such as non-steroidal anti-inflammatory drugs and intraarticular corticosteroid injections. • Clinically inactive disease rates have increased with the widespread use of biological agents in oligoarticular JIA patients who have not responded to initial therapies. WHAT IS NEW: • Approximately one-fifth of patients with persistent oligoarticular JIA on methotrexate may require the addition of a biological disease modifying anti-rheumatic drug during follow-up. • The JADAS10 calculated at 3 and 6 months is a valuable tool to identify patients who should be added biological disease modifying anti-rheumatic drugs in persistent oligoarticular JIA.

Adverse events of biological agents in pediatric rheumatologic diseases.

OBJECTIVES: To evaluate adverse events (AEs) in pediatric patients with rheumatologic diseases being treated with approved or off-label biologic agents (BAs). METHODS: This observational, retrospective, multicenter study was conducted from 2010 to 2022 in patients under 18 years of age with rheumatic diseases who were receiving interleukin-1 antibodies (Anti-IL1), interleukin-6 antibodies (Anti-IL6), and tumor necrosis factor alpha inhibitors (anti-TNF). Efficacy, AEs, and timing of AEs were collected from electronic medical records. RESULTS: Three hundred and fifteen BAs were prescribed to 237 patients. Fifty AEs occurred in 44 patients (18.6%). Anti-TNF exposure was present in 8 (72.2%) of 11 patients with latent tuberculosis (TB) and in all 7 patients with herpes infections. Four of 6 patients (66.7%) with recurrent upper respiratory tract infections and 7 of 8 patients (87.5%) with local skin reactions were on Anti-IL1. The cutoff value for latent TB development was determined as 23.5 months by ROC analysis (AUC: 0.684 ± 0.072, p = 0.038, 95% CI: 0.54-0.82). In patients who used BA for 23.5 months or more, the risk of latent TB was 5.94-fold (p = 0.024, 95% CI: 1.26-27.97). Drug rash with eosinophilia and systemic symptoms (DRESS) occurred in 2 patients on anakinra, and anaphylaxis occurred in 1 patient on anti-IL6. There were no cases of malignancy or death in any patient. CONCLUSION: The physician should be vigilant for latent TB in patients exposed to BA for more than 2 years. While local skin reactions are more prevalent in patients receiving anti-IL1, severe skin reactions such as DRESS may also occur.

Serum interleukin-33 and soluble suppression of tumorigenicity 2 in pediatric leukemia with febrile neutropenia.

The purpose of this study was to evaluate the association between interleukin-33 (IL-33) and its receptor Soluble Suppression of Tumorigenicity-2 (sST2) levels and bacterial infections during febrile neutropenia (FN) in pediatric patients with acute lymphoblastic leukemia (ALL). In this prospective, case-control study, participants were divided into 3 groups: ALL patients with FN (Group A), ALL patients without neutropenia and fever (Group B), and healthy children without infection and chronic disease (Group C). There were 30 cases in each group. Blood samples for IL-33 and sST2 have been drawn from patients in Group A before the initiation of treatment and on days 1 and 5 of treatment, and from patients in Groups B and C at initiation. At admission, mean IL-33 level (39.02 ± 26.40 ng/L) in Group B and mean sST2 level (185.3 ± 371.49 ng/ml) in Group A were significantly higher than the other groups (p = 0.038, p < 0.001, respectively). No difference was observed in the mean IL-33 and sST2 levels in the 5-day follow-up of patients in Group A (p = 0.82, p = 0.86, respectively). IL-33 and sST2 levels were not associated with fever duration, neutropenia duration or length of hospitalization. While C-reactive protein (CRP) was significantly higher in patients with positive blood culture (p = 0.021), IL-33 (p = 0.49) and sST2 (p = 0.21) levels were not associated with culture positivity.  Conclusion: IL-33 and sST2 levels were not found valuable as diagnostic and prognostic markers to predict bacterial sepsis in patients with FN. What is Known: • Neutropenic patients are at high risk of serious bacterial and viral infections, but the admission symptom is often only fever. • Febrile neutropenia has a high mortality rate if not treated effectively. What is New: • Febrile neutropenia is not only caused by bacterial infections. Therefore, new biomarkers should be identified to prevent overuse of antibiotics. • Specific biomarkers are needed to diagnose bacterial sepsis in the early phase of febrile neutropenia.

Assessment of quality of life, school performance, and physical activity in adolescents with Familial Mediterranean fever.

OBJECTIVES: The aim of this study is to evaluate differences in school performance, school attendance, quality of life, and physical activity in adolescents with Familial Mediterranean fever (FMF) compared to healthy controls. METHODS: One hundred and twenty-nine patients with FMF and 154 healthy controls between 13 and 18 years were included in the study. Demographic, school performance (according to grade point average), school absenteeism, and type and frequency of exercise were recorded. Quality of life was evaluated with the Pediatric Quality of Life Inventory (PedsQL) 4.0. RESULTS: The mean age of FMF patients was 15.1 ± 2.7 years, and 69 patients (53.5%) were female. School performance was significantly higher in the control group compared to FMF patients (P < 0.001). In the control group, there were significantly higher participants who engaged in professional sports (P < 0.001). Patients with FMF had significantly lower self-reported PedsQL scores in school functioning, physical, and psychosocial health domains compared to those in the control group (P = 0.001, P < 0.001, and P = 0.028, respectively). CONCLUSIONS: FMF patients demonstrated lower school performance and quality-of-life scores compared to healthy controls. In addition to improving symptoms in chronic diseases, it is important to evaluate and improve the quality of life of patients in routine practice and to ensure psychosocial well-being.

Assessment of quality of life and physical activity in patients with oligoarticular juvenile idiopathic arthritis in remission.

The purpose of this study was to evaluate physical activity (PA) and health-related quality of life (HRQOL) in children with oligoarticular juvenile idiopathic arthritis (JIA) in remission in comparison with healthy peers and to determine the disease-related factors affecting PA levels. This study was conducted with 50 oligoarticular JIA patients in remission and 50 healthy peers between 9 and 14 years. Demographic and clinical characteristics, laboratory parameters, and treatments were noted from electronic medical records. HRQOL was assessed with the Pediatric Quality of Life Inventory (PedsQL). PA was evaluated with the Physical Activity Questionnaire for Children (PAQ-C). Oligoarticular JIA patients had significantly lower self-reported median PedsQL scores in the domains of school functioning and social functioning compared to the control group (67.5 (10) vs. 75 (25), p = 0.001 and 70 (15) vs. 85 (26.3), p < 0.001, respectively). The median PAQ-C score was 2.6 (1.1) in patients with JIA and 3 (0.9) in their healthy peers (p = 0.02). The PAQ-C score was 2.8 (1.2) in patients < 8 years at the disease onset and 2.3 (1) in those aged ≥ 8 years (p = 0.022). There was no significant difference in the number of affected joints, type of affected joint, MTX and biologic agent treatment, and remission with or without drugs with the total score of the PedsQL and PAQ-C. All PedsQL domains were positively correlated with the PAQ-C.  Conclusion: Oligoarticular JIA patients demonstrated lower PA and HRQOL scores compared to healthy controls despite favorable disease control. What is Known: • Oligoarticular JIA has fewer functional limitations and disabilities compared to other JIA subtypes. • As JIA can affect all aspects of a child's life, there is a need to improve the quality of life related to the disease. What is New: • It should be considered that patients with oligoarticular JIA may show lower PA and HRQOL scores compared to healthy controls despite favorable disease control. • Since there may be a relationship between PA and HRQOL, factors that may affect PA should be investigated to provide a holistic approach to JIA treatment.

The effect of vitamin D supplementation on attacks in PFAPA syndrome patients with low vitamin D levels.

BACKGROUND-AIM: To evaluate the effect of vitamin D supplementation on the frequency and duration of attacks in patients of PFAPA syndrome with low vitamin D levels. METHODS: This retrospective study comprised PFAPA patients with vitamin D deficiency/insufficiency between 2018 and 2023. The frequency and duration of PFAPA attacks before and after vitamin D supplementation were noted. RESULTS: Seventy-one patients were included. Of the 71 patients, 24 (33.8%) had vitamin D insufficiency, and 47 (66.2%) had vitamin D deficiency. In patients with vitamin D insufficiency, mean attack frequency and mean attack duration before vitamin D supplementation were 4.3 ± 1.9/year and 2.2 ± 1.6 days, respectively, while mean attack frequency and mean attack duration after vitamin D supplementation were 3.5 ± 2.7/year per year and 1.3 ± 0.9 days respectively (p = 0.2, p = 0.2, respectively). In patients with vitamin D deficiency, mean attack frequency and mean attack duration before vitamin D supplementation were 7.4 ± 2.1/year and 2.2 ± 1.6 days, respectively, while mean attack frequency and mean attack duration after vitamin D supplementation were 3.3 ± 2.4/year and 1.3 ± 0.9 days respectively (p < 0.01, p = 0.04, respectively). When the vitamin D level and the frequency of attacks were compared, the cut-off value of vitamin D was found to be 29.7 nmol/L. CONCLUSIONS: In PFAPA patients with low vitamin D levels, the frequency and duration of PFAPA attacks were reduced with vitamin D supplementation. Especially at vitamin D level cut-off > 29.7 nmol/L, the frequency of attacks reduced significantly.

Does switching from coated colchicine to compressed colchicine improves treatment response in patients with familial Mediterranean fever?

AIM: To evaluate the treatment response to compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine. The secondary aim was to determine the demographic and clinical characteristics of responders to compressed colchicine. METHODS: We retrospectively reviewed the medical records of 1574 pediatric patients with FMF treated at Ankara Bilkent City Hospital. Sixty-one patients did not respond to coated colchicine and were switched to compressed colchicine. In these patients, the number of attacks and the International Severity Score for FMF (ISSF) during the 6 months before and 3, 6, 9, 12, and 24 months after switching from coated colchicine to compressed colchicine were recorded. RESULTS: Twelve of 61 patients (19.7%) who were switched to compressed colchicine due to intolerance responded to treatment. Of the 49/61 patients (80.3%) who were switched due to uncontrolled attacks and persistent subclinical inflammation, 25 responded to treatment. The frequency of attacks and ISSF decreased after switching. At the end of the two-year follow-up, 42 patients responded to compressed colchicine, and 19 patients received compressed colchicine plus interleukin-1-targeting drugs. CONCLUSIONS: Compressed colchicine was shown to be a useful treatment option before initiating biological agents in non-responders to coated colchicine, especially those with side effects.

The Importance of Small Joint Involvement in Oligoarticular Juvenile Idiopathic Arthritis.

OBJECTIVES: Our study aimed to evaluate the relationship of small joint involvement with demographic, clinical, and laboratory findings and to determine its possible effects on prognosis. METHODS: This retrospective observational study was conducted in patients diagnosed with oJIA in the pediatric rheumatology department of our hospital between April 2009-September 2022. The relationship between small joint involvement and demographic, clinical, laboratory findings and prognosis were investigated by statistical methods with the data recorded from the medical records of oJIA patients. RESULTS: Of the 198 patients diagnosed with oJIA, small joint involvement was observed in a total of 20 (10%) patients, 11 (5.5%) at the time of diagnosis, and 9 (4.5%) during the follow-up period. The frequency of small joint involvement in extended oJIA was significantly higher than in persistent oJIA (p=0.001). Patients with small joint involvement had significantly higher ESR and CRP values at admission (p=0.047, p=0.038) and the JADAS at 3, 6, and 12 months (p=0.001, p=0.001, p=0.018). The need for cDMARDs and bDMARDs was significantly higher in patients with small joint involvement (p=0.001, p=0.001). CONCLUSIONS: oJIA patients with small joint involvement may have higher acute phase reactants at diagnosis, a more extended course and active disease in follow-up, and the need for treatment escalation.

The role of indices in predicting disease severity and outcomes of multisystem inflammatory syndrome in children.

BACKGROUND: The aim of this study was to evaluate the role of the systemic immune inflammation index (SII), C-reactive protein/albumin ratio (CAR), the monocyte/lymphocyte ratio (MLR), and the neutrophil/lymphocyte ratio (NLR) in predicting disease severity, treatment, and prognosis in multisystem inflammatory syndrome in children (MIS-C). METHODS: This medical record review retrospectively evaluated the clinical and laboratory findings of 191 MIS-C patients followed in the Department of Pediatric Rheumatology at Ankara City Hospital, Turkey. The patients were grouped by disease severity: mild, moderate, and severe. SII, CAR, MLR, and NLR were calculated for each group. RESULTS: All patients had fever at the time of admission; 153 (80.1%) had gastrointestinal tract involvement, 74 (38.7%) had rash, 63 (33%) had conjunctivitis, 107 (56%) had cardiac involvement, 32 (15.6%) had renal involvement, and 143 (74.9%) had hematological involvement. According to logistic regression analysis, SII, NLR, MLR, and CAR were found to be predictive indexes for disease severity, need for intensive care, need for inotropes, and anakinra treatment in MIS-C. The cut-off values of ≥1605.3 for SII, ≥9.1 for NLR, and ≥3.9 for CAR increased the risk of severe disease by 3.4, 7.1, and 5.7 times, respectively. CONCLUSION: NLR, SII, MLR, and CAR are effective and useful for predicting the severity of MIS-C, the need for intensive care, and the need for anakinra treatment.

Predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis in remission off medication.

The aim of this study was to evaluate the predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis (oJIA) who achieved clinical remission off medication. This retrospective observational study was conducted between June 2009 and July 2022 in 126 patients with oJIA who achieved remission off medication. The relationships between relapse status and demographic, clinical and laboratory findings, and treatment details were evaluated using electronic medical records. Of the 126 oJIA patients who achieved remission off medication, 85 (67.5%) were female. Relapse occurred in 31 patients (24.6%) with remission off medication after a median of 18 months (IQR 7-26). No statistically significant relationship was found between gender, age at diagnosis, oJIA subtype, number of joints, ANA, ESR, CRP level, initial Juvenile Arthritis Disease Activity Score and relapse in oJIA patients who achieved remission off medication (p = 0.66, p = 0.25, p = 1, p = 0.54, p = 0.29, p = 0.59, p = 0.95 and p = 0.52, respectively). There was a statistically significant relationship between the number of intraarticular corticosteroid injections (IACIs) and relapse (p = 0.01). Patients who underwent IACI 2-3 times had more relapses than those who never underwent IACI and those who underwent IACI only once (p = 0.01, p = 0.02, respectively). A relationship was found between the length of follow-up and relapse in patients with oJIA who achieved remission off medication (p = 0.035).    Conclusion: In oJIA patients who achieve remission off medication, the probability of relapse increases in patients who need ≥ 2 IACI during the period until remission. The length of follow-up period is associated with the probability of relapse. What is Known: • Approximately one-fourth of oJIA patients who are in remission off medication have relapse. • There is a need for markers that can predict the risk of relapse in oJIA patients who achieve remission on or off medication. What is New: • The possibility of relapse should be considered in patients with oJIA who need ≥ 2 IACIs until achieving remission off medication. • The relapse rate may increase as the follow-up period prolongs in patients who achieve remission off medication.

The Effect of Clinical and Genetic Variables of Familial Mediterranean Fever Patients: Real Life Data.

BACKGROUND: The Eurofever/the Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for familial Mediterranean fever (FMF) include a combination of clinical symptoms and genotype. The pathogenicity of gene variants associated with FMF is categorized by the International Study Group for Systemic Autoinflammatory Diseases (INSAID) classification criteria. OBJECTIVE: The aim of this study was to evaluate the real-life impact and usefulness of the Eurofever/PRINTO classification criteria and the INSAID classification criteria in patients with FMF and their impact on treatment management. METHODS: In this medical records review study, the files of FMF patients who met the Eurofever/PRINTO classification criteria were reviewed. The MEFV (MEditerranean FeVer) variants were grouped according to the INSAID classification criteria. RESULTS: Of the 1062 patients, the female-to-male ratio was 1:1.01. In group 1, there were 150 patients (14.1%) who met the clinical criteria. Group 2 consisted of 912 patients (85.9%) who met the criteria according to genetic variants. The mean ages at symptom onset in groups 1 and 2 were 5.6 ± 3.8 and 1.5 ± 1.2 years, respectively ( p = 0.024). Whereas the mean annual attack frequency was 2.7 ± 3.1/year in group 1, it was 4.1 ± 2.3/year in group 2 ( p = 0.04). The pathogenic variant was higher in the colchicine-resistant group compared with the responders ( p = 0.12). CONCLUSIONS: The Eurofever/PRINTO classification criteria may provide a new perspective on the diagnosis and clinical follow-up of FMF patients. Patients with a pathogenic variant who meet the Eurofever/PRINTO classification criteria including genetic variables have earlier onset of disease and more frequent attacks than those who meet the criteria including clinical variables. These patients need regular and closer follow-ups in terms of attack frequency, colchicine dose adjustment, and colchicine resistance.

The Juvenile Spondyloarthritis Disease Activity Index Is a Useful Tool in Enthesitis-Related Arthritis: Real-Life Data.

OBJECTIVE: To compare enthesitis-related arthritis (ERA) patients with active and inactive disease at 6 months and define baseline predictors for disease inactivity. In addition, to evaluate the demographic, clinical, and laboratory characteristics of ERA patients and to identify the real-life impact of the Juvenile Spondyloarthritis Disease Activity Index (JSpADA) in predicting active disease in ERA. METHODS: This medical record review study was conducted with 56 patients who were diagnosed with ERA at our clinic between June 2009 and June 2022. Demographic and clinical characteristics, laboratory parameters, treatment, and JSpADA were recorded. RESULTS: The patients were divided into 2 groups as active (n = 34) and inactive (n = 22) according to their disease activity at month six. Sex, age at diagnosis, number and type of affected joints, and presence of sacroiliitis were similar in both groups. There was no difference in baseline erythrocyte sedimentation rate, but there was a significant difference in erythrocyte sedimentation rate at the third month ( p = 0.52 and p = 0.018, respectively). The median JSpADA values at disease onset were 3.5 (interquartile range [IQR], 3.0-4.5) and 3.3 (IQR, 2.5-4.0) in the active and inactive groups, respectively ( p = 0.27). At the third month, the median JSpADA values were 1.5 (IQR, 0.5-2.1) in the active group and 0.5 (IQR, 0.5-1.5) in the inactive group ( p = 0.037). The cutoff value for JSpADA at the third month for active disease persisting at the month six was determined as 1 point (area under the curve, 0.662 ± 0.06; p = 0.042; 95% confidence interval, 0.51-0.80) by receiver operating characteristic curve analysis. CONCLUSION: In ERA patients, a persistently high JSpADA value at follow-up is a predictive factor for active disease at the sixth month.

Biological Agent Switching in Patients With Juvenile Idiopathic Arthritis: A Tertiary Center Experience.

OBJECTIVE: The purpose of this study is to investigate the causes and outcomes of switching biological agents in juvenile idiopathic arthritis (JIA) patients using biological agents and compare the characteristics of patients whose biological agents are switched and those whose are not. METHODS: This medical records review study was conducted with 128 patients who were diagnosed with JIA at our clinic between January 2009 and January 2022 and were receiving biologic agents. Factors affecting the biologic agent switching were investigated. RESULTS: The JIA subtype with the most frequent switching in biological agents was systemic JIA (n = 13, 40.6%). Systemic JIA was followed by rheumatoid factor-negative polyarticular JIA and persistent oligoarticular JIA with 5 patients (15.6%), extended oligoarticular JIA and enthesitis-related JIA with 3 patients (9.3%), rheumatoid factor-positive polyarticular JIA with 2 patients (6.2%), and undifferentiated JIA with 1 patient (3.1%). Among the patients, 32 (25%) patients had their biological agent switched once, and 5 (3.9%) had theirs switched twice. The most frequently used biological agent was etanercept (n = 76, 59.3%), whereas the most frequently observed cases of biological agent switching were from an anti-TNF agent to another anti-TNF agent (40.6%). The reason for switching was unresponsiveness to the agent in 22 patients (68.8%), adverse effects in 6 patients (18.7%), drug intolerance in 1 patient (3.1%), and other reasons in 3 patients (9.3%). CONCLUSIONS: The most frequently used biological agent was etanercept; the most frequent cases of biological agents switching were from an anti-TNF agent to another anti-TNF agent.

A case series of intracardiac thrombi and vascular involvement in pediatric Behçet's disease.

To evaluate the general characteristics of pediatric Behçet's disease (BD) patients with thrombus and to present the clinical features, treatment responses and prognosis of patients with intracardiac thrombus. The clinical characteristics and outcomes of 15 patients with thrombus among 85 pediatric BD patients followed in the Department of Pediatric Rheumatology were evaluated retrospectively. Of the 15 BD patients with thrombus, 12 (80%) were male, 3 (20%) were female. The mean age at diagnosis was 12.9 ± 1.1 years. Thrombus was present at the time of diagnosis in 12 patients (80%), while thrombus developed in three patients within the first three months after diagnosis. The most common site of thrombus was the central nervous system (n = 9, 60%), followed by deep vein thrombus (n = 6, 40%) and pulmonary artery thrombus (n = 4, 26.6%). Three male patients (20%) developed intracardiac thrombus. The overall intracardiac thrombus rate in the 85 patients was 3.5%. Two of the three patients had thrombus in the right, and one had thrombus in the left heart cavity. In addition to steroids, 2 of the 3 patients received cyclophosphamide, while the patient with thrombus localized in the left heart cavity was given infliximab. In the follow-up, the two patients with thrombus in the right heart cavity were switched to infliximab because of resistance to cyclophosphamide. Complete resolution was observed in 2 of the 3 patients on infliximab; a significant reduction in the thrombus of the other patient was achieved. Intracardiac thrombus is a rare presentation of cardiac involvement in BD. It is usually observed in males and in the right heart. Although steroids and immunosuppressive agents such as cyclophosphamide are recommended as first-line treatment, favorable outcomes can be achieved with anti-TNFs in resistant cases.

Preadolescent-versus adolescent-onset immunoglobulin A vasculitis: The impact of age on prognosis.

BACKGROUND: This study aimed to investigate the characteristics of patients with preadolescent- and adolescent-onset immunoglobulin A vasculitis (IgAV) and to determine whether age affects IgAV outcomes in adolescents. METHODS: Demographic, clinical, and laboratory data of 333 patients diagnosed with IgAV at the Department of Pediatric Rheumatology, University of Health Sciences, Ankara City Hospital, were evaluated retrospectively. The patients were classified into two groups: preadolescents (<10 years) and adolescents (10-19 years). Subgroup analyses were also performed by grouping the adolescent patients into early, middle, and late adolescent groups. RESULTS: Of the 333 patients, 219 (65.8%) and 114 (34.2%) were preadolescents and adolescents. Palpable purpura, renal, joint, and gastrointestinal (GI) tract involvement were detected in 333 (100%), 78 (23.4%), 79 (23.7%), and 124 (37.2%) patients, respectively; testicular involvement was observed in 25 (13.3%) of 187 male patients. The frequency of renal involvement was significantly higher in the adolescent group than in the preadolescent group at the time of diagnosis (p = 0.030). Notably, joint involvement was significantly higher in the adolescent group (p = 0.001). The need for aggressive therapy was significantly higher in the adolescent group than in the preadolescent group (p = 0.003). There was no significant difference in clinical data, demographic characteristics, and laboratory findings between the adolescent subgroups (p > 0.05). CONCLUSIONS: Immunoglobulin A vasculitis can occur at any age but the disease prognosis appears to worsen with age. The present study reported that joint involvement, kidney involvement, and the need for more aggressive treatment were higher in the adolescent group than in the preadolescent group.

Intravenous immunoglobulin therapy in immunoglobulin A vasculitis with gastrointestinal tract involvement.

OBJECTIVE: The aim of this study is to evaluate the outcomes of patients who received intravenous immunoglobulin (IVIG) for immunoglobulin A vasculitis (IgAV) with gastrointestinal (GI) tract involvement, and to determine the differences between the groups that responded to IVIG and those that did not. METHODS: This retrospective study comprised 152 patients with IgAV between 2018 and 2022. Sixty-five patients (43%) had GI tract involvement. Patients with IgAV-GI involvement who had been treated with IVIG were evaluated. Patients were classified with IgAV according to the 2008 Ankara-EULAR/PRINTO/PRES. Their demographics, presentation, and management are reported. RESULTS: Twelve (7 boys/5 girls) of these patients were treated with IVIG. The median age was 90.1 (31-177) months. The mean follow-up period was 30.6 ± 9.9 months. All patients had skin involvement, joint involvement (arthralgia or arthritis), and abdominal pain. All 12 patients were given steroids (30 mg/kg/day pulse methylprednisolone for 3-7 days, followed by 2 mg/kg/day steroids) before IVIG. Nine patients received cyclophosphamide treatment (four before IVIG and five after IVIG). Complete remission was achieved in 5 of the patients with IVIG. Four patients were diagnosed with IgAV concomitant familial Mediterranean fever, and colchicine treatment was initiated. CONCLUSIONS: IVIG may be used in steroids and/or immunosuppressive drug resistant IgAV. It can be considered as a treatment option, especially in patients with multi-organ/system involvement, comorbid inflammatory diseases such as familial Mediterranean fever, and in patients with IgAV-GI tract involvement resistant to standard treatment in the advanced pediatric age group.

Factors Associated with the Development of Thrombosis in Pediatric Behçet Disease.

OBJECTIVE: The present study aimed to compare the demographic, clinical, and laboratory characteristics of patients with pediatric Behçet disease (BD) with and without thrombosis to elucidate the factors that may contribute to the development of thrombosis. METHODS: This observational, descriptive, medical records review study included patients with BD (n = 85) who were diagnosed at age younger than 16 years at our clinic between 2010 and 2022. The demographic, clinical, and available laboratory data of patients with and without thrombosis were compared. The potential risk factors for the development of thrombosis were evaluated with multivariable logistic regression analysis. RESULTS: Central venous sinus thrombosis was the most common type of thrombosis. Thrombosis was significantly more common in male patients ( p = 0.002), and regression analysis revealed that being male was a risk factor for developing thrombosis. Genital ulcers were less common in patients with thromboses. Patients with thrombosis had higher erythrocyte sedimentation rates, C-reactive protein, leukocyte, and neutrophil counts, as well as antinuclear antibody positivity. In contrast, mean platelet volume and lymphocyte counts were significantly lower in patients with thrombosis. According to the logistic regression analysis, erythrocyte sedimentation rate value >17 mm/h was a risk factor for developing thrombosis (odds ratio, 1; confidence interval, 1.1-1.8; p = 0.012). CONCLUSIONS: Male sex has been associated with an increased risk of thrombosis in children with BD. Inflammatory parameters may serve as predictive factors for thrombosis in pediatric BD.

Multisystem inflammatory syndrome in children: clinical presentation, management, and short- and long-term outcomes.

OBJECTIVE: In this study, it was aimed to evaluate the demographic, clinical and laboratory characteristics of MIS-C patients in our hospital, to share our treatment approach, and to assess the outcomes of short- and long-term follow-up. METHODS: MIS-C patients who were admitted and treated in our hospital between July 2020 and July 2021 were evaluated. Demographic, clinical, laboratory, and follow-up data were collected from patient records retrospectively. RESULTS: A total of 123 patients with MIS-C (median age, 9.6 years) were included the study. Nineteen (15.4%) were mild, 56 (45.6%) were moderate, and 48 (39%) were severe MIS-C. High CRP, ferritin, pro-BNP, troponin, IL-6, and D-dimer values were found in proportion to the severity of the disease (p < 0.001, p < 0.001, p < 0.001, p < 0.001, p = 0.005, p < 0.001), respectively. Two (1.6%) patients died. The mean follow-up period was 7.8 months. Valve failure, left ventricular dysfunction/hypertrophy, coronary involvement, and pericardial effusion were the most common cardiac pathologies in the short- and long-term follow-up of the patients. In the long-term follow-up, the most common reasons for admission to the hospital were recurrent abdominal pain (14.2%), cardiac findings (14.2%), pulmonary symptoms (8%), fever (7.1%), neuropsychiatric findings (6.2%) and hypertension (3.5%). Neuropsychiatric abnormalities were observed significantly more common in severe MIS-C patients at follow-up (p = 0.016). In the follow-up, 6.2% of the patients required recurrent hospitalization. CONCLUSION: MIS-C is a serious and life-threatening disease, according to short-term outcomes. In addition to the cardiac findings of patients with MIS-C, long-term outcomes such as neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms should be monitored. Key Points • In MIS-C patients, attention should be paid not only to cardiac findings, but also to symptoms related to other systems. • Patients should be followed up in terms of neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms that may occur during follow-up.

Evaluation of the time of patients' admission to the tertiary pediatric rheumatology center for juvenile idiopathic arthritis.

BACKGROUND: To determine the interval between disease onset and admission to pediatric rheumatology clinic of patients with juvenile idiopathic arthritis (JIA) and, to identify the factors that affect the admission time (AT) to rheumatology center. METHODS: We designed a retrospective observational study in children with JIA. The study variables were age, gender, JIA subtype, acute phase reactants (APR), disease activity scales, presence of a pediatric rheumatologist, and distance to a pediatric rheumatology center. Outcome parameter was the duration between onset of symptoms and first visit of rheumatologist. The parameters were evaluated with variance analysis and regression models. RESULTS: 198 patients (female:120 (60.6%)) were included. There were 112 (56.5%) patients in oligo-articular JIA, 27 (13.6%) in rheumatoid factor negative poly-articular JIA, 22 (11.1%) in enthesitis related arthritis (ERA), 29 (14.6%) in systemic-JIA, 4 (2%) in rheumatoid factor positive poly-JIA, two patients each in undifferentiated and psoriatic arthritis. The median AT in the systemic-JIA and other groups was 16 (IQR 10.5-27.5) and 71 (IQR 33.5-211) days, respectively. There was a significantly longer AT in the ERA group than others (p=0.005). We found a correlation between longer AT and older age, low back pain, enthesitis, and low erythrocyte sedimentation rate (ESR). In the multivariate analysis, only low ESR and enthesitis contributed an increase in AT [OR 2.05 (1.07-3.93), 6.22 (1.29-29.99)]. CONCLUSIONS: The older age, low back pain, enthesitis and low ESR contribute to the late AT. JIA requires high suspicion in children with poorly defined findings and low APR.