Evaluation of pediatric rheumatologists' approach to rituximab use: a questionnaire study.

Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration. CONCLUSIONS: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests. WHAT IS KNOWN: • During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring. WHAT IS NEW: • There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment. • This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests.

Towards a standardized program of transitional care for adolescents with juvenile idiopathic arthritis for Turkey: a national survey study.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients. METHODS: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey. Based on the American Academy of Pediatrics' six core elements of transitional care, the survey included 86 questions. The respondents' demographic data, attitudes towards transitional care, and practical implementation were assessed. RESULTS: The survey included 48 rheumatologists, with 43.7% having a transition clinic. The main barriers to establishing transition programs were the absence of adult rheumatologists, lack of time, and financial constraints. Only 23.8% had a multidisciplinary team for transition care. Participants agreed on the importance of coordination and cooperation between pediatric and adult healthcare services. The timing of the transition process varied, with no consensus on when to initiate or complete it. Participants advocated for validated questionnaires adapted to local conditions to assess transition readiness. CONCLUSIONS: The study sheds light on the challenges and perspectives surrounding transitional care for JIA patients in Turkey. Despite recognized needs and intentions, practical implementation remains limited due to various barriers. Cultural factors and resource constraints affect the transition process. While acknowledging the existing shortcomings, the research serves as a ground for further efforts to improve transitional care and ensure better outcomes for JIA patients transitioning into adulthood.

Comparison of Anaerobic Exercise Capacity in Children With Familial Mediterranean Fever and Healthy Controls.

BACKGROUND/OBJECTIVE: Anaerobic exercise capacity is an important component of performing daily activities during childhood. However, diminished anaerobic exercise capacity has been reported in children with chronic conditions. Therefore, the aim of this study was to compare anaerobic exercise capacities between children with familial Mediterranean fever (FMF) and healthy peers. METHODS: Twenty-one children with FMF (FMF group) and 21 physically matched healthy controls (control group) were included. Peak power, peak power/kg, average power, and average power were evaluated using the Wingate Anaerobic Test. RESULTS: The peak power (FMF group: 254.8 W [IQR 25/75: 216.4/293.0 W] vs control group: 333.7 W [IQR 25/75: 241.3/570.5 W], p = 0.009), peak power/kg (FMF group: 6.3 W/kg [IQR 25/75: 5.2/7.0 W/kg] vs control group: 7.0 W/kg [IQR 25/75: 6.1/8.6 W/kg], p = 0.046), average power (FMF group: 186.0 W [IQR 25/75: 164.3/211.2 W] vs control group: 231.8 W [IQR 25/75: 181.8/338.1 W], p = 0.006), and average power/kg (FMF group: 4.5 W/kg [IQR 25/75: 3.8/5.0 W/kg] vs control group: 5.1 W/kg [IQR 25/75: 4.2/5.9 W/kg], p = 0.040) were found significantly higher in the control group compared with FMF group. CONCLUSIONS: Children with FMF seems to have diminished anaerobic exercise capacity compared with their healthy peers.

Clinical features of children with chronic non-bacterial osteomyelitis: A multicenter retrospective case series from Turkey.

OBJECTIVES: This study aims to evaluate demographic, clinical, and radiological characteristics of Turkish children with chronic non-bacterial osteomyelitis. PATIENTS AND METHODS: Between January 2008 and December 2018, a total of 28 patients (10 males, 18 females; median age: 12.5 years; range, 4.5 to 21 years) who were diagnosed with chronic non-bacterial osteomyelitis in three pediatric rheumatology centers were retrospectively analyzed. The demographic, clinical, and laboratory findings of the patients were recorded. RESULTS: The median age at the time of diagnosis was 10.2 years. The median time from symptom onset to diagnosis was 6.5 months. The median follow-up was 18.5 months. The most frequent symptom at onset was arthralgia (75.0%). The most frequently involved bone was the femur (67.9%). Eight (63.6%) of 12 patients had at least one Mediterranean fever gene (MEFV) mutation, indicating a significantly higher prevalence than the Turkish population (14.8%). Five of these patients fulfilled the diagnostic criteria for familial Mediterranean fever (FMF). All patients received non-steroidal anti-inflammatory drugs. Other drugs were methotrexate (46.4%), sulfasalazine (39%), corticosteroids (25%), anti-tumor necrosis factor (anti-TNF) agents (32%), pamidronate (25%), and colchicine (21.4%). Six of eight patients with MEFV mutations were administered with colchicine, and all of them responded to treatment. CONCLUSION: Clinical evolution and imaging investigations should be carefully performed to prevent any delay in the diagnosis of patients with chronic non-bacterial osteomyelitis. Based on our study results, FMF coexistence is worth investigating in patients with chronic non-bacterial osteomyelitis, particularly in the Turkish population.

Comparison of Different Pharmaceutical Preparations of Colchicine in Children with Familial Mediterranean Fever: Is Colchicine Opocalcium a Good Alternative?

BACKGROUND: Colchicine is an anti-inflammatory agent used for preventing familial Mediterranean fever (FMF) attacks and amyloidosis. A significant number of patients are non-responsive or intolerant to the domestic drug colchicum dispert. AIMS: To compare the efficacy and side effects of colchicum dispert and colchicine opocalcium in children with FMF. STUDY DESIGN: A total of 29 children with FMF who used colchicum dispert for at least 6 months initially and colchicine opocalcium for another consecutive 6 months were included. Sex and gender equity in research was considered. Clinical features, visual analog scale for pain scores, exercise-induced leg pain, and FMF severity scores with laboratory parameters were evaluated for both the treatment periods. Bristol stool chart and number of stools per 24 hours were recorded to compare the gastrointestinal side effects. METHODS: A total of 29 children with FMF who used colchicum dispert for at least 6 months initially and colchicine opocalcium for another consecutive 6 months were included. Sex and gender equity in research was considered. Clinical features, visual analog scale for pain scores, exercise-induced leg pain, and FMF severity scores with laboratory parameters were evaluated for both the treatment periods. Bristol stool chart and number of stools per 24 hours were recorded to compare the gastrointestinal side effects. RESULTS: The major indication was non-responsiveness in 18 patients (62%) and intolerance in 11 patients (38%). Usage of colchicine opocalcium (significantly higher dosage than colchicum dispert) showed statistically significant beneficial effects on the number and duration of attacks, visual analog scale for pain, exercise-induced leg pain scores, and FMF severity scores (p<0.05 for each parameter). Bristol stool chart questionnaire scores decreased from 5.62±1.56 to 4.15±1.73 points, and the scores of daily stool number decreased from 0.46±0.894 to 0.03±0.118 points (p<0.05). There were 12 patients who benefited from the switch without a change in dosage, and the clinical features were significantly better with the colchicine opocalcium treatment. CONCLUSION: Pediatric patients with FMF, who have active disease and/or gastrointestinal complaints during the use of colchicum dispert, may benefit from colchicine opocalcium.

Treatment of systemic JIA: When do we need a biologic? Real world data of a single center.

OBJECTIVES: This paper aimed to present real-world data of treatment results of a single center in patients with systemic Juvenile Idiopathic arthritis (SJIA), in which methotrexate (MTX) along with glucocorticoids was preferred as the first-line treatment option. METHODS: The medical records of 50 patients (58 episodes) with SJIA were evaluated. All patients with SJIA were hospitalized and were given high dose glucocorticoid treatment along with subcutaneous MTX. A biological agent was added in which disease activity control was not available with MTX. RESULTS: Forty-one (70.6%) of 58 episodes were controlled by MTX, following discontinuation of steroids, while a biologic drug was needed in the remaining 17 (29.4%) episodes. The patients receiving MTX were divided into two groups: Group I (n = 36) (41 episodes) consisted of patients receiving MTX alone, and Group II (n = 14) (17 episodes) consisted of patients receiving MTX plus a biologic agent. Group I was dominated by the monocyclic course (56.1%), whereas group II was dominated by persistent course (70.6%). The initial erythrocyte sedimentation rate (82 vs 67 mm/h) and neutrophil/lymphocyte ratio (8.6 vs 4.1) were significantly elevated in the Group II (p = .003 and p = .007, respectively). NLR of 5.23 predicted the requirement for biological agents with a sensitivity of 66.7%, specificity 71.4%. Odds ratio for NLR ≥ 5.23 was 5.1 in Group II. CONCLUSION: This study suggested that MTX was highly successful in cases with any SJIA episode, regardless of whether arthritis was present or not, even in cases presenting with MAS. A biologic drug is needed, if NLR is greater than 5.23.Key messagesHigh dose glucocorticoids with high dose SC methotrexate are the initial treatment option in systemic JIA.In glucocorticoid dependent patients, where methotrexate is ineffective, biologic therapy is mandatory.A neutrophil/lymphocyte ratio greater than 5.23 predicts the need for early biologic treatment.High dose S.C. MTX could be an option as an initial treatment in SJIA, especially biologics are not available.