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Objectives: Intra-articular corticosteroid injection (IACI) is a safe first-line or adjunct therapy used in any subtype of juvenile idiopathic arthritis (JIA). Limited studies evaluated the effect of IACI on cartilage. Our study aimed to examine the femoral cartilage thickness of patients with JIA who received IACI to the knee joint using ultrasound. Methods: We randomly selected JIA patients who performed IACI in the knee joint. Baseline bilateral joint cartilage and tendon thicknesses were measured. The articular fluid was aspirated, and applied IACI at the same period. Six months after injection, the exact measurements were repeated. Distal femoral cartilage, quadriceps tendon, and distal and proximal patellar tendon thicknesses were compared at the baseline (before IACI) and 6 months after IACI. Results: Thirty patients with JIA were included, and 23 (76.7%) were female. The median age was 11 years (interquartile range, 6 to 14), and the median disease duration was 3.3 years (interquartile range, 5 months to 5 years). The subtypes of JIA were oligoarticular in 25 (83.3%), polyarticular in 2 (6.7%), enthesitis-related arthritis in 2 (6.7%), and juvenile psoriatic arthritis in 1 (3.3%). Distal femoral cartilage thickness was 2.96 ± 0.79 mm at baseline and 2.85 ± 0.70 mm at 6 months after IACI (P = .35). The tendon thicknesses were similar at 6 months after baseline measurements. Conclusions: Our findings reveal that knee IACI in patients with JIA did not significantly change cartilage and tendon thicknesses. This observation could indicate that IACIs have no detrimental effects on the cartilage and the tendons. [ABSTRACT FROM AUTHOR]

Objectives We aimed to comprehensively analyse the available literature to identify the unmet requirements in transitional programs tailored specifically for patients diagnosed with JIA. Methods According to published guidance on narrative reviews, a systematic review of the literature on transitional care in rheumatology was conducted. Pertinent documents were collected from reputable databases, such as Web of Science, Scopus, and MEDLINE/PubMed. The search encompassed literature published from the inception of each database until January 2023. Results In this study, a comprehensive analysis of the findings of 34 studies was conducted. Among these, 12 studies focused on assessing the readiness of adolescents and young adults diagnosed with JIA. Additionally, 18 studies examined the effectiveness of structured transition programs in terms of adherence and satisfaction. Finally, 4 studies investigated disease-related outcomes in this population. Conclusion The need for transitioning children with rheumatic diseases to adult rheumatology services for continued care is clearly evident. However, the absence of established best practice guidelines presents a challenge in facilitating this transition effectively. Although several scoring systems have been proposed to ensure organized and seamless transfers, a consensus has not yet been reached. Furthermore, the socio-economic and cultural variations across countries further complicate the development of universal guidelines for transitioning children with rheumatic diseases. To address these concerns, our objective in conducting this literature review was to emphasize the significance of this issue and identify the specific requirements based on the unmet needs in the transition process. [ABSTRACT FROM AUTHOR]

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Neurofibromatosis type 1 (NF-1) is a significant autosomal dominant disorder with a wide spectrum of clinical findings. These signs (Café au lait spots, bone dysplasia, Lisch nodules) usually start to emerge after the first months of life and most are benign in nature. On the other hand, neoplasms (optic glioma, neurofibroma, malignant peripheral nerve sheath tumor, soft tissue sarcoma, leukemia, breast cancer) are a major cause of morbidity and mortality in NF-1 patients. Cancer risk during lifetime of a NF-1 patient is almost 10 times more than a person without NF-1, but what drives these patients into cancer is still unknown. This study aims to analyze the possible association of clinical findings with malignancies in children with NF-1. Medical records of 55 children with NF-1 who were followed up in a tertiary care pediatric oncology clinic between January 2005 and December 2014 were analyzed. We assessed clinical and demographic characteristics of patients, as well as the NF-1 diagnostic criteria, NF-1 related complications, and malignancies. The NF-1 patients without malignancy were classified in Group 1 while patients with malignancy were in Group 2. Logistic regression analysis was used to determine the risk factors of malignancy in NF-1. The mean age was 7.68±4.65 years. Female sex was dominant in both groups. Café au lait spots were present in all patients. Axillary-inguinal freckling was observed in 76.4% of patients, followed by neurofibromas in 30.9%, Lisch nodules in 29.1%, bone dysplasia in 14.5%, optic gliomas in 23.6%, and a history of first degree relative with NF-1 in 63.6%. Central nervous system (CNS) tumors were present in 40%. Tumors other than CNS tumors were acute myeloid leukemia and schwannoma. None of the diagnostic criteria was a risk factor for predisposing to malignancy by itself. Having >3 criteria was found to be the risk factor for malignancy in NF-1 (OR:5.891, CI 95%: 1.676-20.705, p=0.006). There are no clearly defined risk factors predicting occurance of malignancies in NF-1 at present. However, we found a higher risk of malignancy association in patients who meet more than 3 diagnostic criteria of NF-1. [ABSTRACT FROM AUTHOR]

Objectives: Familial Mediterranean fever (FMF) may present with various concomitant diseases. This study aims to evaluate the clinical characteristics of patients with FMF with Juvenile Spondyloarthropathy (jSpA). Method: Thirty-two patients diagnosed with FMF/jSpA, sixty-four with FMF, and fifty-four with jSpA were included in this retrospective study. Three patient groups were compared in terms of clinical and laboratory features. Results: The mean ages of patients in the FMF/jSpA, FMF and jSpA groups were 15.75(11.50-19.83), 15,41(6.83-21.50), and 16(9-22) years, respectively. Chronic arthritis (OR: 0.11, p=.049), erythrocyte sedimentation rate values (OR:1.07, p=.011), and C-reactive protein values (OR:1,08, p: .039) of the patients in remission period were found higher, the international severity scores for FMF (ISSF) before and after colchicine treatment (OR: 1.16, p: .021, OR: 2,21, p: .012) were higher in the FMF/jSpA group compared to FMF. Plantar fasciitis was more common and HLA-B27 positivity rate was lower in the FMF/jSpA group (OR:0.08, p=.024), (OR:4.71, p¼.002) compared to jSpA. FMF/jSpA patients were divided as previous diagnosed FMF and jSpA.The diagnosis of jSpA was at a younger age(p=.002), Juvenile arthritis damage index-articular(p=0.022) and extraarticular(p=.026), and the rate of biologic drug usage (p=.015) were higher in the previous jSpA group. The number of FMF attacks before colchicine was lower in the previous jSpA group (p=.02). Conclusion: Our findings suggest that both classical FMF and jSpA findings were lower in patients with FMF/jSpA. Patients who were diagnosed with jSpA at an early age and who had enthesitis and plantar fasciitis should also be evaluated in terms of FMF. [ABSTRACT FROM AUTHOR]

Background: A new semiquantitative classification (SQC) for pediatric Henoch–Schönlein nephritis (HSN) was defined recently. The outcomes of pediatric HSN patients are reevaluated according to the new classification. Methods: Primary kidney biopsies from 80 HSN patients were scored using the new SQC. The International Study of Kidney Disease in Children (ISKDC) and SQC classifications were compared in terms of the patient outcomes. Outcomes were defined as: Outcome A (n = 44) patients with no sign of renal disease, Outcome B (n = 32) patients with minor urinary abnormalities, and Outcome C (n = 4) patients with active renal disease. Results: The patients with outcome C had significantly higher biopsy scores and chronicity indices than patients in group A. There was no significant difference in areas under the curve between total biopsy SQC scores and ISKDC findings. Conclusions: Our results suggest that the modified SQC is not more sensitive than ISKDC classification for predicting the outcome in HSN cases. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to discuss the clinical, laboratory and genetic findings, and treatment options for six patients who were diagnosed with Blau syndrome (BS)/early-onset sarcoidosis (EOS). Patients and methods: The study included four patients (2 males,2 females; mean age 7 years; range 4 to 10 years) with EOS and two siblings (1 male, 1 female; mean age 10 years; range, 9 to 11 years) with BS. Age, age of initial symptoms, age of diagnosis; articular involvement, presence of uveitis, dermatitis, or fever, other organ involvement, laboratory findings, results of metabolic tests for mucopolysaccharidosis and mucolipidosis, results of genetic, pathologic, and immunologic tests, radiologic findings to evaluate skeletal dysplasia, and treatment options were collected. Results: The median age at diagnosis of all patients was 6 years (range, 1 to 10 years). Five patients had camptodactyly and bilateral boggy synovitis in the wrists and ankles, one had granulomatous inflammatory changes in the liver and kidney biopsy, and one had attacks of fever and granulomatous dermatitis. None had uveitis. The detected mutations in nucleotide-binding oligomerization domain containing 2 (NOD2) were P268S (rs2066842), M513T (rs104895473), R702W (rs2066844), V955I (rs5743291), H343Y (rs199858111), and M491L (16:50745293). The treatments of patients included corticosteroids, non-steroid anti-inflammatory drugs, methotrexate, infliximab, adalimumab, anakinra, and canacinumab. Conclusion: Camptodactyly and boggy synovitis are important signs of BS/EOS. Methotrexate and tumor necrosis factor blockers are more effective in patients with predominantly articular symptoms. In patients 5 and 6 and their mother, we determined a novel M491L mutation in the NOD2 gene. Currently, this work is in progress towards identifying the pathogenesis and treatment options for this disease. [ABSTRACT FROM AUTHOR]