BackgroundThe prevalence of the temporomandibular joint (TMJ) involvement in patients affected by juvenile idiopathic arthritis (JIA) ranges from 17% to 87% depending on population. TMJ is frequently the first and unique joint involved in the arthritic process. Unfortunately detection of TMJ arthritis in children with JIA is difficult as early signs and symptoms are missing in most patients. Therefore failure to diagnose and treat TMJ arthritis may have severe consequences on masticatory function, like pain at biting, chewing and yawning and moreover mandibular growth can be impaired and facial asymmetries may develop. Although Magnetic Resonance Imaging (MRI) is the gold standard to identify TMJ involvement, it is affected by lack of informations about bone components. Cone beam CT (CBCT), an imagine diagnostic tool with minimal xray exposure, thanks to its 3D studies provides an accurate image of anatomical bone alteration.ObjectivesProvide a screening imaging tool for TMJ involvement in JIA patients so TMJ dysfunction can be detected and managed at an early stage.MethodsEighteen patients affected by JIA from 2 to 16 yeas of age were included in this prospective study (M/F, 3/15); they were all admitted to a Pediatric Rheumathology and Maxillofacial Surgery Integrated out-patient ambulatory, where an experienced surgeon focused on evaluation of TMJs. All symptomatic patients (uni or bilateral TMJ’s pain) performed MRI: anomalous imaging such as increased joint enhancement or intra-articular effusion, was treated with intra-articular corticosteroid injections (IACI). After about two month from intra-articular treatment CBCT was performed in all the enrolled patients (symptomatic and not).ResultsCBCT is a useful tool to improve the diagnostic and therapeutic iter of TMJ involvement in JIA; in fact on one hand it highlights the unsuccessful and palliative role of IACI and on the other hand it can reveal significant impairment of the joint bone components in frequent asymptomatic patients; systemic therapy from the early phase of TMJ involvement could prevents the irreversible modification of the mandibular growth process of undertreated inflammationConclusionCBCT is a useful tool to improve the diagnostic and therapeutic iter of TMJ involvement in JIA; in fact on one hand it highlights the unsuccessful and palliative role of IACI and on the other hand it can reveal significant impairment of the joint bone components in frequent asymptomatic patients; systemic therapy from the early phase of TMJ involvement could prevents the irreversible modification of the mandibular growth process of undertreated inflammation.References[1] Farronato G. et all “Craniofacial Growth in Children Affected by Juvenile Idiopathic Arthritis Involving the Temporomandibular Joint: Functional Therapy Management”. The Journal of Clinical Pediatric Dentistry 33(4):351–358, 200[2] Keller H., et all.” Is early TMJ involvement in children with juvenile idiopathic arthritis clinically detectable? Clinical examination of the TMJ in comparison with...

Background:Acute Rheumatic Fever (ARF) is an immunomediated multisystem disease that occurs about 2-5 weeks after Group A Streptococcus Pyogenes beta-hemolytic (GAS) pharyngitis. After a negative peak in the 1980s, following the introduction of antibiotic prophylaxis, the disease is currently recovering. Rheumatic carditis is one of the most worrying aspects as it is still one of the major causes of cardiovascular death in the young-adult population. However, if diagnosed early and treated, sequelae with aortic and mitral valve involvement can be prevented.Objectives:The aim of our study is a description of Acute Rheumatic Fever in all its manifestations in a cohort of pediatric patients belonging to the Azienda Ospedaliera Universitaria Integrata of Verona.Methods:A retrospective analysis was conducted collecting all the cases of ARF, diagnosed by Jones’s criteria, related to Pediatric Rheumatology and Pediatric Cardiology of Verona from January 2005 to December 2019. Demographic and clinical data were collected for all patients such as clinical presentation, disease evolution and cardiac involvement.Results:73 patients were analyzed, of whom 53 had an acute onset of ARF and 20 received a diagnosis of previous ARF due to indolent carditis. The prevalent age at the time of diagnosis in both groups was between 5 and 14 years of age. Among patients with acute onset, carditis was the most frequent major manifestation (94.3%), followed by polyarthritis (41.5%), chorea (24.5%) and erythema marginatum (7.5%). Only in one patient we could observe subcutaneous nodules (1.8%). Regarding the minor manifestations, the increase in inflammation markers was present in 83% of cases and fever was present in 75.5%, followed by arthralgia (58.4%) and prolonged of PR interval to ECG (9.4%). Carditis was also present in all 13 patients who presented chorea. Clinically, previously unknown heart murmur occurred in 28 patients. Therefore, the mismatch between cardiac objectivity and carditis finding is clear: infact, compared to an important finding of carditis (50 patients) only slightly more than half of the patients (28 patients) showed an evident clinic finding. Finally, no correlation was found between the levels of the antistreptolysin O titer and the severity of heart damage. Patients with early diagnosis of carditis were treated at onset with corticosteroid therapy according to the American Heart Association scheme and did not show valvular cardiac outcomes. A patient who received a late diagnosis of carditis currently presents a significant and permanent cardiac damage despite adequate steroid treatment undertaken at the time of diagnosis.Conclusion:The description of this cohort of pediatric patients shows that the ARF has not disappeared in industrialized countries. Treatment of streptococcal infection (primary prophylaxis) plays a key role in preventing ARF. Of great impact is the prevalence of carditis which is present in 94.3% of patients. Early diagnosis is therefore of primary importance and the subsequent follow-up path, consisting of periodic therapy with penicillin (secondary prophylaxis) and periodic cardiological checks, greatly affects children’s quality of life. Chorea, unlike what has been described in the literature, occurred simultaneously with the cardiac process, while the cutaneous manifestations (subcutaneous nodules and erythema marginatum), once pathognomonic of the rheumatic disease, are today of rare observation.References:[1]Carapetis, Beaton, Cunningham et al. Acute rheumatic fever and rheumatic heart disease. Nature Reviews Disease Primers. 2016;2:15084.Disclosure of Interests:None declared

BackgroundBone marrow oedema (BMO) in children is a rare clinical condition characterized by joint and bone extremity pain, out of proportion to the clinical findings, exacerbated by weight bearing, in the absence of a known etiologic cause. It is associated with typical increased signal intensity on T2-weighted MRI. Management is still under debate. Treatment has mostly been reported in adult case series encompassing analgesic drugs, a variety of pharmacological treatments (corticosteroids, bisphosphonates, vasodilators), physiotherapy, reduction of weight-bearing, or core decompression. No treatment guidelines for children are to date available.ObjectivesRecently it has become evident that BMO is associated by an increase in bone turnover, in which vitamin D plays a pivotal role. In literature association between hypovitaminosis D and BMO of the foot and ankle in adult patients is reported. No data are reported in cohorts of children. The purpose of this study is to investigate the incidence of hypovitaminosis D in a paediatric population with primary bone BMO of the feet and the role of a vitamin D supplementation therapy.MethodsA retrospective study involving 12 paediatric patients (range age 8-14 years) referred to our Rheumatologic Paediatric Clinic of Verona University in the period 2015-2018 with persistent foot pain and MRI compatible with BMO of the foot has been performed. They had all been misdiagnosed in other institutions as affected by algodystrophy or complex regional pain syndrome. Data collection included sex, age, medical and surgical history, recent or remote trauma history, symptoms at presentation, clinical examination, laboratory bone turnover markers, vitamin D levels, MRI, treatment and outcome.Results2/12 patients are male and 10/12 female (male to female ratio: 1:5). 2/12 had a previous diagnosis of juvenile idiopathic arthritis ANA + with the disease in remission at the moment of evaluation. 10/12 were previously healthy.In all cases history of minor ankle strain or recurrent microtraumas of feet prior to symptom onset had been reported. Joint hypermobility was observed in 75% of cases. One child had been previously treated with bisphosphonates and 5 with limb immobilization, without any improvement. Physical examination revealed weight bearing pain of foot or ankle in all patients. No other sensory, vasomotor, trophic ore neurological signs and symptoms were detected. Vitamin D deficiency was found in all cases (range 10 to 22 ng/ml). All patients were thus treated with adequate vitamin D daily intake. Pain relief was achieved with paracetamol, low dose ibuprofen, or a short course of oral prednisone. Rest from intense physical activity and physical therapy, avoiding detrimental feet and ankle immobilization were recommended. All children (100%) fully recovered in 3-month lag period.ConclusionBMO in children is a cause of disability and it is often misdiagnosed and incorrectly treated. Environmental factors, such as underestimated articular or bone microtraumatisms, as well as joint hy...

Background:Juvenile idiopathic arthritis (JIA) is the most common paediatric rheumatic disease. Its most threatening complication is represented by uveitis, which could cause severe visual impairment if not diagnosed and treated promptly. It is an asymptomatic uveitis and the diagnosis is only instrumental. Therefore, regular ophthalmologic surveillance is crucial in the management of JIA. To date there are no specific predictive markers of uveitis development among JIA patients, including serologic subsets. Anti-Nuclear Antibodies (ANA) positive patients have the highest risk of iridocyclitis, but ANA are not specific. They could be found in patients with JIA without uveitis, in many other rheumatologic and inflammatory conditions and also in healthy subjects (6-12% of children). Anti-DFS70 antibodies (ANA forming a specific pattern in immunofluorescence) have been taken into consideration, but their role has not yet been established in a pediatric setting. Currently, there are few reports, involving small groups of patients and with non-univocal results. Some studies report anti-DFS70 antibodies more frequently in children with rheumatological diseases, in particular JIA-related uveitis; on the contrary, others describe them in healthy ANA positive patients.Objectives:1) To evaluate the correlation between anti-DFS70 autoantibodies and the risk of developing uveitis in a cohort of patients with JIA ANA + in pediatric age;2) to compare the prevalence of anti-DFS70 in patients with JIA, with a group of healthy ANA + children, to better define the role of these autoantibodies (potential risk factor or a protective factor for the development of AARDs in children?)Methods:We evaluated retrospectively 51 patients with JIA ANA +. We divided these patients in two groups, according to the presence (n=11) or the absence (n=40) of uveitis. For each patient we evaluated: gender, current age, age at diagnosis, type of JIA, therapy, presence of other diseases, dosage of ANA (with IF-Hep2), research of anti-ENA and anti-DFS70 antibodies (with chemiluminescence). Subsequently the whole group of patients with JIA was compared with a control group of healthy subjects aged ≤ 18 years (n=30), followed in the pediatric rheumatology clinic for occasional finding of ANA positivity, without pathologies at the moment of the study (in particular without rheumatological or autoimmune diseases).Results:Among patients with JIA without uveitis, anti-DFS70 autoantibodies were positive only in one patient. Anti-DFS70 were negative in all patients with JIA and uveitis. The difference between the two groups is not significant (p=1). In the group of healthy patients 6/30 (20%) presented a positivity of anti-DFS70 autoantibodies, in the absence of anti-ENA.Conclusion:Our data revealed no correlation between the positivity of anti-DFS70 autoantibodies and the risk of uveitis in patients with JIA ANA +, even if the number of patients is small. Further studies are needed to identify a reliable predictive marker of uveitis risk in JIA patients. The finding of a significant greater prevalence of anti-DFS70 autoantibodies in healthy ANA + subjects allows to suppose that this autoantibody could represent a possible protective marker for development of AARDs in asymptomatic children with isolated ANA positivity, as for adults. To confirm this hypothesis, it would be useful to carry on the study prospectively, encompassing children with other rheumatological diseases, and prolonging the clinical and laboratory follow up.References:[1]Clarke S, Sen ES, Ramanan AV. Juvenile idiopathic arthritis-associated uveitis. Ped Reumatol 2016; 14: 27.[2]Seeling CA, Bauer O, Seeling H-P. Autoantibodies Against DFS70/LEDGF Exclusion Markers for Systemic Autoimmune Rheumatic Diseases (SARD). Clin. Lab 2016;62:499- 517.[3]Schmeling H et al. Autoantibodies to Dense Fine Speckles in Pediatric Diseases and Controls. The Journal of Rheumatology 2015;42(12):2419-2426.Disclosure of Interests:None declared