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Background: To date, standardized methods for assessing the disease progression of linear scleroderma of the face (LSF) are lacking. Objectives: We investigated whether Cone Beam Computed Tomography (CBCT) may represent a reliable tool for assessing linear scleroderma of the face (LSF). Methods: Ten patients with LSF and five age-matched controls underwent CBCT assessment. The transverse sections at three anatomic levels of the maxillofacial bones were analyzed. Measurements of soft tissue and total thickness of both affected and unaffected side of the face were made by a standardized methodology. Six raters evaluated CBCTs twice and blindly one from the other. The intra and inter-rater reliability was assessed by the Intraclass Correlation Coefficient (ICC). Results: CBCT was fast and well tolerated by the patients. The inter-rater concordance for the total thickness was excellent, mean ICC 0.75 for patients, 0.89 for controls. The mean ICC for soft tissue thickness was 0.49 for patients, 0.66 for controls. 58.3% of the measurements for patients and 91.2% of those for controls showed excellent ICC results (⩽ 0.75). The intra-rater concordance resulted optimal (ICC 0.77-0.99). Conclusions: CBCT is a reliable technique to assess skin and bony changes of LSF. [ABSTRACT FROM AUTHOR]

An abstract of the conference paper "Complementary and alternative medicine (CAM) in pediatric rheumatology: an European perspective," by G. Zanon, and colleagues, is presented.

An abstract of the conference paper "Tonsillectomy efficacy is comparable to the standard medical treatment in PFAPA syndrome," by G. Vigo, and colleagues, is presented.

An abstract of the conference paper "Unexplained recurrent arthritis as presenting sign of hereditary autoinflammatory syndromes," by G. Vigo, and colleagues, is presented.

An abstract of the conference paper "Long-term methotrexate efficacy in juvenile localized scleroderma," by F. Zulian and colleagues is presented.

Background: Currently, monoarticular Juvenile Idiopathic Arthritis (monoJIA) is included in the ILAR classification as oligoarticular subtype although various aspects, from clinical practice, suggest it as a separate entity., Objectives: To describe the clinical characteristics of persistent monoJIA., Methods: Patients with oligoJIA and with at least two years follow-up entered the study. Those with monoarticular onset and persistent monoarticular course were compared with those with oligoJIA. Variables considered were: sex, age at onset, presence of benign joint hypermobility (BJH), ANA, uveitis, therapy and outcome. Patients who had not undergone clinical follow-up for more than 12 months were contacted by structured telephone interview., Results: Of 347 patients with oligoJIA, 196 with monoarticular onset entered the study and 118 (60.2%), identified as persistent monoJIA, were compared with 229 oligoJIA. The mean follow-up was 11.4 years. The switch from monoarticular onset to oligoarticular course of 78 patients (38.8%) occurred by the first three years from onset. In comparison with oligoJIA, the most significant features of monoJIA were later age at onset (6.1 vs. 4.7 years), lower female prevalence (70.3 vs. 83.4%), higher frequency of BJH (61.9 vs. 46.3%), lower frequency of uveitis (14.4 vs. 34.1%) and ANA+ (68.6 vs. 89.5%) and better long-term outcome., Conclusions: MonoJIA, defined as persistent arthritis of unknown origin of a single joint for at least three years, seems to be a separate clinical entity from oligoJIA. This evidence may be taken into consideration for its possible inclusion into the new classification criteria for JIA and open new therapeutic perspectives., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Objective: To evaluate the efficacy of levofolinic acid (LVF) administered 48 h before methotrexate (MTX) in reducing gastrointestinal side effects without interference with drug efficacy., Methods: A prospective observational study was performed including patients with Juvenile Idiopathic Arthritis (JIA) reporting significant gastrointestinal discomfort after MTX despite taking a dose of LVF 48 h after MTX. Patients with anticipatory symptoms were excluded. A LVF supplemental dose was added 48 h before MTX and patients were followed every 3-4 months. At each visit data on gastrointestinal symptoms, disease activity (JADAS, ESR, CRP values) and treatment changes were collected. Friedman test for repeated measures analyzed differences between these variables over time., Results: Twenty-one patients were recruited and followed for at least 12 months. All patients received MTX subcutaneously (mean 9.54 mg/m2) and LVF 48 h before and after MTX (mean 6.5 mg/dose), 7 received a biological agent too. Complete remission of gastrointestinal side effects was reported in 61.9% of study patients at first visit (T1) and increased over time (85.7%, 95.2%, 85.7% and 100% at T2, T3, T4, T5, respectively). MTX efficacy was maintained as showed by significant reduction of JADAS and CRP (p = 0.006 and 0.008) from T1 to T4 and it was withdrawn for remission in 7/21., Conclusions: LVF given 48 h before MTX significantly reduced gastrointestinal side effects and did not reduce drug's efficacy. Our results suggest that this strategy may improve compliance and quality of life in patients with JIA and other rheumatic diseases treated with MTX., (© 2023. The Author(s).)

Background: Studies published on gender-related differences in the gambling behavior of adolescents have focused mainly on psychological and social factors. The aim of this study was to develop separate risk factor models for male and female adolescents, considering the environmental, psychological, behavioral and socio-economic factors related to their gambling., Methods: A survey was conducted through a questionnaire developed on a dedicated web site in 2014 on a representative sample of the Italian 15-19-years-old population, including 34,922 students attending 438 secondary schools. The SOGS-RA questionnaire was used to measure gambling behavior. To verify the risk factors associated with gambling a logistic regression stratified by gender was performed., Results: In our representative sample of Italian adolescents nationwide, the prevalence of each level of gambling was higher in males than in females. The logistic regression stratified by gender found that for both genders, gambling was positively associated with internet surfing, playing sports, getting into a fight, having unprotected sex, pulling stunts, drinking alcohol at least once in the previous month, having not a satisfactory relationship with teachers, receiving pocket money from parents, spending each week much money and having someone in the family (father, sister/brother, other relatives) who gambles. On the other hand, having poor or average school marks, going to ED in the previous year, smoking at least once in the previous month, having dissatisfied with relationships with father and having a lower family income than their friends was only associated with gambling in boys. Having an accident or injury in the previous year and having a mother who gambled was associated only in girls with higher odd of at risk or problem gambling behavior. A low psychological distress is protective only in girls for risk of gambling., Conclusions: Understanding the gender-related differences, and how they emerge in younger people at the start of their gambling careers, can suggest how best to educate individuals, families and the community on the topic of gambling. Programs to prevent substance use and abuse should be multifaceted, and include efforts to prevent gambling with a gender perspective approach., (© 2022. The Author(s).)

Background: Infrared Thermography (IRT) has been used for over 30 years in the assessment of Raynaud Phenomenon (RP) and other peripheral microvascular dysfunctions in adults but, to date, very little experience is available on its use in children for this purpose. The first aim of the study was to assess reproducibility of thermographic examination after cold exposure by comparing inter-observer agreement in thermal imaging interpretation. The secondary aim was to evaluate whether IRT is reliable to diagnose and differentiate peripheral circulation disturbances in children., Methods: Children with clinical diagnosis of primary Raynaud's phenomenon (PRP), secondary RP (SRP), acrocyanosis (AC) and age-matched controls underwent sequential measurements of skin temperature at distal interphalangeal (DIP) and metacarpophalangeal (MCP) joints with IRT at baseline and for 10 min after cold challenge test. Intraclass correlation coefficient (ICC) was calculated for inter-rater reliability in IRT interpretation, then temperature variations at MCP and DIP joints and the distal-dorsal difference (DDD) were analysed., Results: Fourteen PRP, 16 SRP, 14 AC and 15 controls entered the study. ICC showed excellent agreement (> 0.93) for DIPs and MCPs in 192 measures for each subject. Patients with PRP, SRP and acrocyanosis showed significantly slower recovery at MCPs (p < 0.05) and at DIPs (p < 0.001) than controls. At baseline, higher temperature at DIPs and lower at MCPs was observed in PRP compared with SRP with significantly lower DDD (p < 0.001). Differently from AC, both PRP and SRP showed gain of temperature at DIPs and less at MCPs after cold challenge. PRP but not SRP patients returned to DIPs basal temperature by the end of re-warming time. Analysis of DDD confirmed that controls and PRP, SRP and AC patients significantly differed in fingers recovery pattern (p < 0.05)., Conclusion: IRT appears reliable and reproducible in identifying children with peripheral microvascular disturbances. Our results show that IRT examination pointed out that PRP, SRP and AC patients present significant differences in basal extremities temperature and in re-warming pattern after cold challenge therefore IRT can be suggested as an objective tool for diagnosis and monitoring of disease.

Objectives: To assess reliability of the two indexes of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), the modified Localized Scleroderma Skin Severity Index (mLoSSI) and the Localized Scleroderma Skin Damage Index (LoSDI), when applied by clinicians with different experience in scoring and managing patients with JLS. Secondary aim was to compare LoSCAT and infrared thermography (IRT) in monitoring lesions over time., Methods: Consecutive children with Juvenile Localized Scleroderma (JLS) were blindly evaluated by three examiners with different experience in Paediatric Rheumatology and with no experience in LoSCAT use. At each visit, patients were assessed by LoSCAT and IRT. Sensitivity to change of LoSCAT and IRT was assessed in a group of patients 3-6 months later. Inter-rater reliability was assessed by Intraclass Correlation Coefficient (ICC) and variance analysis (ANOVA)., Findings: Forty-seven patients (129 lesions) entered the study, and 26 (79 lesions) were re-evaluated with same modality after 4.5 (SD 1.5) months. mLoSSI showed excellent inter-rater reliability expressed by ICC 0.965 confirmed by ANOVA. Similarly, inter-rater reliability for LoSDI was good (ICC = 0.774) but worse concordance among examiners was observed. A comparable improvement of mLoSSI in all anatomic sites was noted by all examiners in 79 lesions examined in two subsequent visits and was consistent with thermography., Conclusions: Different clinical experience in JLS did not influence clinical judgement in mLoSSI which showed excellent concordance, whereas LoSDI is less precise in damage assessment and not completely reliable in monitoring skin changes. Infrared thermography confirms to be a helpful tool for detecting disease activity and reliable in monitoring lesions over time.

Background: Chronic Musculoskeletal Pain (MSP) in children can be due to non-inflammatory conditions, such as the benign joint hypermobility syndrome (BJHS) or idiopathic MSP (IMSP). Aim of the study was to evaluate type and persistence of MSP in a cohort of schoolchildren with MSP followed for 3 years, in order to identify the main risk factors., Methods: Healthy schoolchildren, aged 8-13 years, underwent a general and rheumatologic examination, focusing on presence of chronic MSP, defined as continuous or recurrent pain lasting more than 3 months and heavily interfering with daily life activities, presence of generalized joint hypermobility, the body mass index and the pubertal stage. All symptomatic subjects were re-evaluated 3 years later with the same methods., Results: Seventy of the 88 symptomatic subjects of the initial cohort of 289 were re-evaluated 3 years later. Of these, 38 (54.3 %) still presented MSP, including 19 with BJHS and 19 with IMSP. Main symptoms were lower limbs arthralgia and myalgia. MSP persisted more in females than in males (p = 0.038) and in pubertal rather than pre-pubertal subjects (p = 0.022); these subjects recovered significantly more both from BJHS (p = 0.004) and IMSP (p = 0.016). Gender did not influence the distribution of MSP according to pubertal stage., Conclusions: Female gender, BJHS and pubertal stage are important risk factors for persistence of MSP. Further studies are needed to evaluate the natural history of MSP towards adulthood and the role of the pubertal age.