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Background: Over the past two decades there has been a remarkable advance in the management of juvenile idiopathic arthritis (JIA), which has led to considerable improvement in prognosis. In 2018, the introduction of the treat-to-target (T2T) strategy in JIA has been advocated to further ameliorate disease outcome. To provide a benchmark for comparing future outcomes in the "T2T era", this study investigates the percentage of JIA patients who achieved clinical inactive disease (CID) in the decade that preceded the publication of the T2T recommendations in JIA., Methods: The clinical charts of all JIA patients followed at the study center between 2007 and 2017 who were first seen within 6 months after disease onset and had a minimum of 6-month follow-up information available were reviewed retrospectively. The attainment of CID, defined by 2004 Wallace criteria, was assessed cross-sectionally at 6, 12, 24, and 60 months after first observation., Results: A total of 394 patients were included. Patients were classified into four "functional phenotypes": systemic arthritis (7.1%), oligoarthritis (48.2%), polyarthritis (40.4%), and other arthritis (4.3%). The overall frequency of CID was 25.1% at 6 months, 34.5% at 12 months, 44.6% at 24 months, and 49.1% at 60 months. The systemic and oligoarticular subgroups had the highest rates of CID at 6 months (32.1% and 29.5%, respectively) and at 12 months (40% and 41.1%, respectively). At the 60-month evaluation, which was available for 226 out of 394 patients (57.4%), the frequency of CID among patients still followed at study center was 42.9%, 51.7%, 46.7%, and 45.5% for the systemic, oligoarticular, polyarticular, and other arthritis phenotypes, respectively., Conclusion: A sizeable proportion of patients treated in the decade preceding the beginning of the "T2T era" and on continued follow-up did not achieve or maintain the state of CID over the long term. Future studies will determine whether the application of the T2T strategy increases the ability to achieve sustained disease quiescence in patients who respond suboptimally to the conventional therapeutic regimens., Competing Interests: Declarations. Ethics approval and consent to participate: The study protocol was approved by the Ethics Committee of Regione Liguria (Genoa, Italy) under protocol number 642/2022—DB id 12828, dated 16 June 2023. Consent for publication: Not applicable. Competing interests: The authors declare that they have no competing interests., (© 2025. The Author(s).)

Introduction: Lichen striatus (LS) is an acquired blaschkitis, with typical linear and unilateral distribution. It occurs mainly in children and is self-resolving, yet its etiopathogenesis remains widely unknown., Evidence Acquisition: A review of the literature on LS cases was performed using the keyword "lichen striatus" to retrieve all relevant articles through PubMed and Google Scholar., Evidence Synthesis: A total of 27 articles describing 440 LS patients were included in the present review. Mean age of patients was 3.8 years; male: female: ratio was 1:1.9. The present review confirms LS as a primarily pediatric condition, mainly affecting females. Dysregulation of the immune system might be involved in its pathogenesis, with cytotoxic T-cells attacking mosaic keratinocytes after loss of immune tolerance. The review confirms LS is mainly diagnosed clinically (80%) based on its clinical characteristics: erythematous (70%) or hypopigmented (24%) papules, distributed along the lines of Blashko. The benignity of this clinical entity is suggested by the rather short duration of disease (9.5 months on average) and by the uncommonness of therapy, adopted in only 20% of cases, and when needed, administered topically., Conclusions: This review examines the complexities of LS but acknowledges limitations in data sources and calls for further research.

Objectives: To determine features and frequency of ultrasound (US)-detected tenosynovitis in ankles with clinically active disease and to investigate whether its detection may affect the achievement of inactive disease in patients with new-onset juvenile idiopathic arthritis (JIA)., Methods: The study included children with new-onset JIA and clinically active disease of the ankle. Based on US, patients were stratified as having isolated arthritis or as having tenosynovitis irrespective of the presence of concomitant arthritis in the ankle. Estimation of patients who were able to achieve clinically inactive disease 6 months after starting treatment was assessed by the Kaplan-Meier method. Cox model was used to calculate hazard ratio (HR) and 95% confidence interval (CI). Reliability of US was tested using kappa statistic., Results: Forty-five patients were recruited. On US, tenosynovitis of the ankle was detected in 28 patients (62.2%); isolated arthritis was found in 17 patients (37.8%). The medial and lateral tendon compartments were the tendon sites most frequently inflamed. Patients with tenosynovitis had similar likelihood of those without tenosynovitis to achieve clinically inactive disease (60.7% and 58.8%, respectively; HR 1.12, 95%CI:0.51-2.45). In the subanalysis excluding patients who were given biologics, the probability of experiencing inactive disease was slightly higher for patients with tenosynovitis compared to those without (64.7% and 54.5%, respectively; HR 1.56, 95%CI: 0.58-4.24). The rate of US reliability was high., Conclusions: US-detected tenosynovitis is frequent in ankles with clinical arthritis at JIA onset but does not impair the chance of achieving clinically inactive disease in the early disease phase.

Objectives: Limited information is available on the clinical features, treatment modalities and outcomes of the juvenile idiopathic arthritis (JIA) categories of enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA). This study was aimed to describe the characteristics of Italian children with ERA and JPsA and to compare them with those of patients with the other categories of JIA., Methods: Patients were part of a multinational sample included in a study aimed to investigate the prevalence of disease categories, treatment approaches, and disease status in patients from across different geographical areas (EPOCA Study). All patients underwent a retrospective assessment, based on the review of clinical chart, and a cross-sectional evaluation, which included assessment of physician- and parent-reported outcomes and laboratory tests, and recording of ongoing therapies., Results: Of the 9081 children with JIA enrolled in the EPOCA Study, 1300 were recruited at 18 paediatric rheumatology centres in Italy. 45 (3.5%) had ERA and 49 (3.8%) had JPsA. Several remarkable differences in demographic features and frequency of articular and extra-articular manifestations, disease damage, impairment in physical function and health-related quality of life, school-related problems, comorbidities, and ongoing treatments were observed between ERA and JPsA and the other JIA categories., Conclusions: We described the characteristics of Italian children with ERA and JPsA and highlighted their peculiarities and their differences from the other JIA subsets. These data provide useful insights for future revisions of JIA classification and a benchmarking against which the features from other cohorts may be compared.

We describe the unique case of a patient in whom two ciliopathies with autosomal recessive transmission were clinically and molecularly diagnosed: nephronophthisis type 1 (NPHP1) and Alström syndrome (AS). NPHP1 is one of the main genetic causes of terminal kidney failure in childhood. AS is an ultra-rare multi-systemic disease, characterized by progressive kidney disease, hepatic failure, dystrophy of the rods and cones to blindness, slowly progressive neuro-sensory deafness, dilated cardiomyopathy, obesity, insulin resistance/type 2 diabetes mellitus. The coexistence in the same patient of two rare syndromes with overlapping clinical manifestations but genetically different is an eventuality to be considered. This case report would describe the onset and progression of the multi-organ manifestations of both syndromes to highlight that ciliopathies present a strong phenotype overlap but also specific peculiarities. Therefore, to make a correct diagnosis that is essential to achieve the best clinical management could be challenging., (© 2023 S. Karger AG, Basel.)

Introduction: SARS-CoV-2 infection in the pediatric population can be associated with a multiorgan inflammatory syndrome called children's multisystem inflammatory syndrome (MIS-C). The kidneys can be affected by a broad spectrum of possible injuries, whose pathogenetic mechanisms are still unclear. Case report: We report the case of a 5-year-old boy with severe cardiac involvement in the context of MIS-C. After two weeks of hospitalization, an abdominal ultrasound showed massive bladder "debris", followed by the onset of normoglycemic glycosuria. Over time, there was a progressive increase in glycosuria, and the presence of a mat of amorphous phosphate crystals was evidenced on urinary sediment. Together with the findings of hypo-uricemia, increased urinary uric acid, and globally increased urinary amino acids, a clinical picture of kidney proximal tubular damage with secondary Fanconi-like syndrome took shape., Discussion: This case report describes the case of a patient with MIS-C with cardiac and kidney involvement characterized by proximal tubular damage, which slowly improved but still persisted at the 8-month follow-up. The pathogenesis of the damage is unclear and probably multifactorial., Competing Interests: The authors GMG declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Orsi, Pepino, Rossoni, Serafino, Caorsi, Volpi, Palmieri, Faragli, Lugani, Bigatti, Ghiggeri, Verrina, La Porta and Angeletti.)