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Objectives To compare the clinical and laboratory features of paediatric SSc sine scleroderma (ssJSSc) with adult-onset ssSSc. Methods Demographic, clinical and laboratory data of ssJSSc, retrospectively retrieved from our hospital medical records, case reports from the literature and from the Pediatric Rheumatology European Society JSSc registry, were compared with the Padua cohort of adult patients with ssSSc. Patients were defined as having ssSSc if they never had skin involvement but all the following features: (i) RP and/or digital vasculopathy, (ii) positive ANA, (iii) internal organs involvement typical of scleroderma and (iv) no other defined CTD. Results Eighteen juvenile and 38 adult-onset ssSSc patients, mean disease duration 5.8 and 9.7 years, respectively, entered the study. The frequency of females affected was significantly lower in ssJSSc (38.9% vs 89.5%, P  < 0.0001). When compared with adults, ssJSSc displayed fewer SSc-specific capillaroscopy abnormalities (68.8% vs 94.7%, P  = 0.02) while having significantly higher vascular (digital pitting scars, ulcers 35.3% vs 10.5%, P  = 0.042), respiratory (50.0% vs 23.7%, P  = 0.02) and cardiac (50.0% vs 2.6%, P  < 0.0001) involvement. The outcome was significantly worse in ssJSSc as six patients (33%) died (n  = 3) or reached an end-stage organ failure (n  = 3) in comparison with only two deaths (5.3%) in the adult cohort. ACA were significantly lower in children (20.0% vs 68.4%, P  = 0.001) while no difference was noted for other SSc-specific autoantibodies. Conclusion Compared with adults where ssSSc generally has an indolent course, children present with aggressive disease that heralds a worse prognosis characterized by high cardiorespiratory morbidity and mortality. [ABSTRACT FROM AUTHOR]

Introduction: Our aim was to describe a monocentric cohort of young adult patients with juvenile idiopathic arthritis (JIA), assessing the risk of relapse after transition to adult care. Methods: We conducted a retrospective study and collected clinical, serological, and demographic data of young adult patients (18–30 years old) referred to the Transition Clinic of a single Italian centre between January 2020 and March 2023. Patients with systemic-onset JIA were excluded. Primary outcome was disease relapse, defined by Wallace criteria. Risk factors were analysed by Cox proportional hazards regression. Results: Fifty patients with age 18–30 years old were enrolled in the study and followed for a median 30 months. The median disease duration at transition was 15 years. Twenty (40%) patients were on conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) and 38 (76%) were on biological DMARDs through adulthood. Twenty-three patients relapsed after transitioning to adult care for a median 9-month follow-up (IQR 0–46.5). Most relapses involved the knees (69.6%). The univariate analysis identified monoarthritis (HR 4.67, CI 1.069–20.41, p value = 0.041) as the main risk factor for relapse within the first 36 months of follow-up. Early onset, ANA positivity, past and ongoing treatment with csDMARDs or bDMARDs, therapeutic withdrawal, and disease activity within 12 months before transition did not significantly influence the risk of relapse. Conclusion: In JIA patients, the risk of relapse after transitioning to adult care remains high, irrespective of disease subtype and treatment. The main risk factor for the early occurrence of articular activity is monoarticular involvement. [ABSTRACT FROM AUTHOR]

Objectives To evaluate whether in juvenile localized scleroderma (JLS), non-invasive imaging can differentiate affected from non-affected skin and whether imaging correlates with a validated skin score [Localised Scleroderma Cutaneous Assessment Tool (LoSCAT)]. Methods A total of 25 children with JLS were recruited into a prospective study and a single 'target' lesion was selected. High-frequency ultrasound (HFUS, measuring skin thickness), infrared thermography (IRT, skin temperature), laser Doppler imaging (LDI, skin blood flow) and multispectral imaging (MSI, oxygenation) were performed at four sites: two of affected skin (centre and inner edge of lesion) and two of non-affected skin (1 cm from the edge of the lesion 'outer' and contralateral non-affected side) at four visits at 3 month intervals. Results Differences between affected and non-affected skin were detected with all four techniques. Compared with non-affected skin, affected skin was thinner (P  < 0.001), with higher temperature (P  < 0.001–0.006), perfusion (P  < 0.001–0.039) and oxygenation (P  < 0.001–0.028). Lesion skin activity (LoSCAT) was positively correlated with centre HFUS [ r  = 0.32 (95% CI 0.02, 0.61), P  = 0.036] and negatively correlated with centre LDI [ r  = −0.26 (95% CI −0.49, −0.04), P  = 0.022]. Lesion skin damage was positively correlated with centre and inner IRT [ r  = 0.43 (95% CI 0.19, 0.67), P  < 0.001 and r  = 0.36 (95% CI 0.12, 0.59), P  = 0.003, respectively] and with centre and inner LDI [ r  = 0.37 (95% CI 0.05, 0.69), P  = 0.024 and r  = 0.41 (95% CI 0.08, 0.74), P  = 0.015, respectively]. Conclusion Non-invasive imaging can detect differences between affected and non-affected skin in JLS and may help to differentiate between activity (thicker, less well-perfused skin) and damage (thinner, highly perfused skin). [ABSTRACT FROM AUTHOR]

To evaluate the long-term efficacy of methotrexate (MTX) monotherapy in patients with juvenile idiopathic arthritis-associated uveitis (JIA-U). We analyzed a cohort of patients with JIA-U treated with MTX monotherapy, divided into two groups depending on whether MTX was started before (on-MTX group) or after uveitis diagnosis (MTX-naïve group). The primary endpoint was the time between uveitis inactivity and first relapse. 84 patients entered the study. The median duration of remission on MTX monotherapy resulted 8.2 months. The on-MTX group showed a significant longer time interval between arthritis and uveitis onset and higher need for biologic agents (bDMARD). During follow-up, 40 patients (47.6%) needed bDMARD due to poor control of uveitis. Clinical remission off medication was achieved in 11.9% of patients, all belonging to the MTX-naïve group. MTX monotherapy, although effective in early stages of JIA-U, showed poor disease control in the long term. [ABSTRACT FROM AUTHOR]