Andrea Taddio

The aim of this study is to describe characteristics of children with the International League of Associations for Rheumatology (ILAR)-defined juvenile idiopathic arthritis (JPsA) and to assess whether more sensitive and specific criteria for defining JPsA can be identified. A retrospective observational multicentre study was conducted including patients with a diagnosis of JPsA according to ILAR criteria. In this population, we identified clusters using as variables the clinical criteria of JPsA according to ILAR and the CASPAR clinical criteria. In addition, we defined as undifferentiated arthritis patients that met the ERA criteria, as defined by ILAR, and presented psoriatic features such as psoriasis or a history of psoriasis or psoriatic arthritis in a first-degree relative. 73 patients were enrolled. Three clinical clusters were found using unsupervised principal component analysis for the patients. Cluster 1 differed significantly for older patients and psoriasis. In contrast, Cluster 2 was mainly characterised by dactylitis and Cluster 3 was defined by family history of psoriasis and a significant prevalence of dactylitis. We also showed a statistically significant presence of familiarity for psoriatic arthritis in Cluster 2. The significance for all parameters evaluated did not change even when we included the patients with undifferentiated arthritis, except for MTX treatment, which was significantly more common in Cluster 2 (p=0.02), and tenosynovitis, also in Cluster 2 (p=0.05). Moreover, we evaluated our cohort by the Vancouver criteria. Combining the ILAR, CASPAR, and Vancouver criteria only two patients remain undifferentiated. Our study showed three clinical clusters with diverse demographic and clinical characteristics, indicating JPsA heterogeneity. The findings highlight the need to look beyond ILAR criteria for clinical variables, including family history of psoriatic arthritis. The ILAR, CASPAR, and Vancouver criteria improve the diagnosis of paediatric psoriatic spectrum arthritis. This complex disease population needs larger cohorts and clinical data, especially on axial involvement, to better categorisation and treatment.

Anakinra is a recombinant human interleukin-1 receptor antagonist primarily administered by subcutaneous injection for the treatment of autoinflammatory co

Background: Kawasaki disease (KD) is a systemic vasculitis of medium arteries, particularly involving coronary arteries. Coronary artery lesions (CALs) is the most serious complication in the acute stage, potentially leading to ischemic cardiomyopathy, myocardial infarction and sudden death. Environmental factors and genetic background contribute to individual susceptibility to develop CALs. The aim of this study was to define the risk factors for CALs in an Italian cohort. Methods: Data of KD patients from 10 Italian sites were registered into a REDCap database where demographic and clinical data, laboratory findings and coronary status were recorded. KD was diagnosed according to AHA definition. We used multiple logistic regression analysis to identify independent risk factors for CALs. Results: A total of 517 patients were enrolled, mainly Caucasians (83.6%). Presentation was complete in 321 patients (62.8%) and IVIG responsiveness in 360 (70%). CALs developed in 136/517 (26.31%). Gender, age, ethnicity, clinical presentation, fever duration, non-coronary cardiac events, Hb, albumin and CRP were significantly different between patients with and without CALs, while seasonality was not. Male gender, age < 18 months, Asian ethnicity, incomplete presentation and fever > 10 days were independent risk factors for CALs. Conclusions: Age younger than 18 months, incomplete KD and longer fever duration are risk factors for CALs. Asian ethnicity also represents a risk factor in our Italian Cohort.

Background:The definition of psoriatic arthritis in pediatrics (JPsA) is controversial. The study purpose to describe clinical and laboratory characteristics and responses to therapies in children with psoriatic arthropathies. Methods: A retrospective multicentre observational study enrolling subjects meeting the ILAR criteria for juvenile psoriatic arthritis. We also included subjects meeting the enthesitis-related arthritis criteria, except for the presence of psoriasis and/or family history of psoriasis or psoriatic arthritis in a first-degree relative. Disease remission was defined according to the Wallace criteria. Results: 71 patients (44 females, 27 males). Disease onset was before 10 years in 32 patients (median 5.5 years) and after 10 years (median 13 years) in 39. Inflammation indexes and antinuclear antibodies were raised, respectively, in 61% and 47% of the tested patients. Twenty patients (28.2%) got clinical remission with intra-articular glucocorticoids. Eighteen patients (25.3%) with synthetic disease-modifying anti-rheumatic drugs got better, and 33 patients (46.5%) with biological DMARDs. Two patients presented with intractable uveitis. Fifty-five patients (77.5%) met the ILAR criteria for JPsA; eight were HLA B27+ males with disease onset over 6 years of age, and eight had arthritis and enthesitis. Four clinical patterns were found using unsupervised principal component analysis. One of them was mostly made up of early-onset oligoarticular arthiritis, dactylitis, tenosynovitis, and uveitis. The two groups occurring after 10 years of age were more similar to adult-onset psoriatic arthritis or undifferentiated spondyloarthropaties. Conclusions: Post-pubertal clusters of JPsA are similar to typical adult PsA, while peculiar clusters are identified in younger children.

Background: Limping is a common clinical symptom in childhood; different clinical conditions may lead to limping and the diagnosis of the underlying cause may often be a challenge for the pediatrician. Case presentation: We describe the clinical manifestations, radiological pictures and disease course of other causes of limping in childhood, through a case series of seven cases and a brief discussion of each disease. Conclusions: although trauma is the most common cause of acute limping, when there is no history of traumatic events and the limping has a chronic course, Juvenile Idiopathic Arthritis is usually the most likely clinical diagnosis. However, other some rare conditions should be taken into account if JIA is not confirmed or if it presents with atypical clinical picture.

The main aim of this study was to define the best treatment option for multisystem inflammatory syndrome in children (MIS-C) and to analyze the role of anakinra. This is a multicentre retrospective cohort study. Patients were treated according to the attending physician’s decision. The patients were divided in 4 groups on the basis of the first treatment at time of admittance: i) intravenous immunoglobulins (IVIG), ii) IVIG and methylprednisolone (≤ 2 mg/kg/day), iii) IVIG with high dose methylprednisolone (>2 mg/kg/day) and iv) anakinra with or without IVIG and/or methylprednisolone. Primary outcomes were defined as the presence of at least one of the following features: death, the failure of initial treatment, meaning the need of additional treatment for clinical worsening and cardiac involvement at the end of follow-up. Two hundred thirty-nine patients were recruited. At univariate analysis, persistent heart involvement at discharge was more frequent in those not receiving anakinra as initial treatment (3/21 vs 66/189; p= 0.047). After comparison of the 4 treatment regimens adjusting for the propensity score, we observed that early treatment with anakinra was associated with a lower probability to develop persistent heart disease at the end of follow-up (OR: 0.6; 95% CI: 0.4–1.0). We report that early treatment with anakinra is safe and very effective in patients with severe MIS-C. In addition, our study suggests that early treatment with anakinra is the most favorable option for patients with a higher risk to develop a severe disease outcome.

Multisystem inflammatory syndrome in children (MIS-C) is defined as a clinically serious condition requiring hospitalization involving fever, multi-system organ dysfunction, and an increase in inflammatory biomarkers. The syndrome was originally described as a post-infectious complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, which usually causes COVID-19. During the COVID-19 pandemic, not only did the virus undergo mutations but vaccines against SARS-CoV-2 were also developed. Both these conditions led to a decrease in the incidence of MIS-C. This narrative review summarizes the recent updates for MIS-C, particularly regarding the change in incidence, the link between the SARS-CoV-2 vaccine and MIS-C, and new updates of MIS-C treatments.

Article: Final adult height in childhood-onset inflammatory bowel disease - Minerva Pediatrics 2023 June;75(3):354-7 - Minerva Medica - Journals