Introduction: In 2015 the historic Jones criteria for the diagnosis of Acute Rheumatic Fever (ARF) were revised introducing two different sets of criteria for low-risk and for moderate/high-risk populations (according to ARF incidence). In Italy the exact ARF incidence is unknown but small regional or local reports suggest an incidence of 2-5/100.000 per year, suggesting that our population might be considered at moderate risk for ARF. Objectives: To evaluate the performance of the revised Jones criteria in a retrospective population and to compare it with the performance of the previous version of Jones criteria. Methods: We conducted a retrospective study on 288 patients with ARF (108 female; median age 8.5 years, IQR 7.1-10.3) diagnosed from 2001 to 2015 in a Pediatric Rheumatology Division by pediatric rheumatologists, discharged with an ICD 9 code consistent with ARF. We retrospectively applied the two sets (for low-risk and for moderate/high-risk) of the 2015 revised Jones criteria and the 1992 version of the Jones criteria. Results: Of 288 patients, 253 (87.8%) met the 1992 version of the Jones criteria, 237 (82.3%) met the revised criteria for low-risk populations and 259 (89.9%) for moderate/high-risk populations. None of these differences was significant. Prevalence of major and minor criteria is shown in Table. With the exception of difference in arthritis, the 1992 version and the 2015 revised version did not show major differences. Of the 288 patients with a clinical diagnosis of ARF 29 did not meet any version of the Jones criteria. Patients in this group presented with isolated chorea or silent carditis without other manifestations. Prevalence of the clinical characteristics and comparison among the 1992 version of Jones criteria and the 2015 revised Jones criteria (low risk and moderate-high risk populations): Values are expressed in Number (percentage). *p value (Fisher Exact test) Conclusion: The revised Jones criteria for low-risk populations are slightly more sensitive than the 1992 version of Jones criteria, while the revised Jones criteria for moderate/high populations are slightly less sensitive than the 1992 version. In this population, the revised criteria did not substantially modify the diagnosis of ARF. Approximately 10% of patients presented with isolated chorea or silent carditis.
Background Site reactions are the most common but the least serious events of biological treatment. However, the first signs of the serious adverse events may be skin manifestations. Objectives The aim of the prospective study was to evaluate the skin manifestations in children treated with anti-TNF drugs in the center for biological treatment of rheumatic and skin diseases in Eastern Slovakia. Methods A group of thirty one pediatric patients receiving anti-TNF treatment (etanercept or adalimumab) for juvenile idiopathic arthritis (JIA) or psoriasis was evaluated in the period from May 2006 to December 2011. Patients were recruited primarily from the rheumatology department (15 girls and 11 boys). Parameters of efficacy and safety were evaluated every 3 months after therapy initiation. The mean age at initiation was 14.1 years (SD ± 3.9). Average duration of treatment was 3.04 years (range 0.32 – 5.67), 94.24 patient-years in total. Results In the subgroup of JIA patients, four patients developed five different skin manifestations more than 1 year after the initiation. All patients were confirmed for polyarticular seronegative form and ACR-Ped70 responders to etanercept.All underwent a dermatological examination. Three cases were assessed as adverse events. The first patient developed a HPV infection, the second one Varicella and the third one developed both Varicella and mycotic lesions on the face and neck. After temporary discontinuation of anti-TNF drug, skin lesions healed with systemic and topical treatment. All patients continued with anti-TNF treatment thereafter. One case was assessed as a serious adverse event. 13 year-old girl developed Henoch-Schönlein purpura 14 months after initiation of anti-TNF treatment. Although a causative relationship could not be confirmed, anti-TNF treatment was stoped. In the subgroup of psoriasis patients skin-manifested adverse event was observed as well. 15 year-old boy developed histologically verified scleroderma-like lesions 13 months after initiation of etanercept with excellent therapeutic response to the underlying disease. It was assessed as serious adverse event and therefore, anti-TNF treatment was stoped. Conclusions Immunologically mediated cutaneous manifestations could be considered as potential serious adverse events of the anti-TNF treatment although a clear causative relationship can not be confirmed. Careful monitoring of the patients and close cooperation of pediatric rheumatologist and dermatologist is a prerequisite for an early recognition of any infectious or non-infectious skin-manifested complications of anti-TNF drugs and for its treatment. More data possibly from national and international pharmacovigilance registries are necessary to evaluate the actual risk of complications in patients on anti-TNF treatment. Disclosure of Interest None Declared
The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Slovak language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach’s alpha, interscale correlations, test–retest reliability, and construct validity (convergent and discriminant validity). A total of 108 JIA patients (5.6% systemic, 38.9% oligoarticular, 30.5% RF-negative polyarthritis, 25% other categories) and 100 healthy children were enrolled in two centres. Notably, none of the enrolled JIA patients is affected with psoriatic arthritis. The JAMAR components discriminated healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Slovak version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.
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