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:Monogenic auto-inflammatory diseases (mAID) are a group of severe chronic multisystemic diseases with recurring episodes of fever and other manifestations that significantly affect the patients’ life quality. Moreover, the hyper expression of pro-inflammatory cytokines (IL1β, etc.) observed in these patients may have a negative effect on the central nervous system.Objectives:to study the state of the cognitive and emotional spheres in children suffering from monogenic auto-inflammatory diseases.Methods:there were examined 22 children at the age of 7 to 17 years old diagnosed with CAPS-9, TRAPS-8, FMF-5. Among them there were 12 boys and 10 girls. The diagnosis in all the patients was confirmed through detection of pathogenic mutations in the NLRP3, TNFRSF1A and MEFV genes. The following methods were used: a clinical conversation; memory diagnostics (learning by heart of 10 words, a pictogram using cues taking into account the patients’ age); attention diagnostics (Schulte tables); thinking diagnostics (establishing a sequence of events, “four is a crwod”, simple analogies, interpretation of proverbs); emotional and communicative fields (the Eight-Color Luscher Test; CMAS (adaptation by A. Prikhozhan); STAI test, a drawing called “an animal that does not exist” and “a house-a tree-a man”).Results:The memory study revealed in all patients with TRAPS and FMF high and medium values of short-term and long-term memory, in patients with CAPS - a low level of short-term auditory-speech memory, information storage and indirect memorization in 1/3 of patients. In 100% of the examined patients with TRAPS, a significant decrease in all processes of attention and distribution of attention. In 1/3 of patients with CAPS, an increased exhaustion of attention was registered and in 11% - a decrease in its stability. In patients with FMF, attention disorders were not detected. In 44% of patients with CAPS, a decrease in the level of generalization and difficulties in establishing causal relationships were registered. In 25% of patients with TRAPS a decrease in the level of generalization, in 12.5%- difficulties in establishing cause-effect relationships, inertia of thinking in 37.5%. In 60% of patients with FMF: a decrease in the level of generalization, in 80%: difficulties in establishing cause-effect relationships, inertia of thinking in 20%. In the emotional sphere, patients with CAPS, TRAPS, and FMF demonstrated signs of aggression (11.1%, 20% and 20% of patients, respectively), communicative disorders (77.8% -80% - 80%), and reduced social adaptation (55.5% - 80% - 80%), a tendency to form neurotic fears (22% - 40% - 40%). A high level of personal anxiety was noted in 1/3 of patients with CAPS and 40% of patients with FMF.Conclusion:various psychological disorders in the cognitive and emotional fields were noted in the majority of the examined patients with monogenic auto-inflammatory diseases. In patients with TRAPS, attention processes are most significantly affected; in patients with CAPS, memory is more often affected. In patients with FMF, disorders in thinking processes are revealed more often. In the emotional sphere, most patients with all the three forms of AID note communicative disorders and social adaptation.Disclosure of Interests:None declared

П е д и а т р и ч е с к а я р е в м а т о л о г и я ФГБНУ «Научноисследовательский институт ревматологии им. В.А. Насоновой»,

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