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Introduction: Although familial Mediterranean fever (FMF) is a relevant disease in countries surrounding the Mediterranean Sea, there are still few reports from Italy., Methods: We retrospectively evaluated patients with FMF diagnosed according to the EuroFever/PRINTO classification criteria in three pediatric rheumatology referral centers in central-southern Italy. Logistic regression analysis assessed the associations between age at disease onset and symptoms., Results: Overall, 48 patients were enrolled (28 females, 20 males), with a median age at onset of 3.3 [3.1] years, and a median follow-up period of 5.1 [10.8] years. The most common MEFV genotype was M694V/- (11 patients, 22.9%), followed by M694V/M694V (6 patients, 12.5%). At onset, recurrent fever was observed in 47 patients (97.9%), with a median time between attacks of 18 [11] days. Overall, recurrent fever was observed in all patients, abdominal pain in 44 (91.7%), and chest pain in 18 (37.5%). At the last follow-up visit, 24 patients were on colchicine (50%), 2 on biologic (4.2%), and 6 on both (12.5%). Canakinumab was the most used biologic drug, in 6 (12.5%) patients. MEFV genotype was associated with disease severity (p = 0.007) and the use of a biological drug (p = 0.01). FMF prevalence in the Abruzzo region was found highly than expected (at least 1:45,000). Differently, we found a relevant gap among FMF patients expected and observed in the Apulia and Sicily regions., Conclusions: FMF is a relevant issue in central-southern Italy. A large epidemiologic study should be performed to better define its prevalence in the country. Key Points • Italian children with familial Mediterranean fever tend to have an early age of onset, primarily manifesting with recurrent fever and characteristic associated symptoms. • Many MEFV gene variants are present in Italian children with familial Mediterranean fever, and these patients are most often heterozygous, exhibiting a mild to moderate phenotype. • The prevalence of familial Mediterranean fever in Italy is still unknown but recently estimated to be around 1:60,000, probably higher in central and southern Italy. • According to our cohort, the prevalence of FMF in the Abruzzo region is at least 1:45,000, higher than expected. Differently, we found lower prevalence rates of the disease in Apulia and Sicily., (© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

The Artificial Intelligence (AI) research community has used ad-hoc benchmarks to measure the " intelligence " level of Large Language Models (LLMs). In humans, intelligence is closely linked to the functional integrity of the prefrontal lobes, which are essential for higher-order cognitive processes. Previous research has found that LLMs struggle with cognitive tasks that rely on these prefrontal functions, highlighting a significant challenge in replicating human-like intelligence. In December 2022, OpenAI released ChatGPT, a new chatbot based on the GPT-3.5 model that quickly gained popularity for its impressive ability to understand and respond to human instructions, suggesting a significant step towards intelligent behaviour in AI. Therefore, to rigorously investigate LLMs' level of " intelligence ," we evaluated the GPT-3.5 and GPT-4 versions through a neuropsychological assessment using tests in the Italian language routinely employed to assess prefrontal functioning in humans. The same tests were also administered to Claude2 and Llama2 to verify whether similar language models perform similarly in prefrontal tests. When using human performance as a reference, GPT-3.5 showed inhomogeneous results on prefrontal tests, with some tests well above average, others in the lower range, and others frankly impaired. Specifically, we have identified poor planning abilities and difficulty in recognising semantic absurdities and understanding others' intentions and mental states. Claude2 exhibited a similar pattern to GPT-3.5, while Llama2 performed poorly in almost all tests. These inconsistent profiles highlight how LLMs' emergent abilities do not yet mimic human cognitive functioning. The sole exception was GPT-4, which performed within the normative range for all the tasks except planning. Furthermore, we showed how standardised neuropsychological batteries developed to assess human cognitive functions may be suitable for challenging LLMs' performance., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Author(s).)

Objective: Inflammation has been associated with an increased risk for cancer development, while innate immune system activation could counteract the risk for malignancies. Familial Mediterranean fever (FMF) is a severe systemic inflammatory condition and also represents the archetype of innate immunity deregulation. Therefore, the aim of this study is to investigate the risk for cancer development in FMF., Methods: The risk ratio (RR) for malignancies was separately compared between FMF patients and fibromyalgia subjects, Still's disease patients and Behçet's disease patients. Clinical variables associated with cancer development in FMF patients were searched through binary logistic regression., Results: 580 FMF patients and 102 fibromyalgia subjects, 1012 Behçet's disease patients and 497 Still's disease patients were enrolled. The RR for the occurrence of malignant neoplasms was 0.26 (95% Confidence Interval [CI.] 0.10-0.73, p=0.006) in patients with FMF compared to fibromyalgia subjects; the RR for the occurrence of malignant cancer was 0.51 (95% CI. 0.23-1.16, p =0.10) in FMF compared to Still's disease and 0.60 (95% CI. 0.29-1.28, p =0.18) in FMF compared to Behçet's disease. At logistic regression, the risk of occurrence of malignant neoplasms in FMF patients was associated with the age at disease onset (β1 = 0.039, 95% CI. 0.001-0.071, p =0.02), the age at the diagnosis (β1 = 0.048, 95% CI. 0.039-0.085, p =0.006), the age at the enrolment (β1 = 0.05, 95% CI. 0.007-0.068, p =0.01), the number of attacks per year (β1 = 0.011, 95% CI. 0.001- 0.019, p =0.008), the use of biotechnological agents (β1 = 1.77, 95% CI. 0.43-3.19, p =0.009), the use of anti-IL-1 agents (β1 = 2.089, 95% CI. 0.7-3.5, p =0.002)., Conclusions: The risk for cancer is reduced in Caucasic FMF patients; however, when malignant neoplasms occur, this is more frequent in FMF cases suffering from a severe disease phenotype and presenting a colchicine-resistant disease., Competing Interests: The 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 © 2024 Vitale, Caggiano, Tufan, Ragab, Batu, Portincasa, Aragona, Sota, Conti, De Paulis, Rigante, Olivieri, Şahin, La Torre, Lopalco, Cattalini, Maggio, Insalaco, Sfikakis, Verrecchia, Yildirim, Kucuk, Kardas, Laymouna, Ghanema, Saad, Sener, Ercan Emreol, Ozen, Jaber, Khalil, Di Ciaula, Gaggiano, Malizia, Affronti, Patroniti, Romeo, Sbalchiero, Della Casa, Mormile, Silvaroli, Gicchino, Çelik, Tarsia, Karamanakos, Hernández-Rodríguez, Parronchi, Opris-Belinski, Barone, Recke, Costi, Sfriso, Giardini, Gentileschi, Wiesik-Szewczyk, Vasi, Loconte, Jahnz-Różyk, Martín-Nares, Torres-Ruiz, Cauli, Conforti, Emmi, Li Gobbi, Biasi, Terribili, Ruscitti, Del Giudice, Tharwat, Brucato, Ogunjimi, Hinojosa-Azaola, Balistreri, Fabiani, Frediani and Cantarini.)

Human accuracy in detecting deception with intuitive judgments has been proven to not go above the chance level. Therefore, several automatized verbal lie detection techniques employing Machine Learning and Transformer models have been developed to reach higher levels of accuracy. This study is the first to explore the performance of a Large Language Model, FLAN-T5 (small and base sizes), in a lie-detection classification task in three English-language datasets encompassing personal opinions, autobiographical memories, and future intentions. After performing stylometric analysis to describe linguistic differences in the three datasets, we tested the small- and base-sized FLAN-T5 in three Scenarios using 10-fold cross-validation: one with train and test set coming from the same single dataset, one with train set coming from two datasets and the test set coming from the third remaining dataset, one with train and test set coming from all the three datasets. We reached state-of-the-art results in Scenarios 1 and 3, outperforming previous benchmarks. The results revealed also that model performance depended on model size, with larger models exhibiting higher performance. Furthermore, stylometric analysis was performed to carry out explainability analysis, finding that linguistic features associated with the Cognitive Load framework may influence the model's predictions., (© 2023. The Author(s).)

Introduction: The effectiveness of canakinumab may change according to the different times it is used after Still's disease onset. This study aimed to investigate whether canakinumab (CAN) shows differences in short- and long-term therapeutic outcomes, according to its use as different lines of biologic treatment., Methods: Patients included in this study were retrospectively enrolled from the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Still's disease. Seventy-seven (51 females and 26 males) patients with Still's disease were included in the present study. In total, 39 (50.6%) patients underwent CAN as a first-line biologic agent, and the remaining 38 (49.4%) patients were treated with CAN as a second-line biologic agent or subsequent biologic agent., Results: No statistically significant differences were found between patients treated with CAN as a first-line biologic agent and those previously treated with other biologic agents in terms of the frequency of complete response ( p  =0.62), partial response ( p  =0.61), treatment failure ( p  >0.99), and frequency of patients discontinuing CAN due to lack or loss of efficacy ( p  =0.2). Of all the patients, 18 (23.4%) patients experienced disease relapse during canakinumab treatment, 9 patients were treated with canakinumab as a first-line biologic agent, and nine patients were treated with a second-line or subsequent biologic agent. No differences were found in the frequency of glucocorticoid use ( p  =0.34), daily glucocorticoid dosage ( p  =0.47), or concomitant methotrexate dosage ( p  =0.43) at the last assessment during CAN treatment., Conclusion: Canakinumab has proved to be effective in patients with Still's disease, regardless of its line of biologic treatment., Competing Interests: The 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 Vitale, Caggiano, Sfikakis, Dagna, Lopalco, Ragab, La Torre, Almaghlouth, Maggio, Sota, Tufan, Hinojosa-Azaola, Iannone, Loconte, Laskari, Direskeneli, Ruscitti, Morrone, Mayrink Giardini, Panagiotopoulos, Di Cola, Martín-Nares, Monti, De Stefano, Kardas, Duran, Campochiaro, Tomelleri, Alabdulkareem, Gaggiano, Tarsia, Bartoloni, Romeo, Hussein, Laymouna, Parente de Brito Antonelli, Dagostin, Fotis, Bindoli, Navarini, Alibaz-Oner, Sevik, Frassi, Ciccia, Iacono, Crisafulli, Portincasa, Jaber, Kawakami-Campos, Wiesik-Szewczyk, Iagnocco, Simonini, Sfriso, Balistreri, Giacomelli, Conti, Frediani, Fabiani and Cantarini.)

Objective: To evaluate the potential role of Streptococcus salivarius K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction., Patients and Methods: The medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00 ± 7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00 ± 28.00 months., Results: The number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p < 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [ p < 0.001]. Similarly, the highest temperature in°C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p < 0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p < 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis ( p < 0.001), oral aphthae ( p < 0.001) and cervical lymphadenopathy ( p < 0.001) significantly decreased following SSK12., Conclusion: SSK12 prophylaxis given for at least 6.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the duration of the single febrile episode, lowered body temperature by 1°C in the febrile flare, provided a steroid-sparing effect, and significantly reduced the accompanying symptoms related to the syndrome., Competing Interests: The 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 La Torre, Sota, Insalaco, Conti, Del Giudice, Lubrano, Breda, Maggio, Civino, Mastrorilli, Loconte, Natale, Celani, Romeo, Patroniti, Gentile, Vitale, Caggiano, Gaggiano, Diomeda, Cattalini, Lopalco, Emmi, Parronchi, Gentileschi, Cardinale, Aragona, Shahram, Marino, Barone, Moscheo, Ozkiziltas, Carubbi, Alahmed, Iezzi, Ogunjimi, Mauro, Tarsia, Mahmoud, Giardini, Sfikakis, Laskari, Więsik-Szewczyk, Hernández-Rodríguez, Frediani, Gómez-Caverzaschi, Tufan, Almaghlouth, Balistreri, Ragab, Fabiani, Cantarini and Rigante.)

The accurate recognition of others' facial expressions is a core skill for social interactions. The left Dorsolateral Prefrontal Cortex (L-DLPFC) represents a key node in the network for facial emotion recognition. However, its specific role is still under debate. As such, the aim of the current neuromodulation study was to assess the causal role of the L-DLPFC in humans' rating of facial expressions of emotions and implicit attitudes toward other races. In this sham-controlled single-blind between-subject experiment, we offline administered L-DLPFC transcranial direct current stimulation (tDCS) to 69 healthy participants who were divided into three groups of 23 (each receiving anodal 1 mA tDCS, anodal 2 mA tDCS, or Sham), before completing an "Emotion Rating task and two Implicit Association Tests (IATs). The former required the intensity rating of 192 faces (half black and half white) displaying happiness, sadness, anger, or fear. The IATs were designed to assess participants' automatic associations of positive or negative attributes with racial contents. Results on the Emotion Rating task showed participants' gender-specific effect of tDCS. Specifically, a gender bias, with only males showing a tendency to underestimate negative emotions was found in Sham, and absent in the tDCS groups. When considering the race of the stimuli, females but not males in Sham exhibited a racial bias , that is, the tendency to overestimate negative emotions of other-race faces. Again, the bias disappeared in the tDCS groups. Concerning the IATs, no significant effects emerged. We conclude that the L-DLPFC plays a critical role in humans' rating of facial expressions, and for variability in other-race emotional judgements. These results shed light on the neural bases of the human emotional system and its gender-related differences, and have potential implications for interventional settings., Competing Interests: The authors declare no conflict of interest., (© 2021 Published by Elsevier Ltd.)