Autism and intention attribution test: a non-verbal evaluation with comic strips.

BACKGROUND: Despite autism spectrum disorder (ASD) and mentalization being two words often associated in the literature, the assessment of this ability in individuals with ASD in the clinical setting is still limited. Indeed, there are no standardized Theory of Mind (ToM) tests that are adaptable to different cognitive profiles, such as individuals with language poverty, and intellectual or memory impairments. This study proposes a non-verbal test (Intentions Attribution-Comic Strip Test; IA-CST) to evaluate the ability to infer the intentions of others, a basic component of ToM, in the clinical setting. METHOD: In Study 1, the test was administered to 261 healthy individuals and we performed structural validation using Exploratory Graph Analysis. In Study 2, the final version of the test was administered to 32 individuals with ASD to assess the known group validity of the measure by comparing their scores with a sample of IQ-matched controls. Moreover, we performed logistic regression and ROC curve to preliminarily assess the diagnostic performance of the IA-CST. RESULTS: The IA-CST resulted in a 3-dimension measure with good structural stability. Group comparison indicated that the ASD group shows significantly lower performance in intention attribution but not in inferring causal consequences. The test demonstrated known group validity and that, preliminarily, it is suitable for implementation within the clinical practice. CONCLUSIONS: The results support the IA-CST as a valid non-verbal task for evaluating intentions attribution in the clinical setting. Difficulties in ToM are early and relevant in ASD, so assessing these aspects is valuable for structuring individualized and evidence-based interventions.

On the Evaluation of Engagement in Immersive Applications When Users Are on the Autism Spectrum.

New generation wearable devices allow for the development of interactive environments tailored for Virtual Reality (VR)- and Augmented Reality (AR)-based treatment of Autism Spectrum Disorders (ASD). Experts agree on their potential; however, there is lack of consensus on how to perform trials and the need arises for evaluation frameworks, methods, and techniques appropriate for the ASD population. In this paper, we report on a study conducted with high-functioning ASD people in the 21-23 age range, with the objectives of (1) evaluating the engagement of two headsets offering distinct immersive experiences, (2) reasoning on the interpretation of engagement factors in the case of ASD people, and (3) translating results into general guidelines for the development of VR/AR-based ASD treatment. To this aim, we (1) designed two engagement evaluation frameworks based on behavioral observation measures, (2) set up two packages of reference immersive scenarios, (3) defined the association between metrics and scenarios, and (4) administered the scenarios in distinct sessions for the investigated headsets. Results show that the immersive experiences are engaging and that the apparent lack of success of some evaluation factors can become potential advantages within the framework of VR/AR-based ASD treatment design.

Survival analysis up to 7 years of 621 zirconia monolithic single crowns with feather-edge margins fabricated with a cast-free workflow starting from intraoral scans: A multicentric retrospective study.

STATEMENT OF PROBLEM: Clinical studies on the fabrication of monolithic zirconia restorations with a feather-edge tooth preparation from digital scans and a cast-free fully digital workflow are lacking. PURPOSE: The purpose of this retrospective multicentric study in private practices was to evaluate the outcomes of monolithic zirconia crowns fabricated with feather-edge margins and a cast-free approach. MATERIAL AND METHODS: A total of 621 teeth were prepared with feather-edge margins and restored with monolithic zirconia crowns fabricated with a fully digital cast-free workflow. Data were analyzed by using the Kaplan-Meier test and descriptive statistics. The clinical evaluation adopted the California Dental Association-modified criteria after recalling all patients between April and July 2021. RESULTS: The clinical survival of 619 of 621 crowns, including recemented crowns placed in 427 patients (217 men, 220 women) over 5 years (2014 to 2019 with crowns in service between 12 and 85 months), was analyzed. The 2 excluded crowns were delivered to patients who dropped out of the study. Of the 619 crowns, 5 failed during the follow-up period: 4 teeth were extracted because of fracture and 1 restoration fractured. No other technical or biological failures were observed. The mean overall survival time was 84.4 months (standard error, 0.255; 95% confidence interval for the mean, 83.92 to 84.92). The overall survival probability was 99.1% up to 85 months. CONCLUSIONS: The clinical outcomes of the monolithic zirconia crowns with feather-edge margins evaluated were comparable with outcomes reported using other margin designs and materials.

A digital protocol for the fabrication of anatomic-contour implant zirconia crowns directly screwed on external hexagonal implant connections without the interposition of a Ti-base component.

A straightforward and effective restorative option is proposed for implant-supported fixed prostheses with external connections by using monolithic zirconia without the interposition of a Ti-base component. The technique is based on a modification of the Brånemark connection used to link metal-ceramic or metal-composite resin restorations directly to the implant.

A diagnostically and digitally driven tooth preparation protocol by using a patient monitoring tool with an intraoral scanner.

Finding the right balance between the preservation of tooth structure and providing adequate space for the restorative material is a major challenge in prosthetic dentistry. A technique is presented using the patient monitoring tool available in standard software programs of an intraoral scanner to constantly monitor preparation dimensions in relation to the optimal definitive restoration.

Cytokine profile, ferritin and multi-visceral involvement characterize macrophage activation syndrome during adult-onset Still's disease.

OBJECTIVES: To multidimensionally characterize macrophage activation syndrome (MAS) complicating adult-onset Still's disease (AOSD) considering cytokine profile, inflammatory markers and multi-visceral involvement of the disease. To perform a high-dimensional phenotypic analysis of circulating immune cells in AOSD patients with and without MAS. To assess interferon (IFN)-related pathways in AOSD synovial tissues by a bulky RNA sequencing. METHODS: Clinical and biologic data were collected and compared in AOSD patients with and without MAS. Sera biomolecules were analysed by Luminex multiplexing technology. Mass cytometry (CyTOF) was used to characterize circulating immune cells. A bulky RNA sequencing was performed in AOSD synovial tissues. RESULTS: Forty consecutive AOSD patients were assessed, 14 complicated with MAS. Paralleling with increases of systemic score and ferritin, MAS patients showed higher levels of IL-1α, IL-1ß, IL-1Ra, IL-2Ra, IL-6, IL-10, IL-17A, IFN-γ, G-CSF, MCP-1, MIP-1α and SCF. Combining the discriminatory ability of these data in identifying MAS, the best model was composed by systemic score, ferritin, IFN-γ and IL-10. By CyTOF analysis, MAS patients showed an increase of circulating 'classical monocytes' and a reduction of total NK cells. Our assessment showed 3477 IFN-related genes (IRGs) were differently expressed in AOSD synovial tissues. CONCLUSIONS: A multidimensional characterization of AOSD patients suggested that IFN-γ, IL-10, ferritin and systemic score discriminated the occurrence of cytokine storm syndrome associated with MAS. The inflammatory milieu of AOSD and MAS may be related to a signature of circulating immune cells. Finally, our results about IRGs reinforced the role of IFN-γ in these patients.

Circulating tumour cell gene expression and chemosensitivity analyses: predictive accuracy for response to multidisciplinary treatment of patients with unresectable refractory recurrent rectal cancer or unresectable refractory colorectal cancer liver metastases.

BACKGROUND: Patients with unresectable recurrent rectal cancer (RRC) or colorectal cancer (CRC) with liver metastases, refractory to at least two lines of traditional systemic therapy, may receive third line intraarterial chemotherapy (IC) and targeted therapy (TT) using drugs selected by chemosensitivity and tumor gene expression analyses of liquid biopsy-derived circulating tumor cells (CTCs). METHODS: In this retrospective study, 36 patients with refractory unresectable RRC or refractory unresectable CRC liver metastases were submitted for IC and TT with agents selected by precision oncotherapy chemosensitivity assays performed on liquid biopsy-derived CTCs, transiently cultured in vitro, and by tumor gene expression in the same CTC population, as a ratio to tumor gene expression in peripheral mononuclear blood cells (PMBCs) from the same individual. The endpoint was to evaluate the predictive accuracy of a specific liquid biopsy precision oncotherapy CTC purification and in vitro culture methodology for a positive RECIST 1.1 response to the therapy selected. RESULTS: Our analyses resulted in evaluations of 94.12% (95% CI 0.71-0.99) for sensitivity, 5.26% (95% CI 0.01-0.26) for specificity, a predictive value of 47.06% (95% CI 0.29-0.65) for a positive response, a predictive value of 50% (95% CI 0.01-0.98) for a negative response, with an overall calculated predictive accuracy of 47.22% (95% CI 0.30-0.64). CONCLUSIONS: This is the first reported estimation of predictive accuracy derived from combining chemosensitivity and tumor gene expression analyses on liquid biopsy-derived CTCs, transiently cultured in vitro which, despite limitations, represents a baseline and benchmark which we envisage will be improve upon by methodological and technological advances and future clinical trials.

Are Autism Spectrum Disorder and Asexuality Connected?

Asexuality is a lack of sexual attraction to any gender. There is some evidence to suggest that many self-identified asexuals have a formal diagnosis of autism spectrum disorder which is characterized by deficits in social interaction and communication, as well as by restricted and repetitive interests and behaviors. Additionally, the literature shows that asexuality and lack of sexual attraction or low sexual interest is overrepresented in people with autism spectrum disorder compared with neurotypical samples. Nevertheless, no studies have been conducted to investigate the relationship between autism and asexuality in depth. We conducted a systematic review of the literature to examine whether asexuality and autism spectrum disorder are connected. We conclude that asexuality and autism share various aspects, such as a possible role of prenatal factors, reference to romantic dimensions of sexual attraction and sexual orientation, and non-partner-oriented sexual desire, but future research should explore and clarify this link.

Using the Griffiths Mental Development Scales to Evaluate a Developmental Profile of Children with Autism Spectrum Disorder and Their Symptomatologic Severity.

Early diagnosis is crucial for Autism spectrum disorder (ASD) and is achieved through a screening of developmental indicators to recognise children who are at risk of autism. One of the most widely used instruments in clinical practice for assessing child development is the Griffiths Mental Development Scale (GMDS). We sought (a) to assess longitudinally whether children diagnosed with ASD, with a mean age of 33.50 months (SD 7.69 months), show a developmental delay of abilities measured by the GMDS over time and (b) to analyse which skills of the GMDS could be associate to the symptomatologic severity of ASD. Our results showed lower scores of General Quotient and all sub-quotients of GMDS from first (T0) to second assessment (T1), except for the Performance sub-quotient. Three sub-quotients (Personal-Social, Hearing and Language and Practical Reasoning) also associate symptom severity at the time when the diagnosis of ASD is made.

Real-world clinical validity of cardiac magnetic resonance tissue tracking in primitive hypertrophic cardiomyopathy.

OBJECTIVE: Cardiac magnetic resonance (CMR) is an uncontested diagnostic tool for identifying and assessing hypertrophic cardiomyopathy (HCM) patients. Concerning the necessity to identify valid prognosticators for predicting the individual risk of clinical evolution, this study aimed to evaluate the clinical validity of CMR tissue tracking (TT) analysis in patients affected by primitive HCM in a real-world setting. METHODS: This historical prospective study included 33 patients. Diagnostic validity and clinical validation were assessed for strain values. CMR-TT diagnostic validity was studied comparing HCM patients with healthy control groups and phenotypic presentation of HCM. The impact of strain values and all phenotypic disease characteristics were assessed in a long-term follow-up study. RESULTS: The inter-reading agreement was good for all strain parameters. Significant differences were observed between the control group and HCM patients. Similarly, hypertrophic and LGE + segments showed lower deformability than healthy segments. The AUC of predictive model, including conventional risk factors for MACE occurrence and all strain values, reached 98% of diagnostic concordance (95% CI .94-1; standard error: .02; p value .0001), compared to conventional risk factors only (86%; 95% CI .73-99; standard error: .07; p value .002). CONCLUSION: In patients with primitive HCM, CMR-TT strain proves high clinical validity providing independent and non-negligible prognostic advantages over clinical features and traditional CMR markers.

A reverse digital workflow by using an interim restoration scan and patient-specific motion with an intraoral scanner.

Interim crowns provide important information as they have been evaluated on patients and can guide the choice of optimal tooth shape, occlusal vertical dimension, and anterior guidance. A protocol with a reverse digital workflow and dynamic occlusion recorded by using an intraoral scanner is presented.

Interim restoration technique for gingival displacement with a feather-edge preparation design and digital scan.

A straightforward and time-efficient technique is presented for recording digital scans in single- and multiple-unit tooth abutments with feather-edge finish lines. The margins of the interim restoration should be precise and sufficiently deep in the sulcus so that the tissues can be properly displaced. Definitive intraoral scans should be made approximately 3 to 4 weeks after the tooth (or teeth) has been prepared to allow healing of the soft tissues. The interim restoration is first removed and then reseated after removing cement residue. First, a scan is made with the interim restoration in place. The abutment tooth or teeth are then erased from the original digital file, the interim restoration is removed again, and a new scan enclosing only the abutment tooth is made.

Anti-interleukin-1 treatment in patients with rheumatoid arthritis and type 2 diabetes (TRACK): A multicentre, open-label, randomised controlled trial.

BACKGROUND: The inflammatory contribution to type 2 diabetes (T2D) has suggested new therapeutic targets using biologic drugs designed for rheumatoid arthritis (RA). On this basis, we aimed at investigating whether interleukin-1 (IL-1) inhibition with anakinra, a recombinant human IL-1 receptor antagonist, could improve both glycaemic and inflammatory parameters in participants with RA and T2D compared with tumour necrosis factor (TNF) inhibitors (TNFis). METHODS AND FINDINGS: This study, designed as a multicentre, open-label, randomised controlled trial, enrolled participants, followed up for 6 months, with RA and T2D in 12 Italian rheumatologic units between 2013 and 2016. Participants were randomised to anakinra or to a TNFi (i.e., adalimumab, certolizumab pegol, etanercept, infliximab, or golimumab), and the primary end point was the change in percentage of glycated haemoglobin (HbA1c%) (EudraCT: 2012-005370-62 ClinicalTrial.gov: NCT02236481). In total, 41 participants with RA and T2D were randomised, and 39 eligible participants were treated (age 62.72 ± 9.97 years, 74.4% female sex). The majority of participants had seropositive RA disease (rheumatoid factor and/or anticyclic citrullinated peptide antibody [ACPA] 70.2%) with active disease (Disease Activity Score-28 [DAS28]: 5.54 ± 1.03; C-reactive protein 11.84 ± 9.67 mg/L, respectively). All participants had T2D (HbA1c%: 7.77 ± 0.70, fasting plasma glucose: 139.13 ± 42.17 mg). When all the enrolled participants reached 6 months of follow-up, the important crude difference in the main end point, confirmed by an unplanned ad interim analysis showing the significant effects of anakinra, which were not observed in the other group, led to the study being stopped for early benefit. Participants in the anakinra group had a significant reduction of HbA1c%, in an unadjusted linear mixed model, after 3 months (ß: -0.85, p < 0.001, 95% CI -1.28 to -0.42) and 6 months (ß: -1.05, p < 0.001, 95% CI -1.50 to -0.59). Similar results were observed adjusting the model for relevant RA and T2D clinical confounders (male sex, age, ACPA positivity, use of corticosteroids, RA duration, T2D duration, use of oral antidiabetic drug, body mass index [BMI]) after 3 months (ß: -1.04, p < 0.001, 95% CI -1.52 to -0.55) and 6 months (ß: -1.24, p < 0.001, 95% CI -1.75 to -0.72). Participants in the TNFi group had a nonsignificant slight decrease of HbA1c%. Assuming the success threshold to be HbA1c% ≤ 7, we considered an absolute risk reduction (ARR) = 0.42 (experimental event rate = 0.54, control event rate = 0.12); thus, we estimated, rounding up, a number needed to treat (NNT) = 3. Concerning RA, a progressive reduction of disease activity was observed in both groups. No severe adverse events, hypoglycaemic episodes, or deaths were observed. Urticarial lesions at the injection site led to discontinuation in 4 (18%) anakinra-treated participants. Additionally, we observed nonsevere infections, including influenza, nasopharyngitis, upper respiratory tract infection, urinary tract infection, and diarrhoea in both groups. Our study has some limitations, including open-label design and previously unplanned ad interim analysis, small size, lack of some laboratory evaluations, and ongoing use of other drugs. CONCLUSIONS: In this study, we observed an apparent benefit of IL-1 inhibition in participants with RA and T2D, reaching the therapeutic targets of both diseases. Our results suggest the concept that IL-1 inhibition may be considered a targeted treatment for RA and T2D. TRIAL REGISTRATION: The trial is registered with EU Clinical Trials Register, EudraCT Number: 2012-005370-62 and with ClinicalTrial.gov, number NCT02236481.

Personalized Symptom Goals and Patient Global Impression on Clinical Changes in Advanced Cancer Patients.

BACKGROUND: The aim of this study was to assess the patients' global impression (PGI) after symptom management, as well as the achievement of personalized symptom goals (PSG). The secondary outcome was to assess related factors. SUBJECTS, MATERIALS, AND METHODS: Advanced cancer patients admitted to palliative care units rated symptom intensity by using the Edmonton Symptom Assessment Score (ESAS) at admission and then after 1 week. For each symptom, patient-reported PGI and PSG, as well as the rate of PSG response, were evaluated. RESULTS: Eight hundred seventy-six patients were taken into consideration for this study. A mean of 1.71-2.16 points was necessary to perceive a bit better improvement of symptom intensity. Most patients had a PSG of ≤3. A statistically significant number of patients achieved their PSG after starting palliative care. Patients with high intensity of ESAS items at admission achieved a more favorable PGI response. In the multivariate analysis, symptom intensity and PSG were the most frequent factors independently associated to a best PGI, whereas high levels of Karnofsky had a lower odd ratio. CONCLUSION: PSG and PGI seem to be relevant for patients' assessment and decision-making process, translating in terms of therapeutic intervention. Some factors may be implicated in determining the individual target and clinical response. IMPLICATIONS FOR PRACTICE: Personalized symptom goals and global impression of change are relevant for patients' assessment and decision-making process, translating in terms of therapeutic intervention. Some factors may be implicated in determining the individual target and clinical response.

The role of sleep in aesthetic perception and empathy: A mediation analysis.

The ability to experience aesthetics plays a fundamental role in human social interactions, as well as the capacity to feel empathy. Some studies have shown that beauty perception shares part of the neural network underlying emotional and empathic abilities, which are also known to affect sleep quality and duration. In this study, we evaluated for the first time the effects of sleep on the relation between aesthetic perception and empathic abilities in healthy subjects using a mediation analysis approach. One-hundred and twenty-six subjects participated in this study. One-hundred and one subjects slept at home (Sleep Group). The remaining 25 subjects were tested as controls after 1 night of sleep deprivation to assess the effects of lack of sleep on aesthetic perception and empathy (Sleep-Deprived Group). All participants underwent one testing session in which they performed a battery of empathy tests and an aesthetic perception task (Golden Beauty). The results showed that sleep duration mediates the relationship between empathy and aesthetic perception in the sleep group. The mediation effect of sleep was more evident on the emotional empathy measures. Conversely, in the sleep deprivation group the lack of correlations among empathy, aesthetic perception and sleep variables did not allow to perform the mediation analysis. These results suggest that adequate sleep duration may play a significant role in improving cognitive and emotional empathic abilities as well as the capability to give accurate aesthetic judgements.

Melphalan hypoxic perfusion with hemofiltration for melanoma locoregional metastases in the pelvis.

BACKGROUND: For patients with melanoma metastases in the pelvic and groin regions, the median survival time (MST) was 8 mo with old treatments, whereas today is approximately 20 mo with new target therapy and novel immunotherapy. Unfortunately, approximately 30% of patients are nonresponsive to these new drugs. MATERIALS AND METHODS: Thirty-six patients, previously progressing after standard treatments, collectively received 146 melphalan (30 mg/m2) hypoxic pelvic perfusions with hemofiltration, in association with palliative excision in most cases. RESULTS: The median follow-up time was 15 mo. Among 36 patients, three patients were alive without evidence of disease after 62, 95, and 118 mo, respectively. Thirty-three patients died of melanoma. The overall MST was 15 mo. The 5-y survival rate was 8%. The MST was 37 mo for stage IIIB; 19 mo for stage IIIC; and 6 mo for stage IV. The MST was 11 mo for patients with ≥1 mitosis per mm2 and 20 mo for patients with <1 mitosis per mm2; 17 mo for patients who received excision and 7 mo for patients who did not receive excision; and 19.5 mo for patients who received >2 treatments and 7.5 mo for patients who received ≤2 treatments. CONCLUSIONS: Pelvic/inguinal perfusion is a safe and feasible treatment for patients with advanced melanoma. Further studies are necessary to establish if it may play a role in patients who fail current systemic therapies.

Sleep disturbances in advanced cancer patients admitted to a supportive/palliative care unit.

The aim of this study was to assess the prevalence of sleep disturbances and possible correlations with associated factors in a sample of patients admitted to an acute palliative/supportive care unit.A consecutive sample of patients with advanced cancer was prospectively assessed for a period of 6 months. Epidemiological and clinical data, treatments received in the last month, Karnofsky status, Edmonton Symptom Assessment System (ESAS), and concomitant medical treatment were also recorded. Patients were administered the Athens Insomnia Scale (AIS) and the Hospital Anxiety and depression scale (HADS).Two hundred nineteen patients met the inclusion criteria. The mean age was 65.4 years (SD 12.4), and 111 patients were males. The mean Karnofsky status was 46.6 (SD = 12). All patients had consistent sleep disturbances (AIS ≥6), with a large number of patients having intense-maximum sleep disturbances. No relationships of AIS with gender, age, primary diagnosis, socio-educational factors, and anticancer treatments were found. AIS score was significantly associated with Karnofsky; intensities of pain, asthenia, anorexia, anxiety, depression, drowsiness, and well-being; and the use of corticosteroids and benzodiazepines. There was a positive correlation of HADS anxiety and HADS depression with sleep disturbances (p = 0.000). In the multivariate analysis, AIS increased only with the level of anxiety and depression assessed by HADS.Sleep disturbances were ubiquitous in advanced cancer patients admitted to a supportive/palliative care unit and were strongly correlated with psychological distress. Assessment of sleep disorders is mandatory in this population for the obvious interference with quality of life.

Does Locoregional Chemotherapy Still Matter in the Treatment of Advanced Pelvic Melanoma?

Pelvic Melanoma relapse occurs in 15% of patients with loco regional metastases, and 25% of cases do not respond to new target-therapy and/or immunotherapy. Melphalan hypoxic pelvic perfusion may, therefore, be an option for these non-responsive patients. Overall median survival time (MST), stratified for variables, including BRAF V600E mutation and eligibility for treatments with new immunotherapy drugs, was retrospectively assessed in 41 patients with pelvic melanoma loco regional metastases. They had received a total of 175 treatments with Melphalan hypoxic perfusion and cytoreductive excision. Among the 41 patients, 22 (53.7%) patients exhibited a wild-type BRAF genotype, 11 of which were not eligible for immunotherapy. The first treatment resulted in a 97.5% response-rate in the full cohort and a 100% response-rate in the 22 wild-type BRAF patients. MST was 18 months in the full sample, 20 months for the 22 wild-type BRAF patients and 21 months for the 11 wild-type BRAF patients not eligible for immunotherapy. Melphalan hypoxic perfusion is a potentially effective treatment for patients with pelvic melanoma loco regional metastases that requires confirmation in a larger multicenter study.

Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers.

BACKGROUND: Adult-onset Still's disease (AOSD) is rare inflammatory disease of unknown etiology that usually affects young adults. The more common clinical manifestations are spiking fevers, arthritis, evanescent rash, elevated liver enzymes, lymphadenopathy, hepatosplenomegaly, and serositis. The multi-visceral involvement of the disease and the different complications, such as macrophage activation syndrome, may strongly decrease the life expectancy of AOSD patients. METHODS: This study aimed to identify the positive and negative features correlated with the outcome of patients. A retrospective analysis of AOSD patients prospectively admitted to three rheumatologic centers was performed to identify the clinical features present at the time of diagnosis and to predict the possible outcome. Furthermore, we investigated the as yet to be validated prognostic value of the systemic score previously proposed. RESULTS: One hundred consecutive AOSD patients were enrolled. The mean systemic score showed that the majority of patients had a multi-organ involvement. Sixteen patients showed different complications, mainly the macrophage activation syndrome. A strong increase of inflammatory markers was observed. All patients received steroids at different dosages, 55 patients in association with immunosuppressive drugs and 32 in association with biologic agents. Sixteen patients died during the follow-up. Regression analysis showed that the higher values of the systemic score and the presence of AOSD-related complications, assessed at the time of diagnosis, were significantly correlated with patient mortality. A prognostic impact of the systemic score of ≥ 7.0 was reported. CONCLUSIONS: Our study showed that a higher systemic score and the presence of AOSD-related complications at the time of diagnosis were significantly associated with mortality. Of note, a cut-off at 7.0 of the systemic score showed a strong prognostic impact in identifying patients at risk of AOSD-related death.