Clinical and laboratory features associated with macrophage activation syndrome in Still’s disease: data from the international AIDA Network Still’s Disease Registry

To characterize clinical and laboratory signs of patients with Still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data. © 2023, The Author(s)

Association between age at disease onset of anti-neutrophil cytoplasmic antibody-associated vasculitis and clinical presentation and short-term outcomes

Objectives: ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- A nd older-onset patients are still incompletely understood. Methods: We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). Results: A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. Conclusion: Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients

Preliminary Field Trails and Simulation Results on Performance of Hybrid Positioning Based on GNSS and 5G Signals

From 1G to 4G, different advances on network-based localization have been developed and included. The 3rd-Generation Partnership Project (3GPP) has being working on these standards defining localization features, such as the Positioning Reference Signals (PRS) and the Long-Term Evolution (LTE) Positioning Protocol (LPP). However, network-based localization has been always considered an optional feature for cellular networks due to its low accuracy, and its methods have been focused mainly on assistance data for GNSS and cell ID enhancement. Now, a new perspective came up in the latest releases of 4G LTE and 5G due to the introduction of high-accuracy positioning services. 3GPP is moving towards including localization for a new range of markets, which has been translated in specific 3GPP activities, aiming at providing high accuracy GNSS for LTE and 5G technologies and designing Radio Access Technology (RAT)- dependent technologies to meet more stringent targets than in previous generations. For high-accuracy positioning, for instance tosupport autonomous driving or industrial automation, the integration of GNSS (augmented with precise or differential corrections), terrestrial (RAT-dependent) technologies and complementary sensors is expected to play a key role on 5G localization. The goal of GINTO5G project is to support the design of PNT solutions in the context of 5G applications by carrying out extensive experiments and simulation campaigns, as well as theoretical assessment of possible disruptive techniques. For downlink TDoA using 5G SRS signal, the field trials of one campaign shows that sub-meter accuracy can be achieved with 100 MHz bandwidth in the 3.7 GHz band. At the same time the evaluation shows a significant discrepancy between achieved TOA accuracy, and the overall positioning performance, especially for the outdoor tests. Based on CEP95 and SEP95 values, it can be stated that a 2D accuracy of sub 3 meter can be achieved an outdoor area where transmitting points have been deployed and optimized for positioning purpose. Similar performance could be seen in the results of the tests carried out in indoor spaces; what is more, half of all measured indoor positions even show a significantly lower error (sub 1 meter for 2D, and sub 3 meters for 3D). Another set of outdoor trials, conducted this time on a set of transmitting points deployed more randomly, revealed a mean 2D positioning error ranging from sub metre to several hundreds of metres