Proceedings from the 2\u3csup\u3end\u3c/sup\u3eNext Gen Therapies for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome symposium held on October 3-4, 2019

© 2020 The Author(s). For reasons poorly understood, and despite the availability of biological medications blocking IL-1 and IL-6 that have markedly improved overall disease control, children with Systemic Juvenile Idiopathic Arthritis (SJIA) are now increasingly diagnosed with life-threatening chronic complications, including hepatitis and lung disease (SJIA-LD). On October 3-4, 2019, a two-day meeting, NextGen Therapies for Systemic Juvenile Idiopathic Arthritis (SJIA) & macrophage activation syndrome (MAS) organized by the Systemic JIA Foundation (www.systemicjia.org/) in Washington, DC brought together scientists, clinicians, parents and FDA representatives with the objectives (1) to integrate clinical and research findings in MAS and SJIA-LD, and (2) to develop a shared understanding of this seemingly new pulmonary complication of SJIA. The current manuscript summarizes discussions and conclusions of the meeting

Identification of backgrounds in the EDELWEISS-I dark matter search experiment

This paper presents our interpretation and understanding of the different backgrounds in the EDELWEISS-I data sets. We analyze in detail the several populations observed, which include gammas, alphas, neutrons, thermal sensor events and surface events, and try to combine all data sets to provide a coherent picture of the nature and localisation of the background sources. In light of this interpretation, we draw conclusions regarding the background suppression scheme for the EDELWEISS-II phase

Measurement of the response of heat-and-ionization germanium detectors to nuclear recoils

The heat quenching factor Q' (the ratio of the heat signals produced by nuclear and electron recoils of equal energy) of the heat-and-ionization germanium bolometers used by the EDELWEISS collaboration has been measured. It is explained how this factor affects the energy scale and the effective quenching factor observed in calibrations with neutron sources. This effective quenching effect is found to be equal to Q/Q', where Q is the quenching factor of the ionization yield. To measure Q', a precise EDELWEISS measurement of Q/Q' is combined with values of Q obtained from a review of all available measurements of this quantity in tagged neutron beam experiments. The systematic uncertainties associated with this method to evaluate Q' are discussed in detail. For recoil energies between 20 and 100 keV, the resulting heat quenching factor is Q' = 0.91+-0.03+-0.04, where the two errors are the contributions from the Q and Q/Q' measurements, respectively. The present compilation of Q values and evaluation of Q' represent one of the most precise determinations of the absolute energy scale for any detector used in direct searches for dark matter.Comment: 28 pages, 7 figures. Submitted to Phys. Rev.

Final results of the EDELWEISS-I dark matter search with cryogenic heat-and-ionization Ge detectors

The final results of the EDELWEISS-I dark matter search using cryogenic heat-and-ionization Ge detectors are presented. The final data sample corresponds to an increase by a factor five in exposure relative to the previously published results. A recoil energy threshold of 13 keV or better was achieved with three 320g detectors working simultaneously over four months of stable operation. Limits on the spin-independent cross-section for the scattering of a WIMP on a nucleon are derived from an accumulated fiducial exposure of 62 kg.d.Comment: 42 pages, 16 figures, submitted to Physical Review D, 1 figure and 2 references added, some little changes in the tex

Calibration of the EDELWEISS Cryogenic Heat-and-ionisation Germanium Detectors for Dark Matter Search

Several aspects of the analysis of the data obtained with the cryogenic heat-and-ionisation Ge detectors used by the EDELWEISS dark matter search experiment are presented. Their calibration, the determination of their energy threshold, fiducial volume and nuclear recoil acceptance are detailed.Comment: 27 pages, Latex, 11 figures. Submitted to NIM

Sensitivity of the EDELWEISS WIMP search to spin-dependent interactions

The EDELWEISS collaboration is searching for WIMP dark matter using natural Ge cryogenic detectors. The whole data set of the first phase of the experiment contains a fiducial exposure of 4.8 kg.day on Ge-73, the naturally present (7.8%), high-spin Ge isotope. The sensitivity of the experiment to the spin-dependent WIMP-nucleon interactions is evaluated using the model-independent framework proposed by Tovey et al.Comment: v2: new references and figures added, other minor changes. Accepted for publication in Physics Letters

Improved Exclusion Limits from the EDELWEISS WIMP Search

The EDELWEISS experiment has improved its sensitivity for the direct search for WIMP dark matter. In the recoil energy range relevant for WIMP masses below 10 TeV/c2, no nuclear recoils were observed in the fiducial volume of a heat-and-ionization cryogenic Ge detector operated in the low-background environment of the Laboratoire Souterrain de Modane in the Frejus Tunnel, during an effective exposure of 7.4 kg.days. This result is combined with the previous EDELWEISS data to derive a limit on the cross-section for spin-independent interaction of WIMPs and nucleons as a function of WIMP mass, using standard nuclear physics and astrophysical assumptions. This limit excludes at more than 99.8%CL a WIMP candidate with a mass of 44 GeV/c2 and a cross-section of 5.4 x 10-6 pb, as reported by the DAMA collaboration. A first sample of supersymmetric models are also excluded at 90%CL.Comment: 14 pages, Latex, 5 figures. Submitted to Phys. Lett.

Development of the autoinflammatory disease damage index (ADDI)

OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. METHODS: We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. RESULTS: More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. CONCLUSIONS: An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process