Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients

Background: Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Several clinical phenotypes have been described, but subepidermal blistering is characteristic of all variants. Limited data on clinical and immunopathological characteristics and treatment outcomes in EBA are available. To fill this gap, we collected this information from EBA cases, meeting current diagnostic criteria, published between 1971 and 2016. Results: We identified 1159 EBA cases. This number must be, however, interpreted with caution, as it is not possible to check for multiple reporting. The analysis of all cases indicated that EBA affects all age groups (median: 50 years, range: 1 to 94 years) at an equal gender distribution. Non-mechanobullous (non-MB) forms of EBA were observed in 55% of patients, whereas the mechanobullous variant (MB-EBA) or a combination of both variants was described in 38 or 7% of patients, respectively. Type VII collagen (COL7)-specific autoantibodies were primarily of the IgG isotype, but anti-COL7 IgA, IgM and IgE were also documented. Comparison of the 2 clinical EBA types showed a higher frequency of IgA deposits in non-MB EBA as opposed to MB EBA. Mucous membrane involvement was observed in 23% of patients, and 4.4% of cases were associated with other chronic inflammatory diseases. Of note, IgA deposits were more frequently observed in cases with mucous membrane involvement. Our analysis indicated that EBA is difficult to treat and that the choice of treatment varies widely. Chi square was applied to identify medications associated with complete remission (CR). Considering all EBA cases, intravenous immunoglobulin (IVIG, p = 0.0047) and rituximab (p = 0.0114) were associated with CR. Subgroup analysis demonstrated that no treatment was associated with CR for non-MB EBA, while IVIG (p = 0.003) was associated with CR in MB EBA. Conclusions: Within the limitations of the study, we here document the clinical and immunopathological characteristics and treatment outcomes in a large cohort of EBA patients. The observed associations of single drugs with treatment outcome may serve as a guide to develop clinical trials

Status of ITER Remote Experimentation Centre

The ITER Remote Experimentation Centre (REC) project (one of the three sub-projects of the InternationalFusion Energy Research Centre (IFERC)) is progressing under the agreement between the Government of Japanand the European Atomic Energy Community for the joint implementation of the Broader Approach (BA) activitiesin the field of fusion energy research. The objectives of the REC activity are to identify the functions andsolve the technical issues for the construction of the REC for ITER at Rokkasho, and to develop the remoteexperiment system and verify the functions required for remote experimentation by using the Satellite Tokamak(JT-60SA) facilities to facilitate the future exploitation of ITER and JT-60SA. The functions of REC will be tested,and the total system will be demonstrated using JT-60SA and existing facilities in the EU, such as JET and WEST.The hardware of the REC has been prepared in Rokkasho Japan, which has the remote experiment room witha large video wall to show the plasma and operation status, IT equipment and a storage system by the reuse ofthe Helios supercomputer tape library. A broadband network infrastructure of 10Gbps has been installed connectedto SINET5. Using this network system, fast data transfer from ITER to REC was examined in 2016, and thetransfer of the data volumes expected for the initial ITER experiments has been demonstrated. A secure remoteexperimentation system has been developed, using JT-60SA, that has functions for preparing and setting of shotparameters, viewing the status of control data, streaming of the plasma status, data-exchange function of shotevents, and monitoring of the facility operation. Remote data analysis techniques, data visualisation software, adocumentation management and experiment planning system and numerical simulation codes for the preparationand performance estimation of discharges have also been developed