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Objectives We evaluated flow-cytometric and transcriptome features of peripheral blood immune cells from early-phase (disease duration <5 years) SSc in comparison with late-phase SSc. Methods Fifty Japanese patients with SSc (12 early SSc cases and 38 late SSc cases) and 50 age- and sex-matched healthy controls were enrolled. A comparison of flow-cytometric subset proportions and RNA-sequencing of 24 peripheral blood immune cell subsets was performed. We evaluated differentially expressed genes (DEGs), characterized the co-expressed gene modules, and estimated the composition of subpopulations by deconvolution based on single-cell RNA-sequencing data. As a disease control, idiopathic inflammatory myositis (IIM) patients were also evaluated. Results Analysing the data from early and late SSc, fraction II effector regulatory T cell (Fr. II eTreg) genes showed a remarkable differential gene expression, enriched for genes related to oxidative phosphorylation. Although the flow-cytometric proportion of Fr. II eTregs was not changed in early SSc, deconvolution indicated expansion of the activated subpopulation. Co-expressed gene modules of Fr. II eTregs demonstrated enrichment of the DEGs of early SSc and correlation with the proportion of the activated subpopulation. These results suggested that DEGs in Fr. II eTregs from patients with early SSc were closely associated with the increased proportion of the activated subpopulation. Similar dysregulation of Fr. II eTregs was also observed in data from patients with early IIM. Conclusions RNA-seq of immune cells indicated the dysregulation of Fr. II eTregs in early SSc with increased proportion of the activated subpopulation. [ABSTRACT FROM AUTHOR]

A 66-year old woman with a 14-year history of rheumatoid arthritis (RA) and uveitis was admitted to our department for evaluation of a mass in the left neck. Fourteen months prior to this admission the patient was started on golimumab. Serum creatine kinase (CK) level was elevated and myositis-specific and -associated antibodies were negative. Manual muscle test showed weakness in the neck flexor, sternocleidomastoid and deltoid muscles. Magnetic resonance imaging (MRI) of the neck, erector muscle of spine, breech, thigh and lower thigh demonstrated high-intensity lesions in the muscles in short-tau inversion recovery images. Electromyography in the right deltoid detected fibrillation potentials. Muscle biopsy from the left neck mass showed granulomatous myositis. Muscle weakness improved and CK levels normalized after discontinuation of golimumab. We report a case of granulomatous myositis under anti-TNF-α treatment for RA. [ABSTRACT FROM AUTHOR]

Objectives. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a disease with significant ethnic differences. Reports on characteristics of Japanese granulomatosis with polyangiitis (GPA) patients are limited, and this study was undertaken to determine the characteristics of Japanese GPA patients. Methods. This was a retrospective chart study of 24 Japanese GPA patients. GPA was defined according to the European Medicines Agency algorithm. Results. The percentage of MPO-ANCA-positive patients was 33.3%, higher than the percentages reported in studies from Western countries. MPO-ANCA-positive GPA patients differed from PR3-ANCA-positive GPA patients in organs involved at diagnosis with MPO-ANCA-positive patients having nose and sinus involvement less frequently compared to PR3-ANCA-positive patients. Interstitial lung infiltrates were more common among MPO-ANCA-positive GPA patients compared to PR3-ANCA-positive GPA patients. Conclusion. Among Japanese GPA patients, the proportion of MPO-ANCA-positive patients is higher compared to reports from Western countries, and those patients are often different from the classical picture of GPA. [ABSTRACT FROM AUTHOR]

This study aimed to develop new image receptor-holding instruments with appropriate horizontal X-ray beam angulation, based on the anatomical data of posterior region interproximal surfaces derived from archived CT images. CT images of 92 patients with sound upper and lower dental arches were collected from our CT database and analyzed to determine the angles between the tangential interproximal contact line and the central groove line of posterior teeth. The average angle for each site was calculated and used to modify instruments using a three-dimensional printer. The utilities of the conventional and the modified instruments for viewing proximal surfaces were compared using two dry skulls. The right and left sides of each site, except for the lower second premolar and first molar sites, did not differ significantly. The difference between the sites was 2.0°; hence, we calculated mean values for the two sides at each site. In the maxilla, the angles of the first and second premolar, second premolar and first molar, and first and second molar to the groove line were 83.9° (±5.4°), 84.4° (±3.9°), 81.6° (±5.1°), while those in the mandible were 85.0° (±9.2°), 85.0° (±4.0°), and 90.6° (±4.9°), respectively. The holding instruments modified to 80° demonstrated better proximal viewing ability in the upper molar region than conventional instruments. The mean angles of the interproximal surfaces were determined from CT data. The image receptor holding-instruments were modified according to these angles to allow appropriate X-ray angulation, which facilitated improved observation of the proximal surfaces of teeth in the posterior region in this pilot in vitro study. [ABSTRACT FROM AUTHOR]