Peripheral blood lymphocyte subset repertoires are biased and reflect clinical features in patients with dermatomyositis.

OBJECTIVE: Dermatomyositis (DM) is an idiopathic inflammatory myopathy which often involves the lungs. DM is likely to be associated with aberrant T- and B-cell activation in the pathogenesis because of the proven effectiveness of T- and B-cell-targeted treatments. Assuming that the aberrant activation is reflected by biases in the lymphocyte subset repertoires, we aimed to elucidate these biases, especially in relation to clinical features of DM. METHOD: Based on the immunophenotyping standardized by the Human Immunology Project Consortium, untreated 13 DM patients, including seven patients with interstitial lung disease (ILD), and 18 age-matched healthy donors (HDs) were examined for proportions of peripheral blood lymphocyte subsets. Six DM patients were examined before and after successful induction of remission. RESULTS: Naïve CD4+ T cells and naïve B cells were more abundant, while there were fewer naïve CD8+ T cells, central memory CD8+ T cells, effector memory CD4+ T cells, Th1 cells, Tfh cells, and memory B cells in DM patients than in HDs. When the patients were subgrouped according to the presence of ILD, the lymphocyte subset repertoires in the patients with ILD contributed to the statistical differences in all the biased lymphocyte subset proportions. After treatment, transitional B cells vanished and there was an increase in memory B cells. CONCLUSION: The lymphocyte subset repertoires in the DM patients were biased, and were associated with the presence of ILD and disease activity of DM.

A positive direct Coombs' test in the absence of hemolytic anemia predicts high disease activity and poor renal response in systemic lupus erythematosus.

We determined the clinical utility of the direct Coombs' test in the absence of hemolytic anemia as an indicator of disease activity and therapeutic response in systemic lupus erythematosus (SLE). SLE patients without hemolytic anemia who visited our hospital from January 2016 to November 2016 were retrospectively evaluated with a direct Coombs' test. Clinical features, including SLE disease activity index (SLEDAI), treatment and laboratory findings were analyzed. For patients with lupus nephritis, we additionally evaluated the cumulative complete renal response rate over one year after induction therapy. Among 182 patients evaluated, 10 (5.8%) patients had a positive direct Coombs' test in the absence of hemolytic anemia. They had a higher SLEDAI ( p < 0.01), higher circulating immune complex levels ( p = 0.01), higher anti-DNA titers ( p < 0.01) and a lower complete renal response rate ( p = 0.03) compared with those who were negative. Multivariate analysis indicated that SLEDAI was an independent factor correlated with the direct Coombs' test without hemolytic anemia (odds ratio 2.4, 95% confidence interval 1.66-4.98, p < 0.01). A positive direct Coombs' test in the absence of hemolytic anemia may therefore represent a useful biomarker for assessing disease activity and therapeutic response.

Successful treatment of severe refractory lupus hepatitis with mycophenolate mofetil.

Systemic lupus erythematosus-related hepatitis, known as lupus hepatitis, is a rare manifestation of systemic lupus erythematosus, and is usually subclinical with mild abnormalities of serum liver enzymes. While cases with clinically significant and refractory lupus hepatitis are uncommon, treatment options for lupus hepatitis are to be established. Here, we report the case of a 45-year-old man with progressive lupus hepatitis accompanied by autoimmune haemolytic anaemia. Lupus hepatitis of this patient was refractory to tacrolimus, azathioprine and cyclophosphamide, but was successfully treated by mycophenolate mofetil. Mycophenolate mofetil might be an effective therapeutic option for refractory lupus hepatitis.

Two-dimensional wave-number spectral analysis techniques for phase contrast imaging turbulence imaging data on large helical device.

An analysis method for unfolding the spatially resolved wave-number spectrum and phase velocity from the 2D CO2 laser phase contrast imaging system on the large helical device is described. This is based on the magnetic shear technique which identifies propagation direction from 2D spatial Fourier analysis of images detected by a 6 × 8 detector array. Because the strongest modes have wave-number at the lower end of the instrumental k range, high resolution spectral techniques are necessary to clearly resolve the propagation direction and hence the spatial distribution of fluctuations along the probing laser beam. Multiple-spatial point cross-correlation averaging is applied before calculating the spatial power spectrum. Different methods are compared, and it is found that the maximum entropy method (MEM) gives best results. The possible generation of artifacts from the over-narrowing of spectra are investigated and found not to be a significant problem. The spatial resolution Δρ (normalized radius) around the peak wave-number, for conventional Fourier analysis, is ∼0.5, making physical interpretation difficult, while for MEM, Δρ ∼ 0.1.

Successful treatment of cerebral large vessel vasculitis in systemic lupus erythematosus with intravenous pulse cyclophosphamide.

A 39-year-old woman with a six-year history of systemic lupus erythematosus (SLE) was admitted because of a prolonged high fever, discoid rash, and multiple lymphadenopathies. She also developed pericarditis, and was treated with intravenous methylprednisolone pulse therapy followed by prednisolone 50 mg daily and cyclosporine 100 mg daily. Meanwhile, she had a progressive headache, and a brain MRI revealed right pons infarction, although she did not have any abnormal neurological findings. An MRA revealed obvious irregular narrowing in the basilar, right vertebral and right posterior cerebral artery. There was no evidence of antiphospholipid syndrome. We concluded that the cause of the asymptomatic brain infarction was cerebral large vessel vasculitis associated with neuropsychiatric SLE. Intravenous cyclophosphamide pulse therapy was started, and two months later, we confirmed that the irregular arterial narrowing had markedly ameliorated.Cerebral large vessel vasculitis in neuropsychiatric SLE is very rare, and a marked amelioration has not been reported to date. Here, we present a rare case of cerebral large vessel vasculitis treated successfully with a clear visual presentation.

Dispersion interferometer using modulation amplitudes on LHD (invited).

Since a dispersion interferometer is insensitive to mechanical vibrations, a vibration compensation system is not necessary. The CO2 laser dispersion interferometer with phase modulations on the Large Helical Device utilizes the new phase extraction method which uses modulation amplitudes and can improve a disadvantage of the original dispersion interferometer: measurement errors caused by variations of detected intensities. The phase variation within ±2 × 10(17) m(-3) is obtained without vibration compensation system. The measured line averaged electron density with the dispersion interferometer shows good agreement with that with the existing far infrared laser interferometer. Fringe jump errors in high density ranging up to 1.5 × 10(20) m(-3) can be overcome by a sufficient sampling rate of about 100 kHz.

Validation experiment of a numerically processed millimeter-wave interferometer in a laboratory.

We propose a new interferometer system for density profile measurements. This system produces multiple measurement chords by a leaky-wave antenna driven by multiple frequency inputs. The proposed system was validated in laboratory evaluation experiments. We confirmed that the interferometer generates a clear image of a Teflon plate as well as the phase shift corresponding to the plate thickness. In another experiment, we confirmed that quasi-optical mirrors can produce multiple measurement chords; however, the finite spot size of the probe beam degrades the sharpness of the resulting image.

First results of electron temperature measurements by the use of multi-pass Thomson scattering system in GAMMA 10.

A multi-pass Thomson scattering (TS) has the advantage of enhancing scattered signals. We constructed a multi-pass TS system for a polarisation-based system and an image relaying system modelled on the GAMMA 10 TS system. We undertook Raman scattering experiments both for the multi-pass setting and for checking the optical components. Moreover, we applied the system to the electron temperature measurements in the GAMMA 10 plasma for the first time. The integrated scattering signal was magnified by approximately three times by using the multi-pass TS system with four passes. The electron temperature measurement accuracy is improved by using this multi-pass system.

Microwave Doppler reflectometer system in LHD.

In order to measure the poloidal rotation velocity, a Doppler reflectometer has been developed in Large Helical Device (LHD). A remotely controlled antenna tilting system has been installed in an LHD vacuum vessel. A synthesizer is used as the source, and the operation microwave frequency ranges are ka-band and V-band. In LHD last experimental campaign we obtained the Doppler shifted signal, which was consistent with CXRS measurements.

Design of the polarization multi-pass Thomson scattering system.

A novel configuration of the multi-pass Thomson scattering (TS) system is proposed to improve the time resolution and accuracy of electron temperature measurements by use of a polarization control technique. This configuration can realize a perfect coaxial multi-passing at each pass, and the number of round trips is not limited by the optical configuration. To confirm the feasibility of the new method, we installed this system in the GAMMA 10 plasma system. As a result, the integrated scattering signal of the double-pass configuration is about two times larger than that of the single-pass configuration. These results are in good agreement with the design.

Development of polarization-controlled multi-pass Thomson scattering system in the GAMMA 10 tandem mirror.

In the GAMMA 10 tandem mirror, the typical electron density is comparable to that of the peripheral plasma of torus-type fusion devices. Therefore, an effective method to increase Thomson scattering (TS) signals is required in order to improve signal quality. In GAMMA 10, the yttrium-aluminum-garnet (YAG)-TS system comprises a laser, incident optics, light collection optics, signal detection electronics, and a data recording system. We have been developing a multi-pass TS method for a polarization-based system based on the GAMMA 10 YAG TS. To evaluate the effectiveness of the polarization-based configuration, the multi-pass system was installed in the GAMMA 10 YAG-TS system, which is capable of double-pass scattering. We carried out a Rayleigh scattering experiment and applied this double-pass scattering system to the GAMMA 10 plasma. The integrated scattering signal was made about twice as large by the double-pass system.

Conceptual design of new polychromator on Thomson scattering system to measure Zeff.

To measure the Z(eff) with electron temperature (T(e)) and electron density (n(e)) profiles at the same time and the same position in the KSTAR tokamak, we design a new polychromator for Thomson scattering system that has additional function. The additional function is measuring bremsstrahlung intensity to calculate Z(eff) independent of Thomson signals. For this new polychromator, we design and fabricate a collimation lens set, and interference filter that has center wavelength of 523 nm and 2 nm FWHM. Finally, we change the lenses, detector diodes, and add the bremsstrahlung filter on the KSTAR edge Thomson scattering polychromator. Then this new polychromator was tested by Tungsten light and monochromator.

Subacute cerebellar ataxia and atrophy developed in a young woman with systemic lupus erythematosus whose cerebrospinal fluid was positive for antineuronal cell antibody.

Subacute cerebellar ataxia in combination with cerebellar atrophy has rarely been reported as one of the manifestations of lupus in the central nervous system (CNS). We describe a 27-year-old woman with systemic lupus erythematosus who developed subacute cerebellar ataxia. Computed tomography and magnetic resonance imaging of her brain showed cerebellar atrophy in both hemispheres, particularly on the right side. Moreover, increased antineuronal cell antibody levels were detected in her cerebrospinal fluid. The cerebellar ataxia improved markedly following high-dose corticosteroid administration. This suggests that a relationship exists between autoantibodies and subacute atrophic processes in CNS lupus.

Observation of long-distance radial correlation in toroidal plasma turbulence.

This Letter presents the discovery of macroscale electron temperature fluctuations with a long radial correlation length comparable to the plasma minor radius in a toroidal plasma. Their spatiotemporal structure is characterized by a low frequency of ∼1-3 kHz, ballistic radial propagation, a poloidal or toroidal mode number of m/n=1/1 (or 2/1), and an amplitude of ∼2% at maximum. Nonlinear coupling between the long-range fluctuations and the microscopic fluctuations is identified. A change of the amplitude of the long-range fluctuation is transmitted across the plasma radius at the velocity which is of the order of the drift velocity.

Development of CO2 laser dispersion interferometer with photoelastic modulator.

A dispersion interferometer is one of the promising methods of the electron density measurement on large and high density fusion devices. This paper describes development of a CO(2) laser dispersion interferometer with a photoelastic modulator for phase modulation. In order to make the dispersion interferometer free from variations of the detected intensity, a new phase extraction method is introduced: The phase shift is evaluated from a ratio of amplitudes of the fundamental and the second harmonics of the phase modulation frequency in the detected interference signal. The proof-of-principle experiments demonstrate the feasibility of this method.

Development of two color laser diagnostics for the ITER poloidal polarimeter.

Two color laser diagnostics using terahertz laser sources are under development for a high performance operation of the Large Helical Device and for future fusion devices such as ITER. So far, we have achieved high power laser oscillation lines simultaneously oscillating at 57.2 and 47.7 µm by using a twin optically pumped CH(3)OD laser, and confirmed the original function, compensation of mechanical vibration, of the two color laser interferometer. In this article, application of the two color laser diagnostics to the ITER poloidal polarimeter and recent hardware developments will be described.

Multifrequency channel microwave reflectometer with frequency hopping operation for density fluctuation measurements in Large Helical Device.

In order to measure the internal structure of density fluctuations using a microwave reflectometer, the broadband frequency tunable system, which has the ability of fast and stable hopping operation, has been improved in the Large Helical Device. Simultaneous multipoint measurement is the key issue of this development. For accurate phase measurement, the system utilizes a single sideband modulation technique. Currently, a dual channel heterodyne frequency hopping reflectometer system has been constructed and applied to the Alfvén eigenmode measurements.

Calibration of electron cyclotron emission radiometer for KSTAR.

We developed and installed an electron cyclotron emission radiometer for taking measurements of Korea Superconducting Tokamak Advanced Research (KSTAR) plasma. In order to precisely measure the absolute value of electron temperatures, a calibration measurement of the whole radiometer system was performed, which confirmed that the radiometer has an acceptably linear output signal for changes in input temperature. It was also found that the output power level predicted by a theoretical calculation agrees with that obtained by the calibration measurement. We also showed that the system displays acceptable noise-temperature performance around 0.23 eV.

Electron cyclotron emission diagnostics on KSTAR tokamak.

A new electron cyclotron emission (ECE) diagnostics system was installed for the Second Korea Superconducting Tokamak Advanced Research (KSTAR) campaign. The new ECE system consists of an ECE collecting optics system, an overmode circular corrugated waveguide system, and 48 channel heterodyne radiometer with the frequency range of 110-162 GHz. During the 2 T operation of the KSTAR tokamak, the electron temperatures as well as its radial profiles at the high field side were measured and sawtooth phenomena were also observed. We also discuss the effect of a window on in situ calibration.