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A 54-year-old man with paroxysmal atrial fibrillation underwent pulmonary vein (PV) isolation using a 28-mm second-generation cryoballoon catheter. Unexpected electrical superior vena cava (SVC) isolation during cryoballoon application to the right superior PV was observed in the first procedure. Thirteen-months after cryoballoon-based PV isolation, sustained SVC fibrillation was confirmed irrespective of sinus rhythm. Voltage mapping visualized a low voltage area around the SVC was close to that around the right superior PV. SVC isolation was obtained by cryoballoon application to the right superior PV and persisted for over a year.

BACKGROUND/PURPOSE Although ursodeoxycholic acid (UDCA) is a first-line treatment for primary biliary cholangitis (PBC), 20%-30% of patients with PBC exhibit an incomplete response to UDCA. Recently, the UDCA Response Score was proposed for predicting response to UDCA using pretreatment parameters in patients with PBC. We aimed to validate the UDCA Response Score in Japanese patients with PBC. METHODS Registry data of Japanese patients (n = 873) were collected. Patients with data on all clinical parameters required for calculating the UDCA Response Score were selected. The endpoint was UDCA response, defined as alkaline phosphatase

A 51-year-old man with dilated cardiomyopathy was resuscitated from ventricular fibrillation. Twenty-days after using a wearable cardioverter-defibrillator (WCD) contact dermatitis with itching was evident and consistent with the self-gelling defibrillation electrodes patch on the back. Itching was controlled with clobetasol propionate application. The WCD was continued until catheter ablation and device implantation. The contact dermatitis was completely recovered two weeks after discontinuing the WCD. Among 58 patients using the WCD, three (5.2%) complained about discomfort with the device, and two (3.4%) complained of itching. Only the patient presented here (1.7%) suffered from contact dermatitis with itching. Contact dermatitis is rarely observed in patients wearing a WCD but physicians should be aware of this complication to maintain WCD compliance.

In Japan, bezafibrate (BF) is a second-line agent for primary biliary cholangitis (PBC) that is refractory to ursodeoxycholic acid (UDCA) treatment. From a retrospective cohort (n = 873) from the Japan PBC Study Group, we enrolled 118 patients who had received UDCA monotherapy for at least 1 year followed by combination therapy with UDCA+BF for at least 1 year. GLOBE and UK-PBC scores after UDCA monotherapy (i.e., immediately before UDCA+BF combination therapy) were compared with those after 1 year of UDCA+BF combination therapy. The real outcomes of enrolled patients estimated by Kaplan-Meier analysis were compared with the predicted outcomes calculated using GLOBE and UK-PBC scores. In addition, the hazard ratio of BF treatment was calculated using propensity score analysis. The mean GLOBE score before the combination therapy was 0.504 ± 0.080, which improved significantly to 0.115 ± 0.085 (P < 0.0001) after 1 year of combination therapy. The real liver transplant-free survival of enrolled patients was significantly better than that predicted by GLOBE score before introducing BF. Combination therapy did not significantly improve the real rates of liver transplantation or liver-related death compared with those predicted by UK-PBC risk score before introducing BF, but the predicted risk was significantly reduced by the addition of BF (P < 0.0001). Cox regression analysis with inverse probability of treatment weighting showed that the addition of BF significantly reduced the hazard of liver transplant or liver-related death in patients who, after 1 year of UDCA monotherapy, had normal serum bilirubin (adjusted hazard ratio 0.09, 95% confidence interval 0.01-0.60, P = 0.013). Conclusion: Addition of BF to UDCA monotherapy improves not only GLOBE and UK-PBC scores but also the long-term prognosis of PBC patients, especially those with early-stage PBC.

18 F‐FDG‐PET/CT is promising tool to visualize not only transvenous lead infection but also epicardial lead infection.

INTRODUCTION The predictive value of left atrial volume (LAV) in atrial fibrillation (AF) is known, but the relationship of right atrial volume (RAV) and biatrial volume (BAV) with AF recurrence after pulmonary vein isolation (PVI) is not clear. Cardiac magnetic resonance (CMR) imaging allows us to more precisely quantify atrial volume. We investigated LAV, RAV, and BAV as predictors of AF recurrence following PVI in AF patients. METHODS AND RESULTS We assessed 100 AF patients (age = 59.8 ± 9.5 years, 74 males, 26 females) who underwent nonenhanced CMR before their first PVI. LAV and RAV were measured using CMR. All patients were in sinus rhythm during CMR. BAV was calculated as the sum of LAV and RAV. During the 8-month follow-up, AF recurrence occurred in 23 patients. LAV, RAV, and BAV were significantly greater in patients with AF recurrence than in those without (LAV, 103.7 ± 25.8 vs 81.8 ± 24.2 mL, P

Aim The 2012 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for polymyalgia rheumatica (PMR) were published in 2012. The present study aimed to assess the 2012 EULAR/ACR classification criteria for PMR in Japanese patients diagnosed with PMR using Bird's criteria. Methods The study included 75 patients diagnosed using Bird's criteria. The patients were divided into fulfilled and not-fulfilled groups according to whether they met the 2012 EULAR/ACR classification criteria for PMR. The factors in the new criteria were morning stiffness duration > 45 min, hip pain or limited range of motion, absence of rheumatoid factor or anti-citrullinated protein antibody, and absence of other joint involvement. Results Thirty-two of the patients diagnosed with PMR using Bird's criteria met the new EULAR/ACR classification criteria, while the remaining 43 patients did not meet the new criteria. Among the factors, only morning stiffness duration > 45 min was an independent predictive factor. Conclusions A morning stiffness duration > 45 min is the only independent predictive factor for differentiating patients diagnosed according to the new 2012 EULAR/ACR classification criteria for PMR.

Background Salivary gland (SG) ultrasonography proved valuable for assessing SG involvement in Sjogren’s syndrome (SS) and seemed to exhibit good diagnostic properties. We have reported that the submandibular gland ultrasonography (SGUS) is a useful noninvasive and inexpensive procedure for the evaluation of the structural changes of SG in SS (ISSS 2002, EULAR 2009, EULAR 2012, EULAR 2015). However, our previously study demonstrated that although SGUS findings were useful for the diagnosis of SS with low salivary flow they were not for early stage SS with normal salivary flow (EULAR 2016). Recently, we reported that the tissue elasticity was decreased due to structural changes in the SG at the advanced stage of the disease and that the shear wave elastography (SWE) is useful to distinguish pathological changes of the SG between early stage with normal salivary flow and advanced stage (EULAR2018). The present study we demonstrated that the tissue elasticity was increased due to inflammation and high viscosity in the SG at the early stage of SS with normal salivary flow comparing that in non-SS patients, but was decreased due to structural changes in the SG at the advanced stage of the disease. The SWE may be a useful tool for elucidation of early stage pathological changes of the SG when salivary gland functions are not impaired in SS. Objectives The aim of this study was to elucidate the usefulness of SWE in early-stage SS patients. Methods Seventeen non-SS patients and eighty patients who fulfilled the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for SS were studied. SS patients were divided into three groups according to salivary flow using gum test (VL/SS: Results The Vs and E values were correlated with US staging score (r=-0.56, p Conclusion The present study we demonstrated that the tissue elasticity was increased due to inflammation and high viscosity in the SG at the early stage of SS with normal salivary flow comparing that in non-SS patients. The SWE may be a useful tool for the differential diagnosis between patients with non-SS and early-stage SS with normal salivary flow in contrast to conventional SGUS. Disclosure of Interests None declared

Background:Primary Sjögren’s syndrome (pSS) is a chronic inflammatory autoimmune disease characterized by lymphocyte infiltration in salivary and lacrimal glands. pSS affects primarily middle-aged and elderly patients, although younger age groups may also be involved. However, differences of etiology and pathogenesis between early-onset pSS (EOpSS) and late-onset pSS (LOpSS) are unknown. Recently, standardized outcome tools for measuring disease-specific activity and patients’ reported symptoms have been formulated by the European League Against Rheumatism (EULAR) SS study group: the EULAR SS Disease Activity Index (ESSDAI) for systemic features of pSS [1]. Also, as the new imaging techniques, salivary gland ultrasonography (SGUS) proved valuable for assessing salivary gland involvement in SS and seemed to exhibit good diagnostic properties. In addition, previous studies have demonstrated usefulness of SGUS for the prognostic stratification of patients with pSS [2], [3], [4].Objectives:The aim of this study was to examine the differences of etiology and pathogenesis between EOpSS and LOpSS using ESSDAI and SGUS.Methods:Fifty-six pSS patients who fulfilled the American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) classification criteria for SS were studied. Based on the disease onset age, all pSS patients were divided into two groups as those with the onset age of 40 years old or younger (EOpSS: n=26) and those with the onset age of older than 65 years old (LOpSS: n=30). The clinical findings were evaluated ESSDAI and OMERACT SGUS score at the first visit to our hospital. The ESSDAI (0–123) proposes the evaluation of 12 domains or organ systems (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system, muscular, hematological and biology). All patients were examined SGUS by a single investigator who was blinded to device (TUS-A300; Canon Medical Systems, Tokyo, Japan) with a linear transducer (7.5-10MHz). The OMERACT SGUS score was used for graded changes in the parenchymal homogeneity of salivary glands: grade 0, normal-appearing salivary gland parenchyma; grade 1, minimal change: mild inhomogeneity without hypo/anechoic areas; grade 2, moderate change: moderate inhomogeneity with focal hypo/anechoic areas; grade 3, severe change: diffuse inhomogeneity with hypo/anechoic areas occupying the entire gland surface [5].Results:The proportions of positive sera of RF, anti-SS-A and anti-SS-B antibodies were not different in the two groups, but the disease activities were higher in the EOpSS than in the LOpSS patients by measuring ESSDAI (7.30 vs 4.23, p=0.008), especially in constitutional domain (1.50 vs 0.60, p=0.03), articular domain (1.54 vs 0.40, p=0.0002) and biological domain (1.35 vs 0.90, p=0.04). No difference in salivary secretion was found between two groups (EOpSS: 8.02 vs LOpSS: 6.31 mL/10min.), but the OMERACT SGUS score was higher in LOpSS than in EOpSS patients (2.00 vs 2.70, p=0.0002).Conclusion:Although serological findings were not different, EOpSS patients had higher disease activity but less severe salivary gland degeneration than that in LOpSS patients, suggesting the pathogenesis of these two groups was different.References:[1]Seror R, et al. Ann Rheum Dis. 2010 Jun;69(6):1103-9.[2]Arthritis Care Res (Hoboken). 2014 Jul;66(7):1102-7.[3]Hammenfors DS, et al. Clin Exp Rheumatol. 2015 Jan-Feb;33(1):56-62.[4]Milic V, et al. PLoS One. 2019 Dec 31;14(12): e0226498.[5]Jousse-Joulin S, et al. Ann Rheum Dis. 2019 Jul;78(7):967-973.Disclosure of Interests:None declared

Background The relationship between the neutrophil-to-lymphocyte ratio (NLR) and outcome in patients with implantable cardioverter-defibrillators (ICDs) is unclear. Methods and results Consecutive patients with cardiomyopathy who had received an ICD (n = 120, mean age 64 ± 11 years) were prospectively enrolled. Blood samples were obtained on the morning of the day of implantation. Patients were followed for a median period of 61.2 months, to an endpoint of all-cause mortality or appropriate ICD shock, which occurred in 35 (29%) and 28 (23%) patients, respectively. Multivariate Cox analysis revealed that secondary prevention was only associated with appropriate ICD shocks. The NLR, brain natriuretic peptide level, and estimated glomerular filtration rate were independent predictors of all-cause mortality but not of appropriate ICD shocks. Subgroup analysis revealed that a high NLR (≥2.1) was valuable for anticipating all-cause mortality among patients who had received ICDs for primary or secondary prevention. A high NLR was also associated with death prior to appropriate ICD shock. Conclusion Evaluating the NLR may be useful for predicting outcomes in patients with cardiomyopathy who have received ICDs.

Biologics used in the treatment of rheumatoid arthritis (RA) rarely cause eosinophilia. We herein report a patient with RA being treated with infliximab, adalimumab, and tocilizumab who developed eosinophilia with skin symptoms. Interestingly, the marked eosinophilia and skin symptoms did not reappear after the patient's medication was switched to golimumab. In this case, the presence of biologics-specific antibodies suggested that immunogenicity caused the eosinophilia. Therefore, switching to a biologic with a lower immunogenicity was effective. These findings may be helpful for clinicians treating patients with biologics-induced eosinophilia.

Background: The aim of this study was to establish a minimally invasive defibrillation testing (DT) protocol for patients with implantable cardioverter defibrillators (ICDs). Methods: Two different energy DTs were performed, immediately after (15 J-DT) and 7 days after (≤10 J-DT) device implantation, in 20 consecutive ICD implantation patients. Cardiac-troponin T (c-TNT) and heart-type fatty acid binding protein (H-FABP) levels were measured before implantation, 2 h after implantation, and 1 day after each DT. For an additional 122 patients with ICD, we retrospectively analyzed 203 DTs immediately and 7 days after device implantation. Results: Serum c-TNT levels were significantly elevated 2 h after 15 J-DT [0.008 (0.004–0.019) vs. 0.053 (0.037–0.068) ng/mL, p

Ablation of ventricular tachycardia originating from the great cardiac vein involves the difficult step of deep engagement with an ablation catheter. The catheter and a steerable sheath (MobiCath, Biosense Webster, Diamond Bar, CA, USA) were advanced alternately only when the contact force vector was parallel to the coronary venous system. Deep engagement with a steerable sheath ensured a powerful backup force during ablation.

Background:Sjögren’s syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocyte infiltration in salivary and lacrimal glands. Recently, salivary gland ultrasonography (US) proved valuable for assessing salivary gland involvement in SS and seemed to exhibit good diagnostic properties. In the literature, studies conducted by the scoring of the structural changes according to B-Mode US of salivary glands showed a wide variability regarding sensitivity and specificity. Our previously study demonstrated that although conventional B-mode US findings were useful for the diagnosis of SS with low salivary flow they were not for subclinical SS with normal salivary flow (EULAR 2016). Recently, we reported that the tissue elasticity was decreased due to structural changes in the submandibular glands (SG) at the advanced stage of the disease and the shear wave elastography (SWE) is useful to distinguish pathological changes of the SG in patients with SS (EULAR2018).Objectives:The aim of this study was to compare the usefulness of SG conventional B-mode US and SWE findings in non-SS and SS patients classified by salivary flow.Methods:Twenty-two non-SS patients and 99 SS patients who fulfilled the American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) classification criteria for SS were studied. SS patients were divided into three groups according to salivary flow using gum test (VL/SS 10mL/10min. (n=20)). All patients were examined SGUS by a single investigator who was blinded to device (TUS-A300; Canon Medical Systems, Tokyo, Japan) with a linear transducer (7.5-10MHz). The examination consisted of conventional B-mode US (US staging score), pulsed wave Doppler US (PD grading score) and SWE with quantitative assessment. US staging scores were assessed by glandular size, inhomogeneity and contrast of diagastric muscle (stage 0 to 3). PD grading scores were graded by pulsed wave pattern in pulsed wave Doppler US at the internal SG facial arteries (grade 0 to 2). With the region-of-interest (ROI) placed over the stiffest areas of the lesion on SWE, the quantitative means of the elasticity values were measured by shear wave velocity (Vs; m/s) and elasticity (E; kPa) for each lesion.Results:The US staging score, the PD grading score, the values of Vs and E were significantly higher in patients with SS than in non-SS group (SS vs non-SS; US staging score 2.10±1.07 vs 0.86±0.99, pConclusion:The present study demonstrated that although the tissue elasticity was decreased due to structural changes at the advanced stage, it increased due to inflammation and high viscosity in the SG at the subclinical SS with normal salivary flow comparing that in non-SS patients. The SWE may be a useful tool for the differential diagnosis between patients with non-SS and subclinical SS with normal salivary flow, which is difficult to distinguish by conventional B-mode US.Disclosure of Interests:None declared

Objectives This study sought to determine the feasibility of a novel simplified ablation protocol targeting only the pulmonary vein antrum using the radiofrequency hot-balloon catheter in patients with paroxysmal atrial fibrillation. Background Radiofrequency hot-balloon (RHB) catheter has been recently introduced into clinical practice for pulmonary vein isolation (PVI). The authors hypothesized that a novel simplified ablation protocol targeting only the PV antrum with energy application for a longer time (single-shot technique) could be an alternative approach to achieve PVI, while avoiding unnecessary energy application at the PV ostium. Methods A total of 61 consecutive paroxysmal atrial fibrillation patients (age 64.1 ± 10.9 years, 48 male) who underwent antrum RHB-PVI were enrolled. Energy applications were performed following the pre-specified protocol only targeting the PV antrum. If the PVI was not achieved after 2 energy applications using the RHB, a touch-up ablation was performed. Results Of 241 PV, including 3 left common PV, 194 (80%) were isolated exclusively using the RHB. The target PVI average per group of 15 consecutive procedures improved from 75% (initial 15) to 89% (last 16) of patients. The injected volume was greatest in the right superior PV (13.1 ± 2.0 ml) and the smallest in the left inferior PV (10.8 ± 1.1 ml), and 23 PV (9.5%) required over 15 ml (estimated balloon diameter of 30 mm). Periprocedural complications were noted in 3 patients (4.9%), but phrenic nerve injury was not observed. Sinus rhythm maintenance at 12-month follow-up was achieved in 57 patients (93%). Conclusions A novel simplified antrum RHB-PVI appears to be a feasible technique for the treatment of paroxysmal atrial fibrillation.

Outdoor images may suffer from haze, and the clearness of the image is greatly lost. The haze removal, which is known as dehazing, is an important issue. In this paper, we propose a fast haze removal method, which is an improving version of an existing method using dark channel prior proposed by He et al. [1]. In our proposed method, we significantly reduce the computational time by improving the method to estimate the dark channel. Our proposed dark channel estimation method uses a down-sampled image and do not need a soft-matting process [2]. Experiments with haze images show that our proposed method is faster and an acceptable quality level compared with the existing He's dark channel method.

Background We have reported that the submandibular gland ultrasonography (SGUS) is a useful noninvasive and inexpensive procedure for the evaluation of the structural changes of salivary gland (SG) in Sjogren’s syndrome (SS), International Symposium on SS 2002 , EULAR 2009 , EULAR 2012 . EULAR 2015 However, our previously study demonstrated that although SGUS findings were useful for the diagnosis of SS with low salivary flow they were not for early-stage SS with normal salivary flow. EULAR 2016 Recently, ultrasound elastography has been reported to be a new tool to evaluate tissue stiffness and diagnose tumour. Objectives The aim of this study was to examine the usefulness of SGUS using US staging and PD grading score in combination with shear wave elastography (SWE) in patients with SS. Methods Fifty-eight patients who fulfilled the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for SS were studied. SS patients were divided into three groups according to salivary flow using gum test (VL/SS: Results The US staging scores of SS patients were 0.0% in stage 0; 17.2% in stage 1; 8.6% in stage 2; 74.1% in stage 3. The PD grading scores of SS patients were 20.7% in grade 0, 17.2% in grade 1, 62.1% in grade 2. The US staging and grading scores were significantly lower in N/SS patients (1.40±0.70, 0.10±0.32) than in L/SS (2.74±0.66, p Conclusions The present study demonstrated that the tissue elasticity was increased due to inflammation and high viscosity in the SG at the early stage, but was decreased due to structural changes in the SG at the advanced stage of the disease. The SWE may be a useful tool for elucidation of early stage pathological changes of the SG when salivary gland functions are not impaired in SS. Disclosure of Interest None declared

Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, especially the salivary and lacrimal glands. As a result of salivary gland dysfunction, most patients with SS have xerostomia, related to a reduced salivary flow rate. In addition to the discomfort due to xerostomia, dry mouth can cause various intraoral manifestations such as refractory stomatitis, ulcer and atrophic changes in the oral mucosa and tongue, and patients' quality of life (QOL) is impaired severely. These manifestations are believed to be caused mainly by a decrease in the clearance in the oral cavity owing to hyposalivation. However, since saliva has several beneficial physiological effects on the intraoral environment, qualitative changes in sialochemistry should also be considered a cause of the refractory intraoral manifestations in SS. Salivary epidermal growth factor (EGF) is considered an important cytoprotective factor against injuries, and it contributes to wound healing in the oral cavity. We evaluated changes in salivary EGF levels and assessed the association between salivary EGF levels and the severity of intraoral manifestations in SS patients. The results showed that the salivary EGF levels decreased with the progression of SS, and this deterioration in saliva quality as well as hyposalivation could play a role in the pathogenesis of refractory intraoral manifestations in SS patients. Our findings provide new target for therapeutic intervention in SS.

Nongated DSCT for AF Catheter Ablation Introduction Using a high-pitch dual-source CT (DSCT), we aimed to quantify the amounts of contrast media, radiation doses, and image qualities in patients undergoing pulmonary vein (PV) isolation. Methods and Results The study enrolled 60 patients who were randomly assigned in a 1: 1: 1 ratio to undergo ECG-gated 64-slice multidetector computed tomography (MDCT; group I, n = 20), ECG-gated 128-DSCT (group II, n = 20), and nongated 128-DSCT (group III, n = 20). The total amount of contrast media was lower in groups II and III compared with group I (I: 54.7 ± 5.6, II: 26.6 ± 2.7, and III: 28.7 ± 6.9 mL, P < 0.001). The CT dose index was lower in groups II and III compared with group I (I: 73.1 ± 5.2, II: 3.5 ± 0.1, and III: 3.7 ± 0.1 mGy, P < 0.001). The dose length product was lower in groups II and III compared with group I (I: 1154.8 ± 82.8, II: 75.4 ± 2.3, and III: 77.2 ± 1.9 mGy × cm, P < 0.001). The total CT effective radiation dose was lower in groups II and III compared with group I (I: 16.2 ± 1.2, II: 1.1 ± 0.1, and III: 1.1 ± 0.1 mSv, P < 0.001). The total CT scan duration was shorter in group III compared with groups I and II (I: 30.8 ± 2.2, II: 23.4 ± 3.6, and III: 16.0 ± 2.4 minutes, P < 0.001). There were no significant differences in quality for integrated electroanatomical mapping (EAM) and parameters associated with PV isolation among the 3 groups. Conclusion Nongated 128-DSCT provides sufficient image quality to allow integrated EAM while exposing the patient to less contrast media, lower radiation doses, and shorter CT scan durations.

Background Immediate recurrence of atrial fibrillation (AF) after radiofrequency (RF) catheter ablation is commonly observed within 3d after the procedure. The mechanism and pharmacological management of immediate AF recurrence remain unclear. Methods A total of 50 consecutive patients with paroxysmal AF were randomized to receive either low-dose landiolol (landiolol group) or a placebo (placebo group). In the landiolol group, intravenous landiolol (0.5μgkg −1 min −1 ) was administered for 3d after AF ablation. Results No serious adverse event associated with RF catheter ablation or landiolol administration was observed. The prevalence of immediate AF recurrence (≤3d after RF catheter ablation) was significantly lower in the landiolol group than in the placebo group (16% vs. 48%, p =0.015). Although the postprocedural change in heart rate was significantly lower in the landiolol group compared to that in the placebo group, the changes in blood pressure and body temperature were not different between the two groups. Multiple logistic regression analysis revealed that landiolol treatment was the only independent predictor of immediate AF recurrence after ablation (odds ratio: 0.180; 95% confidence interval: 0.044–0.729; p =0.016). Conclusions Prophylactic administration of low-dose landiolol after AF ablation may be effective and safe for preventing immediate AF recurrence within 3d after AF ablation.

A 62-year-old male was admitted to our hospital due to left lower abdominal pain. Three days before admission, he ate seafood in a Japanese restaurant. Two days before admission, he suffered from intermittent left lower abdominal pain. One day before admission, he developed a high fever and subsequently visited our hospital the following day. Localized tenderness and rebound pain were observed in the left lower abdomen, and C-reactive protein was elevated. Computed tomography revealed a linear high-density object in the distal portion of the small intestine accompanied by edema of the wall, suggesting penetration by something like a fishbone. On the ninth hospital day, double-balloon endoscopy was performed via the transanal route. Yellow foreign material was found in the ileum and was then successfully removed with biopsy forceps. The removed material measured 3 mm in width, 3 cm in length and was slightly curved. It proved to be a bone of the forefoot of a soft-shelled turtle, which had been included on the menu of the restaurant. The patient was completely cured and discharged on the 14th hospital day.

Background Systemic sclerosis (SSc) is an inflammatory autoimmune disease characterized by fibrosis and small vascular involvement in the skin, lungs, heart and gastrointestinal (GI) tract. The esophagus is the most frequently involved GI tract disorder. Although the small intestinal involvement such as malabsorption and pseudo-obstruction is less common, it has been related to morbidity and mortality of SSc patients. We previously reported a close correlation between the esophageal diameter and the small intestinal clearance (SIC) (EULAR 2011). We also suggested that anti-centromere antibodies (ACA) are an important factor of esophageal dilatation in SSc patients (EULAR 2010). However, changes of small intestinal involvement during long-term follow-up have not yet been defined. Objectives The aim of this study was to evaluate a correlation between the small intestinal involvement and clinical features in SSc patients during ten years follow-up. Methods Fifty-five patients with a definite diagnosis of SSc [52 female and 3male with mean age of 59.4 years (range 29–77 years)] were included in the study. Thirteen (23.6%) patients were classified as diffuse SSc and 42 (76.4%) as limited SSc. The SIC grade was classified according to barium meal reach at 30 min after intake; grade 1 (>2/3 of the whole small intestine), grade 2 (1/3∼2/3), grade 3 ( Results The mean durations of follow-up period were 9.5±0.6 years. The number of SSc patients in each SIC grade at the initial evaluation and the end of follow-up were as follows; grade 1: initial vs end; 23 vs 24, grade 2: 20 vs 9, grade 3: 10 vs 16, grade 4: 2 vs 6. The SIC change “progressive” was 40.0% of SSc patients. Positive correlation between the esophageal diameters and the SIC grade was observed in SSc patients at the initial evaluation (r=0.61 p Conclusions The present study demonstrated that the progression of small intestinal involvement was associated with esophageal dilatation at the initial evaluation during long-term follow-up of SSc patients. Our findings also suggested that ACA and skin thickening of the fingers were important factors of small intestinal involvement in SSc patients. The SIC may be a useful tool for the assessment of GI tract involvement in SSc patients during long-term follow-up. Disclosure of Interest None declared

Background:The difference between left atrial (LA) and systemic coagulation activity in paroxysmal atrial fibrillation (PAF) is unclear.Methods and Results:We enrolled 100 patients with PAF who underwent AF ablation. Warfarin was stopped 1 day before the procedure. LA volume index and LA emptying fraction were measured by 64-slice multidetector computed tomography. Immediately after transseptal puncture, blood samples were simultaneously collected from the LA and systemic circulation (SC). In addition, to evaluate the effect of warfarin on D-dimer levels we recruited an additional 27 PAF patients on continuous warfarin. Even in patients with low CHADS2scores (mean 0.59±0.68) and during sinus rhythm, the prevalence of positive LA-D-dimer (≥0.5 µg/ml) was greater than that of SC-D-dimer (23% vs. 10%, P

The relationship between the neutrophil-to-lymphocyte ratio (NLR) and outcome in patients with implantable cardioverter-defibrillators (ICDs) is unclear.Consecutive patients with cardiomyopathy who had received an ICD (n = 120, mean age 64 ± 11 years) were prospectively enrolled. Blood samples were obtained on the morning of the day of implantation. Patients were followed for a median period of 61.2 months, to an endpoint of all-cause mortality or appropriate ICD shock, which occurred in 35 (29%) and 28 (23%) patients, respectively. Multivariate Cox analysis revealed that secondary prevention was only associated with appropriate ICD shocks. The NLR, brain natriuretic peptide level, and estimated glomerular filtration rate were independent predictors of all-cause mortality but not of appropriate ICD shocks. Subgroup analysis revealed that a high NLR (≥2.1) was valuable for anticipating all-cause mortality among patients who had received ICDs for primary or secondary prevention. A high NLR was also associated with death prior to appropriate ICD shock.Evaluating the NLR may be useful for predicting outcomes in patients with cardiomyopathy who have received ICDs.

Anticentromere antibody (ACA) is generally considered to be a serological marker for systemic sclerosis (SSc). ACA-positive patients with primary Sjögren's syndrome (pSS) have also been reported. ACA often recognizes centromere proteins (CENPs): CENP-A, CENP-B, and CENP-C, and sometimes reacts to heterochromatin protein 1 (HP1)α. We compared the reactivity against six different epitopes for three ACA-positive clinical subgroups: 29 patients with pSS, 36 SSc patients with sicca symptoms, and 28 SSc patients without sicca symptoms.We utilized enzyme-linked immunosorbent assays (ELISAs) with recombinant proteins covering six different epitope regions of ACA (the amino terminus (Nt) of CENP-A, CENP-B, and CENP-C, the carboxyl terminus (Ct) of CENP-B and CENP-C, and HP1α).The patients with pSS were found to have IgG-class autoantibodies against CENP-C-Nt and HP1α, and IgA-class autoantibodies against CENP-C-Ct with significantly higher frequencies than the SSc patients with or without sicca symptoms. The positive predictive value and the negative predictive value of the combination of these three autoantibodies for pSS were 73% and 82%, respectively, for pSS.Based on the result that reactivities against CENP-C and HP1α in patients with pSS differ from those in patients with SSc, we propose ACA-positive pSS as a clinical subset of SS that is independent of SSc.

Background Since previous studies have investigated the population dynamics of Japan-indigenous genotype 3 hepatitis E virus (HEV) using virus sequences, more nucleotide sequences have been determined, and new techniques have been developed for such analysis. Aims To prevent future hepatitis E epidemic in Japan, this study aimed to elucidate the cause of past HEV expansion. Methods The epidemic history of Japan-indigenous genotype 3 HEV was determined using the coalescent analysis framework. Bayesian skyline plot (BSP) and Bayesian estimate of phylogeny with relaxed molecular clock models were calculated using Markov chain Monte Carlo sampling. Results Japan-indigenous strains consist of New World strains (subtype 3a), Japanese strains (3b) and European strains (3e). The oldest lineage, 3b, appeared around 1929. Lineages 3a and 3e appeared around 1960. BSPs indicated similar radical population growth of the 3a and 3b lineages from 1960 to 1980. Conclusions Population dynamics of the three lineages shared some common characteristics, but had distinguishing features. The appearance of 3a and 3e lineages coincides with the increase of large-race pig importation from Europe and the USA after 1960. The epidemic phase of 3a and 3b strains from 1960 to 1980 could be related to increased opportunity for HEV infection arising from large-scale pig breeding since 1960. Our observations revealed new findings concerning the close relationship between the epidemic history of Japan-indigenous genotype 3 HEV and the improvement of the Japanese pig industry. Infection control in pig farms should be an effective method of preventing HEV infection in humans.

In May 2009, a 57-year-old woman who had rheumatoid arthritis since 9 years was admitted to our hospital for dyspnea due to interstitial pneumonia (IP). On admission, she exhibited proximal scleroderma, finger edema, Raynaud's phenomenon, digital pitting scars, ankyloglossia, and esophageal dysmotility. The patient was diagnosed as having systemic sclerosis (SSc), according to the American College of Rheumatology criteria. After initiation of high-dose corticosteroid therapy, gradual amelioration of IP was observed. However, the patient complained of abdominal fullness. Computed tomography and intestine series findings revealed significant dilatation of the small intestine due to intra-abdominal free air and pneumatosis cystoides intestinalis but no mechanical obstruction, leading to a diagnosis of SSc with pseudo-obstruction. The patient underwent decompression with a long intestinal tube, which led to improvement in her symptoms. Although erythromycin (EM) and some prokinetic agents were administered, abdominal involvement recurred several days after resumption of oral ingestion. Therefore, we changed the antibiotic from EM to metronidazole (750 mg/day). Her manifestations were promptly ameliorated by metronidazole therapy in 4 days and did not recur. Metronidazole is an antibiotic used to treat intra-abdominal anaerobic bacterial infections and is also commonly used in preoperative treatment for colorectal surgery. In conclusion, we report a case where SSc-associated pseudo-obstruction was successfully managed by metronidazole therapy.

A 56-year-old-woman presented a local otolaryngologist with a complaint of hearing loss. She was treated with antibiotics as acute otitis media, however her symptom did not improved. She admitted to our hospital because of hearing loss on both sides, fever, otorrhea and vertigo. On admission, an audiogram showed bilateral mixed conductive-sensorineural hearing loss, and CT image revealed the exudates in bilateral middle ear cavities and mastoid air cells. Moreover, serum level of myeloperoxidase anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) elevated (133EU). Although pulmonary, renal and cutaneous involvements were not noted and the histopathological examination of operated specimen taken from otitis media revealed non-specific inflammatory changes, in the absence of any other obvious causes of otitis media, these findings might be associated with positive serum MPO-ANCA value itself. After the initiation of therapy with methylprednisolone and azathioprine, her symptoms and hearing ability ameliorated and both CRP value and the titer of ANCA became normalized. But, after the improvement by the immunosuppressive treatment, MRSA in the otorrhea persisted. This case suggests that otitis media may be one of the symptoms of vasculitis, and some previous cases described otitis media or hearing loss as rare manifestations of vasculitis. It is important to make an early diagnosis for good prognosis of hearing ability, and we have to consider the differential diagnosis including of ANCA-related vasculitis.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor of gastrointestinal tract, and bleeding is among the primary symptoms. If the tumor is located in the small intestine, diagnosis might be difficult because of its inaccessibility by endoscope. This report presents a case of duodenal GIST diagnosed with the aid of diffusion-weighted (DW) magnetic resonance imaging (MRI). A 66-year-old male was admitted to the hospital because of shortness of breath and melena. The patient demonstrated marked anemia. Esophagogastroduodenoscopy and colonoscopy revealed no involvement of the mucosa of the gastrointestinal tract in regard to the observed bleeding. Thereafter, computed tomography was done and showed a tumorous lesion 4.2 cm in diameter located around the ligament of Treitz. The area showed significant high intensity by DW-MRI, strongly suggesting malignant character. Examination with a long endoscope demonstrated a large submucosal tumor with wide ulceration located at the third portion of the duodenum. The biopsy sample contained spindle-shaped cells that were positive for CD34 and c-kit on histochemistry, and the tumor was diagnosed as GIST. The patient underwent surgery and survived for 16 months without any recurrence.

New covalently C60-connected zinc phthalocyanine (ZnPc) derivatives have been synthesized by utilizing successive cycloaddition reactions of C60 with a ZnPc derivative containing a pyridazine moiety employing Komatsu's method in reaction of C60 with phthalazine. The UV/Vis absorption spectrum of the fused conjugate (5) shows red shifts from the corresponding absorption of ZnPc derivative (8), indicating interactions between the ZnPc and C60 moieties. The DFT calculations under non-polar medium predict that the HOMO and LUMO of 5 localize on the ZnPc moiety, whereas LUMO+1 localizes on the C60 moiety, which reasonably explain the magnetic circular dichroism (MCD) and absorption spectra in toluene. Electrochemical redox potentials of 5 in polar solvents indicate the first-oxidation potential arises from the ZnPc moiety, whereas the first reduction potential is associated with the C60 moiety, suggesting the LUMO localizes on the C60 moiety in polar solvent. This reversal of the LUMO is supported by the ZnPc-fluorescence quenching with a nearby C60 moiety in benzonitrile, which leads to the charge-separation via the excited singlet state of the ZnPc moiety. In toluene on the other hand, such a ZnPc-fluorescence quenching owing to the photoinduced charge separation is not observed as predicted by the DFT-calculated LUMO on the ZnPc moiety.

A 63-year-old woman was admitted to our hospital because of auricular chondritis, conjunctivitis, polyarthralgia, productive cough and dyspnea. On admission, pulmonary function test demonstrated an obstructive pattern, and flow-volume curve (FVC) revealed a constrictive upper airway flow pattern. Chest CT showed a thickened tracheal wall and narrowing of the airway. The laboratory findings revealed an elevation of CRP and high titer of anti-type II collagen antibody. She was diagnosed as relapsing polychondritis (RP) according to Damiani's criteria. After the initiation of the therapy with 32 mg/day of methylprednisolone, her symptoms, pulmonary function, FVC and CT findings ameliorated promptly, and the titer of anti-type II collagen antibody became normalized. Moreover, we measured the airway wall thickness, percentage wall area (WA%) and percentage wall thickness (WT%), by CT and HRCT, and also evaluated the airway involvement quantitatively. Both WA% and WT% were inversely correlated with FEV1.0%. The airway inlolvement is most important prognostic factor in patients with RP, and sequential evaluation of airway manifestation are necessary. We suggest that a quantitative evaluation of bronchial structures by sequential CT is useful for the evaluation of RP as well as pulmonary function tests.

Mixed condensation of tetramethylsuccinonitrile and either 2,3-dicyano-5,6-diethylpyrazine, 2,3-dicyanopyridine, or 3,4-pyridinedicarboximide in the presence of nickel chloride forms novel pyrazine-, 2,3-pyridine-, or 3,4-pyridine-ring-fused tetraazachlorin (TAC), tetraazabacteriochlorin (TABC), and tetraazaisobacteriochlorin (TAiBC) derivatives. All possible structural isomers were separated using repeated thin-layer chromatography and have been investigated by absorption and magnetic circular dichroism spectroscopy. Similarly to previously reported TAC analogues, the TAC and TABC derivatives show large splitting of the Q band, while a single, intense absorption band is observed for the TAiBC derivatives. Although the absorption spectra are practically identical in shape for the separated structural isomers of TACs and TABCs, the Q-band maxima of the TAiBCs depend significantly on their structures. The observed spectroscopic properties were interpreted on the basis of electrochemical data and the results of (time-dependent) DFT calculations.

Primary Sjögren’s syndrome (SS) is an autoimmune disease characterized by inflammatory mononuclear cell infiltration and destruction of epithelial cells of lacrimal and salivary glands. Sphingosine 1-phosphate (S1P) and signaling through its receptor S1P1 have been implicated in many critical cellular events including inflammation, cancer, and angiogenesis. This study was undertaken to examine the role of S1P1 signaling in the pathogenesis of primary SS. S1P1 and sphingosine kinase 1, which converts sphingosine to S1P, were detected in the cytoplasm of inflammatory mononuclear cells, vascular endothelial cells, and epithelial cells in all labial salivary glands by immunohistochemistry. The expression of S1P1 in inflammatory mononuclear cells was enhanced in advanced stages of primary SS. S1P enhanced proliferation and IFN-γ production by CD4+ T cells. The enhancing effect of S1P on IFN-γ production by CD4+ T cells was stronger in patients with primary SS than in healthy controls. S1P also enhanced Fas expression and Fas-mediated caspase-3 induction in salivary gland epithelial cells. IL-6 expression was detected in the cytoplasm of inflammatory mononuclear cells and ductal epithelial cells and was enhanced in advanced stages of primary SS. Furthermore, both IFN-γ and S1P augmented IL-6 secretion by salivary gland epithelial cells. These effects of S1P were inhibited by pretreatment of pertussis toxin. Our data reveal that S1P1 signaling may modulate the autoimmune phenotype of primary SS by the action of immune as well as epithelial cells.

症例は75歳，女性．65歳時に胃癌にて幽門側胃切除術を受けた．微熱と肝機能障害で近医より紹介受診．来院時，肝胆道系酵素の上昇を認めた．ウイルス性肝炎および自己免疫性肝炎は否定的だった．単純CTで肝および脾のCT値が著明に上昇し，MRIでT1W, T2Wともに著明な低信号を認め，鉄沈着症を疑った．他臓器への鉄沈着を示唆する所見はなかった．血清鉄は210 μg/dlと高値，血清フェリチン値も6600 ng/mlまで上昇していた．生検した肝組織では，Kupffer細胞のみならず肝細胞内にも著明な鉄の沈着を認めた．患者に輸血歴は無かったが，胃癌術後の鉄欠乏性貧血に対し，鉄剤を断続的に約30カ月間静注された既往があり，その前後でのCT所見に変化を認めた．二次性鉄過剰症（続発性ヘモクロマトーシス）と診断し，デフェロキサミン500 mgの筋肉注射を開始，その後瀉血療法を併用し血清フェリチン値は1199 ng/mlまで改善した．鉄剤静注継続の際に注意すべき病態である．

Ablation of ventricular tachycardia originating from the great cardiac vein involves the difficult step of deep engagement with an ablation catheter. The catheter and a steerable sheath (MobiCath, Biosense Webster, Diamond Bar, CA, USA) were advanced alternately only when the contact force vector was parallel to the coronary venous system. Deep engagement with a steerable sheath ensured a powerful backup force during ablation.

Introduction: High intensity statin therapy is established for secondary prevention of coronary heart disease (CHD). However, additional therapy is required to reduce residual risk in CHD patients with aggressive lipid-lowering therapy. Eicosapentaenoic acid (EPA) was reported to be beneficial especially in secondary prevention. The aim of this study was to investigate whether coronary plaque regression and stabilization are reinforced by additional administration of EPA to high dose pitavastatin (PTV) therapy. Methods: We enrolled 200 CHD patients who underwent percutaneous coronary intervention in 6 hospitals. Patients were randomly allocated to PTV group (PTV 4 mg/day, n=98) and PTV/EPA group (PTV 4 mg/day and EPA 1800 mg/day, n=102), and prospectively followed for 6 to 8 months. Coronary plaque volume and composition in non-stenting lesion were analyzed by integrated backscatter intravascular ultrasonography at baseline and follow up. Results: EPA / arachidonic acid (AA) ratio was significantly increased in PTV/EPA group compared to PTV group at follow up period. Plaque volume and lipid volume were significantly reduced in PTV/EPA group, but not PTV group. There was a significant inverse correlation between change in EPA/AA ratio and changes in plaque volume (r=-0.332, p Conclusions: Additional administration of EPA to high dose PTV therapy significantly reduced coronary plaque volume, suggesting that combination therapy of EPA and PTV might reduce residual risk in CHD patients with aggressive lipid-lowering therapy.