Objective We aimed to stratify the risk of major cardiovascular (MCV) events in Japanese patients with known or suspected coronary artery disease (CAD) who had normal single-photon emission computed tomographic myocardial perfusion imaging (SPECT MPI) findings and to compare the risk by generation.

Methods This was a retrospective study. The composite endpoint was the occurrence of cardiovascular death, non-fatal myocardial infarction, or non-fatal stroke.

Patients The study subjects were 2,035 patients with normal SPECT MPI findings at baseline who had been followed up to confirm their prognosis for 3 years. The patients were categorized into 3 age groups: very elderly (≥80 years old, n=311), elderly (65-79 years old, n=1,097), and younger (<65 years old, n=542).

Results During the follow-up, 68 patients experienced MCV events: cardiovascular death (n=29), non-fatal myocardial infarction (n=15), and non-fatal stroke (n=24). The MCV event rate was significantly higher in very elderly patients than in other patients. Multivariate predictors were age categories, the estimated glomerular filtration rate, atrial fibrillation, and stress left ventricular ejection fraction. The MCV event rate was 6.1% in very elderly patients. However, the MCV event rate in those with normal cardiac and renal functions without atrial fibrillation was 3.3%, which was similar to that in elderly and younger patients.

Conclusion The MCV event rate was high in very elderly patients despite their normal SPECT MPI findings at baseline. Therefore, very elderly patients with multivariate risks should be carefully followed to avoid a poor prognosis.

Objective Delays in insulin initiation can lead to the development of complications in the management of type 2 diabetes.

Methods In this study, the effects of the timing of insulin initiation on glycemic control in patients with type 2 diabetes were evaluated retrospectively. Changes in the HbA1c levels of 237 patients were analyzed after insulin initiation.

Results The patients were divided into 4 groups according to the duration of diabetes at the time of insulin initiation: ≤3 years, 4 to 6 years, 7 to 9 years, or ≥10 years. Patients with a diabetes duration of ≤3 years were more frequently hospitalized at the time of insulin initiation, had a higher HbA1c level before insulin initiation and a lower HbA1c level at 1 year after insulin initiation and exhibited significant decreases in HbA1c at 1, 3, or 5 years after insulin initiation than those in the other 3 groups with longer durations of diabetes. In the group receiving 4 insulin injections per day, the reduction in HbA1c after 5 years of treatment was larger in patients with a diabetes duration at the time of insulin initiation of ≤3 years than in those with a duration of 7 to 9 years or ≥10 years.

Conclusion Our results suggested that an earlier initiation of insulin therapy was crucial for sustaining glycemic control in Japanese patients with type 2 diabetes, particularly in those with a history of obesity or receiving multiple insulin injections daily.

Objective Limbic encephalitis (LE) is an inflammatory condition of the limbic system that has an acute or subacute onset. Several types of antibodies are related to the onset of LE, including anti-N-methyl D-aspartate receptor (NMDAR) antibodies and voltage-gated potassium channel (VGKC)-complex antibodies. However, the characteristics and prevalence of LE remain unclear, especially in Asian cohorts, due to the rarity. We aimed to survey their characteristics.

Materials and Methods Data of 30 cases clinically defined as "definite autoimmune LE" (based on the standard criteria) were retrospectively collected. These patients were categorized into four subtypes: NMDAR (+) (n=8), VGKC (+) (n=2), antibodies related to paraneoplastic syndrome (n=2), and an antibody-negative group (uncategorized) (n=18).

Results LE is rare in Japan, and affected only 30 of 16,759 hospital patients (0.2%) over a ten-year period. The NMDAR (+) group showed distinctive symptoms, while the other three groups had similar indications. Brain MRI indicated significant medial temporal lobe atrophy at one year follow up after discharge. The prevalence of cognitive dysfunction as a complication was 64% (9/14). First-line immunotherapy resulted in a good outcome. A drastic improvement was seen from 4.0±1.1 to 1.1+ on the modified Rankin Scale. A good treatment outcome was observed in all groups (NMDAR, VGKC, and uncategorized), suggesting the importance of an early clinical diagnosis and the early initiation of treatment. Furthermore, we reviewed 26 cases that were clinically diagnosed as definitive autoimmune LE in previous case reports.

Conclusion Our findings show that the establishment of a clinical diagnosis based on the clinical criteria of definitive autoimmune LE is important for the initiation of immunotherapy.

Objective Pneumocystis pneumonia (PCP) is a serious fungal infection that can be life threatening in immunocompromised hosts. We evaluated the association between the radiological patterns of PCP on high-resolution computed tomography (HRCT) and clinical characteristics and the prognosis of patients with connective tissue disease (CTD).

Methods All CTD patients who developed PCP from January 1999 to April 2017 were retrospectively evaluated. Patients were divided into three groups based on their chest HRCT findings: Ground glass opacity (GGO) sharply demarcated from the adjacent normal lung by interlobular septa (demarcated GGO), diffuse GGO without obvious demarcation (diffuse GGO), and GGO with mixed consolidation (mixed GGO). We compared the clinical characteristics at the onset of PCP and the outcomes among the groups.

Results A total of 35 cases were identified: demarcated GGO (n=8, 23%), diffuse GGO (n=19, 54%), and mixed GGO (n=8, 23%). The mixed GGO group showed a higher serum C-reactive protein level (p<0.0001), lower lymphocyte count (p=0.07), lower serum albumin (p<0.001), and lower partial pressure of arterial oxygen/fraction of inspiratory oxygen ratios (p<0.001) in comparison to the demarcated and diffuse GGO groups. The mixed GGO group showed significantly higher mortality in comparison to the demarcated and diffuse GGO groups (88% vs. 7%, p<0.0001).

Conclusion GGO with mixed consolidation on chest HRCT was associated with a poor outcome of PCP in patients with CTD.

Objective The hospitalist system is considered successful with respect to the quality of care and cost effectiveness in the United States. Studies have consistently demonstrated an improved clinical efficiency with this system. In Japan, however, the efficacy of the hospitalist system has not yet been examined. As a "super-aged society", Japan has a high number of elderly patients with multiple comorbidities who may theoretically receive better care by the hospitalist system than by the conventional system. This study investigates the impact of the hospitalist system on the quality of care and healthcare economics in a Japanese population.

Methods We analyzed 274 patients ≥65 years of age in whom the most resource-consuming diagnosis at admission was aspiration pneumonia over a 1-year period. We categorized patients as those managed by hospitalists and those managed by various departments (control group) and compared the groups. Propensity score matching was used to minimize selection bias.

Results For matched pairs, the length of hospital stay in the hospitalist group was shorter than that in the control group. Care by the hospitalist system was associated with significantly lower hospital costs. The quality of care (rate of switching from intravenous to oral antibiotics, duration of antibiotics therapy, number of chest X-rays and blood tests during hospitalization) was also considered to be favorably impacted by the hospitalist system. There was no statistically significant difference in the mortality rate or readmission rate between the groups.

Conclusion This study showed that the hospitalist system had a favorable impact on the quality of care and cost effectiveness, suggesting the potential utility of its implementation in the Japanese medical system.

Fibromuscular dysplasia (FMD) and segmental arterial mediolysis (SAM) are noninflammatory, nonatherosclerotic arterial diseases that cause aneurysm, occlusion, and thromboses. These diseases are rarely seen in mesenteric arterial lesions; however, as they can be lethal if appropriate management is not provided, the accumulation of clinical information from cases is essential. We herein report the cases of a 57-year-old man diagnosed with FMD and a 63-year-old man diagnosed with SAM. We conclude that an early diagnosis with imaging modalities and clinical information followed by the appropriate treatment improves the prognosis of these arterial diseases.

Colonic varices are usually associated with portal hypertension. Idiopathic colonic varices are extremely rare. A 68-year-old man with a positive fecal occult blood test result underwent colonoscopy. We detected idiopathic ileocolonic varices and a coexisting ascending colon polyp. While reviewing the literature, we found cases of biopsies and polypectomies resulting in significant bleeding. We herein report a case of idiopathic ileocolonic varices coexisting with a colon polyp treated successfully by endoscopy. The coexistence of colonic varices and a colorectal lesion that requires endoscopic treatment may lead to significant bleeding. During management, the development of a treatment strategy and obtaining informed consent are necessary.

We herein report a case of Brachyspira pilosicoli-caused severe colitis presenting with portal venous gas. A 75-year-old man was admitted because of a fever, severe abdominal pain and bloody diarrhea. He was negative for anti-HIV antibodies. He had been in close contact with a dog earlier. Abdominal computed tomography detected severe wall-thickening and fat-stranding of the entire colon accompanied by portal venous gas. A smear examination of his stool showed many Gram-negative spiral rods, suggesting intestinal spirochetosis. A polymerase chain reaction assay using stool samples detected an amplified band specific for B. pilosicoli. He responded well to antimicrobial agents including metronidazole.

In 2018, the CorPath GRX system (Corindus) was approved for use in Japan, marking the introduction of the first robotic-assisted system for percutaneous coronary intervention (PCI) in the country. The present report describes the first experience of robotic-assisted PCI for four coronary lesions in two cases in a single center. All procedures succeeded without any complications, although one procedure was converted to manual PCI by the operator's decision. Post-marketing surveillance to assess the impact of this novel system on both Japanese patients and physicians is currently ongoing in Japan.

Cryoballoon ablation is a well-established therapeutic tool for paroxysmal atrial fibrillation (PAF). We herein report a rare case of a 69-year-old man with PAF undergoing hemodialysis due to chronic kidney disease who developed hyperkalemia caused by possible cold agglutinin disease during cryoballoon ablation therapy. During the procedure, his electrocardiogram showed wide QRS when we finished cryoablation therapy. We detected hyperkalemia and performed urgent hemodialysis. We should bear in mind that cold agglutinin disease can occur during cryoballoon ablation.

Glucocorticoid therapy is effective for treating autoimmune pancreatitis, but autoimmune pancreatitis itself and steroid therapy aggravate glycemic control. A 77-year-old man with type 2 diabetes was consulted due to aggravation of glycemic control. He was diagnosed with autoimmune pancreatitis. We promptly started glucocorticoid therapy for autoimmune pancreatitis and insulin therapy for glycemic control. Subsequently, both pancreatitis and diabetes were markedly ameliorated. After stopping glucocorticoid therapy, good glycemic control continued with diet therapy alone. Starting glucocorticoid therapy at an early stage of autoimmune pancreatitis is very important for preserving the insulin secretory capacity and improving glycemic control.

We herein report a 28-year-old woman with type 1 diabetes with an asymptomatic pontine lesion and diabetic amyotrophy. She had suffered from diabetes from 10 years old. Treatment in a hospital reduced the hemoglobin A1c level from 14.2% to 7.2% for approximately 2 months. She suffered from acute-onset pain and weakness of the lower limb muscles without central nervous system manifestations. Magnetic resonance imaging showed high-intensity lesions at the brainstem and lower limb muscles on T2-weighted images. These findings and symptoms gradually resolved. Rapid treatment of poor glycemic control might increase the risk of asymptomatic pontine lesions and diabetic amyotrophy.

Type III collagen is the major protein in the walls of blood vessels and hollow organs; it is decreased in patients with vascular Ehlers-Danlos syndrome (EDS). A 52-year-old man was admitted for severe back pain, and right hemothorax was suspected by chest computed tomography. Immediately after embolization for bleeding bronchial artery, aortic dissection occurred and was treated conservatively in the intensive-care unit. Vascular EDS with a mutation of COL3A1 cDNA (c.3175G>A) was diagnosed. When vascular EDS is suspected, the patient should be treated prophylactically, and a genetic examination should be performed to confirm the diagnosis.

Myeloid/lymphoid neoplasms with PDGFRB rearrangement are a distinct type of myeloid neoplasms that occur in association with rearrangement of PDGFRB at 5q32. The hematological features most often show prominent eosinophilia. We herein report a patient with myeloid/lymphoid neoplasms with PDGFRB rearrangement with t (5;10) (q33;q22) who showed atypical chronic myeloid leukemia-like clinical features without eosinophilia and achieved an optimal response to imatinib. A sequence analysis showed a CCDC6-PDGFRB fusion gene with a new break point in the PDGFRB gene. This is the sixth case of myeloid/lymphoid neoplasm with PDGFRB rearrangement harboring a CCDC6-PDGFRB fusion gene, and it has a new breakpoint in the PDGFRB fusion gene.

We experienced a pregnant woman with superior vena cava syndrome at 15 weeks of pregnancy who was diagnosed with primary mediastinal large B-cell lymphoma and given chemotherapy. In this case, the clinical courses of both the mother and infant were favorable without any serious complications because of close multidisciplinary cooperation. Based on a retrospective review of this case, the administration of CHOP-like regimens during the second and third trimesters appears relatively safe. Because pregnancy and continuation of pregnancy are rare in patients with hematopoietic malignancies, the accumulation of detailed information is important.

A 72-year-old man presented with a 6-month history of systemic edema. Hyperpigmentation, hemangioma, pleural effusion, IgG-kappa-type monoclonal protein, high vascular endothelial growth factor values, renal failure, and nerve conduction study abnormalities were also present. Multiparameter flow cytometry (MFC) showed 0.2% neoplastic plasma cells (CD38-, CD56-, and kappa-positive; CD19-, CD27-, and lambda-negative) in the bone marrow leading to POEMS syndrome. Cases involving kappa-type POEMS syndrome are extremely rare. A kidney biopsy revealed membranous proliferative glomerulonephritis-like changes in our case. Lenalidomide-dexamethasone therapy improved the renal function. Detection of neoplastic plasma cells by MFC was useful for the accurate diagnosis and treatment evaluation.

An absence of skin lesions at the neurological onset may obscure the diagnosis of neuro-Sweet disease (NSD). We herein report a 32-year-old man with NSD in whom neurological symptoms preceded the development of skin lesions by 10 years. The patient exhibited four distinct neurological episodes: meningoencephalitis, scattered brain lesions, ocular flutter, and isolated seizures. Acute relapses responded to corticosteroid therapy, and the patient was successfully maintained on corticosteroid and dapsone combination therapy. NSD should be considered in the differential diagnosis of patients with recurrent neurological manifestations, especially with both meningeal and brain parenchymal involvement, even if no skin lesions are observed.

Adult-onset Still's disease (AOSD) sometimes demonstrates hematologic disorder, whereas acquired amegakaryocytic thrombocytopenia (AAT) involvement is extremely rare. We herein report a 67-year-old woman with relapse of AOSD who concomitantly developed AAT. Thrombocytopenia along with high disease activity of AOSD was resistant to high-dose prednisolone, even in combination with methotrexate and tacrolimus. However, alternative treatment with cyclosporine after administering tocilizumab resulted in the improvement of thrombocytopenia, ultimately demonstrating that combination therapy based on suppressing the intractable disease activity of AOSD and subsequently adding a reliable immunosuppressant was required to achieve remission.

A 62-year-old woman with no previous history developed a Capnocytophaga canimorsus infection followed by thrombotic microangiopathy (TMA) and disseminated intravascular coagulation (DIC). She was treated with antibiotics and plasma exchange (PE) and recovered. C. canimorsus sepsis sometimes causes not only DIC but also TMA. The mortality of TMA is extremely high, so we should not hesitate to perform PE when a patient shows TMA symptoms.