Objective The HELT-E₂S₂ score is a novel risk assessment tool for ischemic stroke in patients with atrial fibrillation. We explored the prognostic impact of the HELT-E₂S₂ score on cardiovascular outcomes in patients with heart failure (HF), beyond its original scope.

Methods We enrolled 997 patients hospitalized for acute decompensated HF (ADHF) (median age, 81 years; 44.3% female) from the Clue of Risk Stratification in the Elderly Patients with Heart Failure (CURE-HF) registry. We evaluated the relationship between the HELT-E₂S₂ score and all-cause death over a median follow-up of 2.7 years.

Results The patients were categorized into four quartiles based on the HELT-E₂S₂ score: score 0 or 1 (group 1, n=234), score 2 (group 2, n=244), score 3 (group 3, n=255), and score ≥4 (group 4, n=264). Atrial fibrillation was present in 592 (59.4%) patients (paroxysmal, 15.0%; persistent, 44.3%). All-cause death was observed in 384 patients (14.7 per 100 patient-years). A Kaplan-Meier analysis showed that the higher score group was associated with an increased risk of all-cause death (log-rank p<0.001). After multivariable adjustment, the higher score groups had an elevated risk of all-cause death compared to group 1 [group 2; hazard ratio (HR): 1.56; 95% confidence interval (CI): 1.03-2.37; p=0.037, group 3; HR: 2.74; 95% CI: 1.85-4.07; p<0.001, group 4; HR: 3.32; 95% CI: 2.34-4.94; p<0.001].

Conclusion Higher HELT-E₂S₂ scores were associated with a higher risk of all-cause death in patients with ADHF.

Objective Malnutrition is a common problem among patients with chronic kidney disease (CKD). It is unknown whether serum cholinesterase, an indicator of the nutritional status, has a significant value as a prognostic marker in patients with non-dialysis-dependent CKD, including those in the early phase.

Methods A total of 4,505 middle-aged and older people with stage 1-4 CKD who were free from cardiovascular events (mean age: 67.2 years) were followed up for an average of 10.3±2.4 years. We investigated the association of serum cholinesterase levels with all-cause mortality using multivariable regression analyses and restricted cubic spline analyses.

Results A total of 840 (18.6%) participants died. The multivariable-adjusted hazard ratios (HRs) for all-cause mortality were significantly increased in patients with serum cholinesterase in the first and second quartiles compared to patients in the highest quartile in the entire cohort [HR=1.61, 95% confidence interval (CI), 1.28-2.03; HR=1.37, 95% CI, 1.09-1.71, respectively]. A reverse-J relationship between the serum cholinesterase levels and all-cause mortality was found in the entire cohort as well as in patients grouped according to stage 1-2 CKD and stage 3-4 CKD (all p for nonlinearity: <0.030). The inclusion of the serum cholinesterase level improved the accuracy of reclassification in a conventional prediction model for 10-year all-cause mortality (continuous net reclassification improvement=0.167, p<0.001; integrated discrimination improvement=0.005, p<0.001).

Conclusion In middle-aged and older people with stage 1-4 CKD, the serum cholinesterase level had significant value in predicting all-cause mortality and added prognostic information to conventional risk assessments.

Objective The number of patients using direct oral anticoagulants (DOACs) has recently increased. However, the association between DOAC use and bronchoscopy (BS) safety has not been elucidated. This study examined the association between BS and DOAC use and safety.

Methods Using data from the Japanese Diagnosis Procedure Combination database from July 1, 2010, to March 31, 2022, patients taking DOACs who underwent BS (n=603, DOAC group) and those who did not (n=187,827, non-DOAC group) were identified. Then, 1:4 matched-pair cohort analyses were performed based on the institution, sex, age, and treatment year. The primary outcome was all-cause in-hospital mortality. The secondary outcomes were 28-day mortality, mechanical ventilation use, pneumothorax, bleeding, and thromboembolism after BS.

Results A total of 603 and 2,320 patients taking and not taking DOAC, respectively, were identified via matching. The all-cause in-hospital mortality rates in the DOAC and non-DOAC groups were 12.1% and 5.8%, respectively. In a multivariable logistic regression analysis, the DOAC group had a significantly higher all-cause in-hospital mortality rate than the non-DOAC group (odds ratio = 2.84, 95% confidence interval = 1.77-4.55). Secondary and composite outcomes (mechanical ventilation, pneumothorax, bleeding, and thromboembolism after BS combined) were more frequent in the DOAC group than in the non-DOAC group.

Conclusion DOACs were associated with mortality and complications in patients undergoing BS. Further studies comparing BS outcomes between patients with and without DOAC treatment are necessary.

Objective The immature platelet fraction (IPF) reflects the thrombopoietic activity and it is useful for diagnosing thrombocytopenic disorders. TAFRO syndrome is a fatal disease that requires an accurate diagnosis and urgent treatment. Thrombocytopenia is essential for the diagnosis of TAFRO syndrome; however, it can develop later during the course of the disease. The significance of IPF and the frequency of late-onset thrombocytopenia in TAFRO syndrome remains unclear. This study investigated the relationship between the platelet count and IPF as well as the absence of thrombocytopenia upon admission for TAFRO syndrome.

Methods We herein present the follow-up of our previously reported case and two other cases of TAFRO syndrome with serial IPF measurements performed at our hospital. We reviewed cases published between 2010 and 2024 from the PubMed database. This study included patients diagnosed according to the criteria for TAFRO syndrome proposed in 2019.

Results Of the 167 eligible patients, pretreatment IPF was measured in 11, including four with serial measurements. In these 11 patients, the median pretreatment IPF was 18.2% (range 9.8-32.0) and that of the corresponding platelet count was 67,000/μL (range 8,000-179,000). Pretreatment IPF was elevated (≥ 10%) in 10 patients (90.9%), including four without thrombocytopenia. There was no significant correlation between the platelet count and IPF. Thirty-four of 167 patients (20.3%) did not have thrombocytopenia upon admission.

Conclusion The pretreatment IPF levels were elevated in most of the patients. Late-onset thrombocytopenia was common. An increased pretreatment IPF level may be a diagnostic indicator of TAFRO syndrome, particularly in patients without thrombocytopenia on admission.

Objective To analyze the influence of age of the onset on myositis organ damage and to identify the factors influencing myositis organ damage, as clinical manifestations of myopathies differ by the age of onset and the background of patients.

Methods Factors influencing organ damage [the Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI)] were identified using the Japanese multicenter myositis registry (MYKO, n=220). Factors influencing organ damage were identified using a multivariate analysis. SDI was compared among juvenile-onset (<20 years old), adolescent-onset (20-64 years), and elderly-onset (>64 years) groups.

Results There was a correlation between the age at onset and the SDI score (Spearman's rank correlation coefficient ρ=0.28). Elderly patients exhibited more widespread organ damage, including neuropsychiatric, renal, pulmonary, cardiovascular, peripheral vascular, gastrointestinal, skin, and diabetes, whereas juvenile-onset patients exhibited musculoskeletal damage. Adolescent-onset patients had the lowest incidence of ocular and malignant damage. A regression analysis revealed that an older onset age (coefficient, β=0.03), longer disease duration (β=0.05), and total dose of glucocorticoid (β=3.35×10^-5) influenced SDI. After adjusting for disease duration, the influences of anti-melanoma differentiation-associated gene 5 (MDA5) [hazard ratio (95% confidence interval), 4.47 (2.17-9.21)] on pulmonary fibrosis and a history of steroid pulse [2.16 (1.16-4.05)] on muscle atrophy or weakness were shown.

Conclusion There were associations between the age of onset and autoantibodies with myositis organ damage. Musculoskeletal damage was greater in patients with a juvenile onset. An older age of onset is associated with severe organ damage. These findings highlight the importance of considering the age of onset and autoantibodies for assessing the prognosis and developing treatment plans for myopathies.

Amyloidosis is a rare cause of common bile duct (CBD) stricture that often presents with nonspecific symptoms. We herein report the case of a 61-year-old woman with CBD stricture caused by amyloidosis diagnosed through cholangioscopy and confirmed by a biopsy, with subsequent findings identifying it as AA-type amyloidosis. This condition poses diagnostic challenges owing to its rarity and non-specific manifestations. When common causes are excluded, amyloidosis should be considered in the differential diagnosis of CBD stricture. Advanced imaging and cholangioscopy with targeted biopsies are essential for making an accurate diagnosis. Documenting such cases and further research are vital to improve the understanding and management of amyloidosis-related biliary stricture.

Although glucocorticoids are commonly used to suppress allergic reactions, systemic glucocorticoid-induced anaphylaxis has also been reported. A man with acute hepatitis B was administered intravenous methylprednisolone to prevent the progression to liver failure. Shortly thereafter, the patient developed a rash and hoarseness, and methylprednisolone-induced anaphylaxis was diagnosed. The injection was stopped, an antihistamine was administered, and the symptoms resolved quickly. Subsequently, antiviral therapy and 9.9 mg/day of dexamethasone were initiated. The patient recovered completely without persistent hepatitis B virus infection. This case highlights the importance of recognizing glucocorticoid-induced anaphylaxis and provides insights into alternative treatment strategies for acute hepatitis B.

Mass-forming pancreatitis and lipodystrophy are rare immune-related adverse events (irAEs). A 71-year-old woman received 12 cycles of pembrolizumab for recurrent uterine endometrial cancer. The patient presented with mass-forming pancreatitis and lipodystrophy. A needle biopsy of the pancreatic tissue revealed lymphocytic infiltration and fibrosis. Simultaneously, the patient exhibited lipodystrophy. The recurrent tumors disappeared, so the patient discontinued immune checkpoint inhibitor treatment and was administered oral prednisolone. In addition, the pancreatic mass disappeared; however, the fat loss did not improve. Considering pancreatitis and lipodystrophy to be irAEs, prompt management is important.

Screening for colorectal cancer (CRC) in individuals with Li-Fraumeni syndrome (LFS) typically recommends initiating colonoscopy at 25 years old. We herein report a case of LFS with dual CRC and liver metastasis diagnosed before the recommended screening age. A 17-year-old boy with a history of sarcoma underwent colonoscopy, revealing 2 advanced CRCs: neuroendocrine carcinoma in the descending colon and adenocarcinoma in the rectum, each displaying distinct histological features. In addition, a TP53 missense mutation (p.His193Arg) was detected by a detailed genetic examination. LFS is associated with a heightened risk of rapid progression to invasive carcinomas due to TP53 variants. Therefore, earlier initiation of colonoscopy screening may be necessary for patients with LFS.

Doublet chemotherapy with encorafenib and cetuximab has demonstrated a survival benefit for BRAF V600E mutation-positive advanced colorectal cancer, which generally has a poor prognosis, as shown in the BEACON CRC trial. We encountered a case of rectal cancer with this mutation, complicated by grade 3 pancreatitis, one month after starting this doublet therapy. Despite aggressive treatment efforts, the pancreatitis led to duodenal ulcer perforation and a fatal intra-abdominal abscess. This case highlights pancreatitis as a rare, but severe, side effect of combination chemotherapy.

A 56-year-old woman with high-grade or invasive intraductal papillary mucinous neoplasm (IPMN) underwent 3 pancreatic surgeries for initial and recurrent IPMN lesions. Immunostaining and genetic analyses of all lesions revealed a consistent mutation in KRAS and loss of serine/threonine kinase 11 (STK11), indicating the same origin of these lesions and the possibility of intraductal dissemination. Other immunostaining findings, such as mucin expression and a high Ki-67 labeling index, were consistent among all the lesions and supported their malignant potential. IPMNs with loss of STK11 may benefit from tailored follow-up of the remnant pancreas due to their high likelihood of recurrence through intraductal spread.

Patients with anti-mitochondrial antibody positivity rarely develop tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN). A previous report observed elevated serum IgM and urinary β2 microglobulin levels in all cases of IgMPC-TIN. This case involved IgA nephropathy with latent coexisting IgMPC-TIN; however, no such changes were observed. These results suggest that some patients with primary biliary cholangitis may have latent IgMPC-TIN without specific urine or blood results. Furthermore, the patient simultaneously developed IgA nephropathy and IgMPC-TIN, suggesting that the triggers and exacerbating factors for both conditions share a common underlying cause.

Renal salt-wasting syndrome (RSWS) induced by cisplatin causes hyponatremia and dehydration. A 75-year-old woman with thymoma underwent cisplatin chemotherapy. She presented with hypovolemic shock, seizures, and impaired consciousness (Japan Coma Scale II-30) with a decreased serum sodium level of 116 mmol/L on day 9. In addition, the patient had hypokalemia, hypomagnesemia, hypocalcemia, and hypophosphatemia. We herein describe the first reported case of RSWS complicated by hypovolemic shock. This is the second reported case with multiple electrolyte abnormalities. Thus, we should recognize that severe RSWS with hypovolemic shock and multiple electrolyte abnormalities can occur in patients undergoing cisplatin treatment.

A 79-year-old woman was admitted to our hospital with a persistent fever, malaise, and anemia requiring frequent transfusions. A peripheral blood smear revealed erythrocyte agglutination and a high titer of cold agglutinins. Imaging examination revealed pulmonary emboli and multiple subacute cerebral infarctions. Despite the absence of lymphadenopathy or splenomegaly, the diagnosis of intravascular large B-cell lymphoma (IVLBCL) was confirmed through random skin biopsies. Immunochemotherapy successfully induced remission of her fever and anemia with normalization of her cold agglutinin titer. This case highlights that cold agglutinin syndrome can be associated with aggressive lymphomas, including IVLBCL.

Amyloid-related imaging abnormalities (ARIAs), including edema/effusion (ARIA-E) and hemosiderin deposition (ARIA-H), are complications of anti-amyloid β (Aβ) monoclonal antibody therapy for Alzheimer's disease (AD). A Japanese woman in her 70s with mild cognitive impairment due to AD, confirmed by positive amyloid positron emission tomography, developed a new lobar cerebral microbleed (CMB) after participating in aducanumab clinical trials. Magnetic resonance imaging revealed juxtacortical white matter hyperintensities on fluid-attenuated inversion recovery, which were later resolved as a novel CMB. Juxtacortical white matter hyperintensities may precede ARIA-H and may suggest cerebral amyloid angiopathy-related changes during anti-Aβ therapy. Monitoring of these lesions is essential.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disease of the central nervous system, and many details, including pathological findings, are still unclear. We herein report a man in his 70s who had been diagnosed with CLIPPERS 4.5 years ago. He was treated with corticosteroids, and his symptoms were managed; however, he eventually died of coronavirus disease 2019. A postmortem autopsy revealed perivascular lymphocyte aggregation and demyelination centered in the brainstem and cerebellum. This is the world's first autopsied case of CLIPPERS with more than four years of follow-up.

The coexistence of sporadic Creutzfeldt-Jakob disease (sCJD) and intracerebral hemorrhaging (ICH) has rarely been reported, and the relationship between these conditions remains unclear. This report presents the case of a 49-year-old woman without any hemorrhagic risk factors who developed subcortical hemorrhaging in the early stages of sCJD. This case suggests that sCJD is associated with ICH, particularly in cases of subcortical hemorrhaging.

A 43-year-old woman with systemic lupus erythematosus on immunosuppressive therapy developed right hemiparesis and motor aphasia five months after the onset of herpes zoster. Brain magnetic resonance imaging revealed an infarction in the left anterior cerebral artery and severe middle cerebral artery (MCA) stenosis. Despite antiplatelet therapy, recurrent strokes occurred, and vessel wall imaging (VWI) showed circumferential thickening of the MCA, suggesting vasculitis. Cerebrospinal fluid revealed varicella-zoster virus (VZV) deoxyribonucleic acid (DNA) and an elevated anti-VZV IgG index, consistent with VZV vasculopathy. The administration of acyclovir and corticosteroids eliminated VZV DNA. This case highlights the risk of VZV vasculopathy in immunocompromised patients and the usefulness of VWI for its diagnosis.

A 60-year-old woman with a history of malignant lymphoma was referred to us with fever, sore throat, right-sided neck pain, and swelling while she was currently undergoing chemotherapy. Imaging studies, including computed tomography and ultrasonography of the neck, revealed significant perivascular wall thickening of the right carotid artery, which was suggestive of giant cell arteritis. However, fever, neck pain, and swelling resolved spontaneously and rapidly. Transient perivascular inflammation of the carotid artery (TIPIC) syndrome was most likely. However, these symptoms developed shortly after the administration of granulocyte colony-stimulating factor (G-CSF). Accordingly, the patient was diagnosed with G-CSF-induced aortitis mimicking TIPIC syndrome.

Non-traumatic sternoclavicular arthritis is typically caused by either degeneration or non-infectious inflammatory conditions, although septic arthritis can often occur due to Staphylococcus aureus, particularly in immunocompromised individuals.

We herein present a case of Escherichia coli (E. coli)-induced sternoclavicular arthritis in a Japanese woman with no underlying disease. As no common source of E. coli bacteremia was identified, colonoscopy revealed early-stage rectal cancer.

To our knowledge, this is the first report of septic sternoclavicular arthritis in which colorectal cancer was identified as the source of infection.

Clinicians should consider colorectal cancer when septic arthritis caused by gut bacteria is diagnosed without a clear source.

Exophiala spp. cause dematiaceous mycoses, but rarely infect human lungs. A 61-year-old man presented with bloody black sputum. Chest computed tomography showed consolidation in the truncus intermedius and peripheral bronchus, and a mass shadow in the S₇ region of the right lung. Bronchoscopy revealed a protruding black lesion in the bronchial orifice of the truncus intermedius, and Exophiala phaeomuriformis was isolated. The consolidation resolved spontaneously, and the residual tumor was diagnosed as a carcinoid tumor. This case highlights the fact that pulmonary chromomycosis may occur in immunocompetent individuals without any preexisting structural changes in the airways.

The patient was an 89-year-old woman who suddenly collapsed in a hospital cafeteria. Responding medical personnel determined that the patient was in cardiac arrest. After advanced cardiac support, the patient regained spontaneous circulation. Chest compressions were continued for approximately 15 min, and the computed tomographic scan revealed kyphosis, diffuse idiopathic skeletal hyperostosis, an extension fracture of the second lumbar vertebra, and a large right Gerota's intrafascial hematoma. This case illustrates the need to consider alternative methods of chest compression to prevent spinal extension fractures and related complications when an elderly individual with a kyphotic spine experiences cardiac arrest.