Tenri Hospital

We evaluated the prognostic significance and implications of a major pathologic response (MPR) after preoperative treatment for pancreatic ductal adenocarcinoma (PDAC). Preoperative treatment is increasingly used for PDAC to improve oncologic outcomes. The pathologic response represents a potential indicator of treatment efficacy; however, its prognostic value in PDAC remains unclear. We retrospectively analyzed 739 patients who underwent pancreatectomy for PDAC after preoperative treatment at 21 institutions in Japan. The pathologic response was graded using Evans' classification, with MPR defined as Evans grade III/IV. Survival outcomes and prognostic factors were evaluated, and factors associated with achieving MPR were analyzed to develop a predictive model. MPR was achieved in 11.5% of patients. The MPR group had a significantly longer median overall survival (71.5 vs. 40.9mo) and recurrence-free survival (55.5 vs. 15.2mo) than the non-MPR group. Multivariate analysis identified MPR as an independent prognostic factor for overall survival. In the MPR subgroup, neither overall nor recurrence-free survival differed according to adjuvant chemotherapy administration; multivariate analysis did not identify adjuvant therapy as an independent prognostic factor. Predictive factors for achieving MPR included chemoradiotherapy, preoperative duration ≥6 months, normal carbohydrate antigen 19-9 after preoperative treatment, and complete or partial radiologic response. Finally, we developed a simplified predictive model for achieving MPR. MPR was independently associated with favorable survival in PDAC. The prognostic impact of adjuvant chemotherapy was not observed among patients who achieved MPR, suggesting that MPR may inform individualized postoperative management and warrants prospective validation.

Primary Percutaneous Coronary Intervention (PCI) has significantly contributed to reducing the mortality of patients with ST segment elevation myocardial infarction (STEMI) even in cardiogenic shock and is now the standard of care in most of Japanese institutions. The Task Force on Primary PCI of the Japanese Association of Cardiovascular Intervention and Therapeutics (CVIT) proposed an expert consensus document for the management of acute myocardial infarction (AMI) focusing on procedural aspects of primary PCI in 2018 and updated in 2022 (Ozaki et al. in Cardiovasc Interv Ther 33:178-203, 2018), (Ozaki et al. in Cardiovasc Interv Ther 37:1-34, 2022). Following the publication of the 2023 European Society of Cardiology (ESC) Guidelines for the management of acute coronary syndromes, the CVIT Task Force released another revised version in 2024 (Ozaki et al. in Cardiovasc Interv Ther 39:335-375, 2024). In light of new clinical evidence and technological advances that have emerged since then, the Task Force now proposes an updated expert consensus document for the management of ACS focusing on procedural aspects of primary PCI in 2026 version.

Anti-synthetase syndrome is an autoimmune disorder associated with anti-aminoacyl-tRNA synthetase antibodies, including anti-Jo-1. However, antibody positivity alone is insufficient for the diagnosis because overlapping features with other diseases may lead to a misdiagnosis. We herein report a 69-year-old woman initially suspected of having anti-synthetase syndrome due to anti-Jo-1 antibody positivity and muscle weakness. Additional findings, including mononeuritis multiplex, purpura, crescentic glomerulonephritis, and strongly positive myeloperoxidase-antineutrophil cytoplasmic antibodies, led to a diagnosis of microscopic polyangiitis. Although anti-Jo-1 antibodies are highly specific, false-positive results can occur. This case highlights the need for a comprehensive evaluation beyond antibody testing to avoid diagnostic errors.

Self-expanding metal stents (SEMS) are widely used as a bridge to surgery in obstructive colon cancer, offering short-term benefits by reducing postoperative complications and avoiding emergency stoma creation. Additionally, early initiation of systemic chemotherapy may improve oncological outcomes. However, the safety and feasibility of preoperative chemotherapy following SEMS placement remain unclear. This study aims to evaluate the safety and efficacy of neoadjuvant CAPOX chemotherapy after SEMS placement in patients with obstructive colon cancer. This is a prospective, multicenter, single-arm Phase II trial involving patients with clinical stage II or III obstructive colon cancer. Eligible patients undergo successful SEMS placement followed by two cycles of CAPOX chemotherapy prior to elective surgery. The primary endpoint is the incidence of severe perioperative complications, defined as a composite of stent-related adverse events and Clavien - Dindo Grade III or higher postoperative complications. Secondary endpoints include chemotherapy-related adverse events, pathological response, and 2-year relapse-free survival. A total of 75 patients are planned for enrollment across 16hospitals in Japan over 1.5 years. The study is registered in the Japan Registry of Clinical Trials (jRCTs051240077).

Fibrillary glomerulonephritis (FGN) is a rare kidney disease characterized by the deposition of Congo red (CR) negative fibrils measuring 12 to 24 nm in diameter on electron microscopy. Recently, DnaJ heat shock protein family (Hsp40) member B9 (DNAJB9) detected by liquid chromatography-tandem mass spectrometry (LC-MS/MS) was discovered to be a sensitive and specific biomarker for FGN. This report presents the first documented case of recurrent congophilic FGN, a rare glomerular disease with features resembling amyloidosis, occurring two years and eight months after a renal transplant. Diagnosis of both primary and recurrent FGN was confirmed through DNAJB9 detection using advanced laser microdissection and LC-MS/MS techniques. The recurrent FGN exhibited features consistent with amyloidosis, including CR positivity, characteristic microscopic findings, and fibrils on electron microscopy with a 9 to 15 nm diameter. This case demonstrates that FGN could recur in the transplanted kidney as congophilic GN with characteristic findings of amyloidosis, and it is difficult, if not impossible, to render a correct diagnosis without LC-MS/MS or immunohistochemistry.

To compare the efficacy and safety of rituximab (RTX) to those of intravenous cyclophosphamide (IVCY) as remission induction therapy in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) and severe renal impairment. Using the REVEAL cohort, this retrospective multicentre study enrolled 96 newly diagnosed patients with AAV and an estimated glomerular filtration rate of <30 mL/min/1.73 m2. The patients were divided into RTX and IVCY groups. Matching weights were used to adjust for baseline characteristics. The primary outcome was a 2-year composite of all-cause mortality or end-stage kidney disease (ESKD). Secondary outcomes included all-cause mortality, ESKD, relapse, and hospitalisation-requiring infections. Outcomes were assessed using multivariable Cox proportional hazards models. In the weighted population, the hazard ratio (HR) for RTX versus IVCY was 0.73 (95% confidence interval: 0.31-1.72) for the primary composite outcome, with no significant intergroup difference. Similarly, intergroup differences in the HRs for all-cause mortality, ESKD, relapses, and hospitalisation-requiring infections were not significant. RTX and IVCY demonstrated comparable efficacy in preventing mortality and ESKD in this high-risk population. RTX is a viable option for patients with AAV and severe renal impairment.

Atrial fibrillation (AF) after bioprosthetic valve (BPV) replacement is common in older patients with multiple comorbidities and is associated with a heightened risk of thromboembolism. Anticoagulation therapy is often indicated, but renal impairment and other comorbidities elevate bleeding risk, making clinical decisions complex. This study compared clinical outcomes between warfarin and direct oral anticoagulants (DOACs) in this high-risk population. This subgroup analysis of the BPV-AF Registry included 612 patients treated with oral anticoagulants after BPV replacement, stratified by renal function: normal or mild impairment (creatinine clearance [CCr] ≥50 mL/min), mild-to-moderate impairment (30 mL/min ≤ CCr < 50 mL/min), and moderate-to-severe impairment (15 mL/min ≤ CCr < 30 mL/min). Baseline characteristics and outcomes were analyzed within each stratum. The composite outcome of stroke, systemic embolism, and cardiovascular events was numerically less frequent in the DOAC than warfarin group across all strata, although the differences were not statistically significant. Major bleeding also tended to be lower in the DOAC group. In this study from a Japanese nationwide registry comparing outcomes of AF patients after BPV replacement with severe renal impairment between those treated with DOACs and those treated with warfarin, comparative conclusions between DOACs and warfarin cannot be drawn because of the small sample size. Nonetheless, both anticoagulants may be acceptable in clinical practice, highlighting the need for individualized decision-making based on patient risk.