Tottori Prefectural Central Hospital

Glucocorticoids (GC) comprise a cornerstone in the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV); however, prolonged GC exposure leads to substantial toxicity and immune system-related complications. Hence, identifying treatment strategies that enable early GC withdrawal while maintaining disease control is of clinical importance. To investigate the proportion and characteristics of patients achieving GC-free clinical remission (GFCR) 48 weeks after treatment initiation in patients with newly diagnosed microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) and examine their long-term clinical outcomes (up to week 96). We conducted a retrospective cohort study using clinical data from a multicenter, nationwide registry in Japan comprising patients with newly diagnosed MPA or GPA and at least 48 weeks of follow-up. GFCR was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 with complete GC withdrawal. Univariable and multivariable logistic regression analyses were used to identify independent predictors of GFCR at week 48, and a sensitivity analysis using a matched cohort was performed to validate robustness. A total of 728 patients were enrolled in the registry, of whom 544 were followed for ≥ 48 weeks; among them, 29 (5.3%) achieved GFCR at week 48. Use of rituximab (RTX) for induction therapy and avacopan within 48 weeks were independently associated with achieving GFCR (multivariable analysis, model 1: RTX: odds ratio [OR]: 3.9, 95% confidence interval [CI]: 1.5–10.0; avacopan: OR: 24.3, 95% CI: 5.8–101.9; both p < 0.01). Conversely, methylprednisolone pulse therapy was associated with a lower likelihood of achieving GFCR (multivariable analysis, model 1: OR: 0.08, 95% CI: 0.02–0.44; p < 0.01). Patient’s demographic and baseline disease characteristics were not predictive of GFCR. In the long-term (weeks 48 through 96), the rates of death, relapse, and serious infection were similar regardless of GFCR status at week 48. The use of RTX for induction therapy and avacopan within 48 weeks is independently associated with achieving GFCR at week 48 in patients with MPA and GPA, supporting their potential as key components of GC-sparing strategies in AAV. Prospective studies are needed to confirm these results and optimize treatment algorithms to ensure disease control and minimize GC-related toxicity.

Plastic bronchitis is a severe condition characterised by the formation of dendritic mucus plugs within the trachea and bronchi, leading to respiratory failure due to acute airway obstruction. Bronchoscopic mucus plug removal is considered effective; however, in paediatric patients, it is often difficult to perform due to limitations in tracheal tube size. We report a case of plastic bronchitis associated with influenza A infection during a relapse of nephrotic syndrome. Bronchoscopic mucus plug removal was performed using a cuffed endotracheal tube one size larger than standard, thereby avoiding the need for extracorporeal membrane oxygenation (ECMO). The use of modified cuffed tubes with minimal outer diameter differences highlights the importance of flexible size selection tailored to the patient's condition.

ABSTRACTGiardia duodenalis can be incidentally detected during pancreatic tumor evaluation via cytology. Even in asymptomatic patients without risk factors, recognition is essential. Careful cytologic examination enables early treatment to prevent complications before surgery. Although rare, incidental detection is clinically significant and guides preoperative management.

Rotator cuff tears are often associated with shoulder pain and functional impairment. Evidence suggests that contributing factors include increased blood flow in the dorsal scapular artery (DSA) supplying the periscapular tissues and abnormal scapular position. However, a direct relationship between the DSA blood flow velocity and scapular position has not been investigated. This study examined the relationship between the DSA blood flow velocity and scapular position. The study sample included 34 patients with rotator cuff tears. Clinical evaluations included range of motion (ROM) (anterior elevation (AE), abduction (AB), and external rotation (ER)) and the peak systolic velocity (PSV) in the DSA. Scapular position was assessed using X-rays to measure the distance between the scapula and spine (i.e., DSS: distance between the superior border and the corresponding spine; DMS: distance between the medial border and the corresponding spine; DIS: distance between the inferior border and the corresponding spine), and the anterior tilt angle was measured using a digital inclinometer. The PSV in the DSA, and scapular position were compared between the affected and unaffected sides. The results showed significantly higher PSV in the DSA on the affected side compared to the unaffected side (95 % confidence interval (CI): from 0.6 to 7.4, p < 0.05). A negative correlation between PSV in the DSA and DMS was also found (r = −0.41, p < 0.05). These findings suggest that patients with symptomatic rotator cuff tears experience increased PSV in the DSA on the affected side of patients with symptomatic rotator cuff tears can potentially be attributed to the condition of the scapular position.

In this Japanese cohort, TsB and TcB levels were consistently lower in HIE newborns undergoing TH during the first week of life. COHb levels were initially comparable but increased later in the case group. These findings suggest that reduced HO activity may contribute to suppressed heme degradation in moderate to severe HIE cases receiving TH. • Newborns with hypoxic ischemic encephalopathy (HIE) often exhibit lower bilirubin levels than expected, despite risk factors for hyperbilirubinemia. • This study indicates that reduced heme oxygenase activity may contribute to the lower bilirubin levels observed in HIE newborns undergoing therapeutic hypothermia.

Several studies have investigated the safety of laparoscopic gastrectomy (LG) for gastric cancer (GC). However, many of these studies excluded older patients, and few multicenter studies have compared LG and open gastrectomy (OG) in older patients with GC. We compared the postoperative short- and long-term outcomes of LG and OG in >75 years old patients with GC in a multicenter study. The study included 926 patients aged >75 years who underwent gastrectomy from January 2003 to December 2017 at 12 regional hospitals. After propensity score-matching (PSM), the two surgical groups' short- and long-term outcomes were analyzed. PSM was applied for variables such as sex, depth of tumor invasion, lymph node metastasis, histologic type, pathological stage, gastrectomy, and lymph node dissection. After 1:1 matching, 258 cases in each group were analyzed. The LG group showed significantly longer surgery times and reduced blood loss (p<0.001). There were no significant differences in the incidence of postoperative complications graded Clavien-Dindo ≥ II between the groups (23.6% vs. 19.4%, p=0.239). However, the LG group had lower and higher incidences of pneumonia (1.9% vs. 5.4%, p=0.035) and pancreatic fistula (PF; 4.7% vs. 0.04%, p=0.003), respectively. The two groups had no significant overall and disease-specific survival differences across all stages. In patients aged >75 years with GC, LG can be a viable approach with careful clinical management for the risk of PF, without compromising long-term survival.

ObjectiveTo compare clinicopathological phenotype-based, anti-neutrophil cytoplasmic antibody (ANCA) serotype-based, and unsupervised data-driven classifications in relation to clinical outcomes and patient heterogeneity in a large Japanese cohort with ANCA-associated vasculitis (AAV).MethodsThis multicentre, retrospective cohort study analysed data from a nationwide Japanese registry of 729 newly diagnosed patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA), all positive for myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA. Patients were classified by phenotype, serotype, combined phenotype-serotype groupings, and data-driven clustering based on baseline clinical and laboratory features. Associations with clinical outcomes—including mortality, relapse, and response to rituximab (RTX) versus cyclophosphamide (IVCY)—were evaluated using inverse probability of treatment weighting (IPW).ResultsPhenotype-based classification more accurately distinguished all-cause mortality risk (MPA vs. GPA: hazard ratio [HR] 2.53, 95% CI 1.34–4.76). Combined phenotype-serotype analysis identified MPO-MPA patients with the highest mortality (HR 3.45, 95% CI 1.09-11.0, vs. PR3-GPA) and PR3-GPA with the highest severe relapse. Discordant groups, such as MPO-GPA, demonstrated unique clinical characteristics. After IPW adjustment, no significant difference in 24-week remission rates was observed between RTX and IVCY across classifications, both overall (RR 1.02, 95% CI 0.95–1.09) and within subgroups. Unsupervised clustering identified four distinct clinical subgroups, with limited concordance with conventional phenotype or serotype classifications.ConclusionPhenotype and serotype classifications provide complementary, not competing, prognostic insights in Japanese patients with AAV. Data-driven clustering revealed additional clinical heterogeneity not captured by traditional systems, underscoring the need for integrated, multi-dimensional stratification approaches to improve personalised risk assessment and treatment strategies.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00296-025-06014-y.