We experienced a case of resection of a pancreatic body bearing a serotonin-producing pancreatic neuroendocrine neoplasm( PanNEN). The patient was a female in her 70s. Contrast-enhanced CT of the pancreatic body showed a 12 mm tumor that was well enhanced in the early, portal, and equilibrium phases. The main pancreatic duct was stenosed at the tumor position, and the distal side was dilated. Although the contrast pattern was indicative of PanNEN, the stenosis of the main pancreatic duct suggested the possibility of invasive pancreatic ductal carcinoma. A serotonin-producing subtype of PanNEN, which causes stenosis of the main pancreatic duct despite its small diameter, was included in the differential diagnoses. We performed resection of the pancreatic body and tail with lymph node dissection. Pathological examination indicated that the tumor was PanNEN G1, and immunostaining revealed positivity for serotonin. Most PanNENs are not accompanied by stenosis of the main pancreatic duct. However, it has been reported that even a small-sized serotonin-producing PanNEN is likely to cause main pancreatic duct stenosis owing to its proliferation pattern. Although there are few reports of serotonin-producing PanNENs, an understanding of the characteristic imaging findings of this disease may be useful in the differential diagnosis of pancreatic tumors.
Abstract Objectives B cell depletion by rituximab (RTX) is an effective treatment for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). However, peripheral B cell phenotypes and the selection criteria for RTX therapy in AAV remain unclear. Methods Phenotypic characterization of circulating B cells was performed by 8-color flow cytometric analysis in 54 newly diagnosed AAV patients (20 granulomatosis with polyangiitis and 34 microscopic polyangiitis). Patients were considered eligible to receive intravenous cyclophosphamide pulse (IV-CY) or RTX. All patients also received high-dose glucocorticoids (GC). We assessed circulating B cell phenotypes and evaluated the efficacy after 6 months of treatment. Results There were no significant differences in the rate of clinical improvement, relapses, or serious adverse events between patients receiving RTX and IV-CY. The rate of Birmingham Vasculitis Activity Score (BVAS) improvement at 6 months tended to be higher in the RTX group than in the IV-CY group. The proportion of effector or class-switched memory B cells increased in 24 out of 54 patients (44%). The proportions of peripheral T and B cell phenotypes did not correlate with BVAS at baseline. However, among peripheral B cells, the proportion of class-switched memory B cells negatively correlated with the rate of improvement in BVAS at 6 months after treatment initiation ( r = − 0.28, p = 0.04). Patients with excessive B cell differentiation were defined as those in whom the proportion of class-switched memory B cells or IgD − CD27 − B cells among all B cells was > 2 SDs higher than the mean in the HCs. The rate of BVAS remission in patients with excessive B cell differentiation was significantly lower than that in patients without. In patients with excessive B cell differentiation, the survival rate, the rate of BVAS-remission, and dose reduction of GC were significantly improved in the RTX group compared to those in the IV-CY group after 6 months of treatment. Conclusions The presence of excessive B cell differentiation was associated with treatment resistance. However, in patients with circulating B cell abnormality, RTX was effective and increased survival compared to IV-CY. The results suggest that multi-color flow cytometry may be useful to determine the selection criteria for RTX therapy in AAV patients.
Continuous haemodiafiltration (CHDF) with a cytokine-adsorbing haemofilter (AN69ST–CHDF) was successfully used to treat systemic lupus erythematosus (SLE) complicated with macrophage activation syndrome (MAS) and haemophagocytic lymphohistiocytosis (HLH) in a 35-year-old male. Five months before admission, serositis, proteinuria, and neuropsychiatric SLE recurred. He was treated with glucocorticoid, rituximab, and intravenous cyclophosphamide. He was subsequently admitted for the examination of hepatic disorder and leukopenia. Following admission, he was diagnosed with MAS/HLH, and glucocorticoid pulse therapy was initiated. Although leukopenia temporarily improved, his pancytopenia and hepatic dysfunction worsened, leading to multiple organ dysfunction syndrome (MODS) on day 22. Because hypercytokinemia contributed to MAS/HLH exacerbation, we implemented a combination therapy with cyclosporine A and plasma exchange. This strategy failed to improve MODS; thus, AN69ST–CHDF was initiated and added rituximab which led to improved hypercytokinemia and eventual recovery from MODS. AN69ST–CHDF might be an effective therapeutic option for MAS/HLH.
Abstract Importance Patient trust plays a central role in the patient-physician relationship; however, the impact of outpatient visits with a covering physician (covered visits) on the level of trust in usual physician among patients with chronic conditions is unknown. Objective To determine whether the number of outpatient visits with a covering rheumatologist is associated with patient trust in the usual rheumatologist. Design Cross-sectional study. Setting This study used data from the TRUMP 2 -SLE project conducted at five academic medical centers in Japan. Participants The participants were Japanese adults with systemic lupus erythematosus who met the 1997 revised classification criteria of the American College of Rheumatology. The enrollment period was February 2020 to October 2021. Exposure Outpatient visits with a covering rheumatologist in the past year. Main Outcomes and Measures The main outcome was patient trust in their usual rheumatologist, assessed using the 11-item Japanese version of the modified Trust in Physician Scale (range 0–100). A general linear model with cluster robust variance estimation was used to evaluate the association between the number of outpatient visits with a covering rheumatologist and the patient’s trust in their usual rheumatologist. Results Of the 515 enrolled participants, 421 patients with systemic lupus erythematosus were included in our analyses. The median age was 47.0 years, and 87.2% were women. Thirty-nine usual rheumatologists participated in this study. Patients were divided into groups according to the number of outpatient visits with a covering rheumatologist in the past year as follows: no visits (59.9%; reference group), one to three visits (24.2%; low-frequency group), and four or more visits (15.9%; high-frequency group). The median Trust in Physician Scale score was 81.8 (interquartile range 72.7–93.2). Both the low-frequency and high-frequency groups exhibited lower trust in their usual rheumatologist (mean difference: -3.03 [95% confidence interval -5.93 to -0.80], -4.17 [95% confidence interval -7.77 to -0.58, respectively]). Conclusions and Relevance This study revealed that the number of outpatient visits with a covering rheumatologist was associated with lower trust in a patient’s usual rheumatologist. Further research is needed to address the potential adverse effects of physician coverage on trust in patient’s usual rheumatologist. Key Points Question Is the number of outpatient visits with a covering rheumatologist associated with the loss of trust in usual rheumatologist in patients with systemic lupus erythematosus (SLE)? Findings This multicenter cross-sectional study which included 421 patients with SLE revealed that the number of outpatient visits with a covering rheumatologist in the past year was associated with lower levels of trust in the usual rheumatologist. Meaning This study alerts us about the need to prepare for the possible adverse effects of unavoidable outpatient coverage.
Abstract Objectives Poor medication adherence among patients with systemic lupus erythematosus (SLE) is a critical problem associated with adverse outcomes. This study examined the relationship between trust in one’s physician and goal-oriented thinking, hope, and medication adherence among Japanese patients with SLE who were ethnically matched to their physicians. Methods This cross-sectional study was conducted in the rheumatology outpatient clinics at five academic centers. Patients with SLE who were prescribed oral medications were included. The main exposure was trust in one’s physician measured via the 5-item Japanese version of the Wake Forest Physician Trust Scale and the 18-item Health-related Hope Scale, with each score ranging from 0 to 100 points. The outcome was medication adherence measured using the 12-item Medication Adherence Scale with scores ranging from 5 to 60 points. A general linear model was created after adjusting for demographics, socioeconomic status, disease activity, disease duration, basic health literacy, depression, medication variables, experiencing adverse effects, and concerns regarding lupus medications. Results Altogether, 373 patients with SLE were included. The mean age of the patients was 46.4 years, and among them, 329 (88.2%) were women. Both trust in one’s physician (per 10-point increase: 0.88, 95% confidence interval [95%CI]: 0.53 to 1.24) and the Health-related Hope score (per 10-point increase: 0.64, 95%CI: 0.33 to 0.95) were associated with better medication adherence. Conclusions Physician communication to build trust and coaching on self-management to maintain or achieve what is important in the patient’s life and to enhance hope may lead to better medication adherence. Key messages What is already known on this topic The possible association of loss of trust in the attending physician with medication adherence in systemic lupus erythematosus has been conflicting in previous research, in which the effect of physician–patient racial mismatch has not been considered. The protective role of hope and goal-oriented thinking for mental symptoms has been suggested among patients with SLE. What this study adds This study revealed that both trust in one’s physician and health-related hope were associated with better medication adherence in Japanese patients who were ethnically matched to their physicians. How this study may affect research, practice, or policy The results indicated that physician communication to build trust and coaching on self-management to maintain or achieve what is important in the patient’s life may lead to better medication adherence.
Tuberculosis (TB) detection is mainly classified as being either passive case-finding, at a hospital, or positive case-finding through a mass screening. Pulmonary TB patients detected while being examined for other complaints at a hospital are classified as passive findings. It is suspected that these patients have special characteristics which separate them from patients that are detected only after the onset of TB symptoms. The aim of this study is to elucidate those special characteristics of patients where detection occurred while being examined for other diseases by comparing them with patients detected by the other means. A survey was conducted on 686 pulmonary TB patients, diagnosed between 1986 and 1988, from the area served by the Kochi Prefectural Chuo Health Center, with the following results. 1) Among the 533 patients who were detected at a hospital, and excluding those detected by mass screening, 331 patients were detected only after the onset of TB symptoms (group B), with 202 patients being treated for other diseases before being diagnosed as having TB (classified as group A). In group A, 130 patients were detected because of manifesting additional symptoms related to tuberculosis such as fever and cough (group A-1), while the remaining 72 patients were detected by chance while being examined for other diseases (group A-2). 2) The number of elderly people and relapse cases was higher in group A compared to B group and the group detected by mass screening. 3) Symptoms, the rate of tubercle bacillus positives, and the period from the onset of symptoms to diagnosis (defined delay) in group A-1, were similar to group B. In group A-2, symptoms and the rate of tubercle bacillus positives were less than those of the other groups. 4) From the above findings, it is concluded that passive case-findings should be classified into 3 distinct groups.
