Myasthenia gravis (MG) requires long-term therapeutic strategies that control symptoms while minimizing exposure to oral corticosteroids (OCS). Fast-acting treatments (FT), such as intravenous immunoglobulin (IVIG), are commonly used during exacerbations, whereas efgartigimod (EFG) has recently emerged as an alternative outpatient therapy. This study aimed to compare the efficacy and quality of life (QOL) outcomes of IVIG and EFG in patients with MG. A retrospective analysis was conducted in 67 patients with MG. Fifty-five patients continued IVIG during exacerbations (IVIG group), whereas 12 patients switched from IVIG to EFG (Switched-EFG group). The primary outcome was the achievement of minimal manifestations (MM) or MM with prednisolone ≤ 5 mg/day (MM-5 mg). The secondary outcomes included changes in the Myasthenia Gravis Activities of Daily Living scale (MG-ADL) scores and reduction of prednisolone (PSL) dose between the two groups. The number of hospitalizations and scores on the Japanese version of the Myasthenia Gravis Quality of Life 15-Item Scale-Revised (MG-QOL15r-J) were compared before and after EFG initiation. No significant differences were observed between the groups in MM, MM-5 mg, ΔMG-ADL, or ΔPSL scores. In the Switched-EFG group, the number of hospitalizations was significantly decreased, whereas the MG-QOL scores improved following EFG initiation. The questionnaire responses consistently favored EFG, particularly in supporting family roles, work participation, and social activities. Notwithstanding the study's limited sample size and observational nature, Switching to EFG yielded notable therapeutic benefits compared with IVIG, while additionally reducing the hospitalization rate and enhancing QOL. Therefore, EFG may be a valuable outpatient treatment option for refractory MG.

Many medicines are ineffective in the treatment of ischaemic optic neuropathy (ION). The effects of all ten medicines for the treatment of ION were assessed through a meta-analysis using direct and indirect comparisons of multiple treatments. This systematic review of nine randomised controlled trials (1,012 participants) from April 1994 to May 2022 compared the efficacy of placebo (PLA), ranibizumab (RAN), dexamethasone (DEX), prednisolone (PRE), oxygen (OX), troxerutin (TRO), RPh201, erythropoietin (ERY), levodopa + carbidopa (LEV+CAR), and prednisolone + ranitidine (PRE+RA) for the treatment of ION. The primary measure of success was the percentage of patients who either responded to the treatment or discontinued the study. Intention-to-treat analysis was conducted. DEX, PRE, OX, TRO, ERY, LEV+CAR, and PRE+RA were significantly more effective than PLA (odds ratios (ORs) 43.64, 1.19, 1.17, 4.22, 2.64, 3.32, and 1.10, respectively). RAN was significantly more effective than DEX (OR 1.67). RPh201 is significantly less effective than all other medicines. Acceptance of PLA was good. RAN and DEX are the best options for treating ION. Therefore, these results should be considered in clinical practice. Key Words: Ischaemic optic neuropathy, Meta-analysis, Treatment.

Prognostic value of 18F-fluorodeoxyglucose positron emission tomography one month after initiation of prednisolone therapy in patients with cardiac sarcoidosis.

Peripheral blood UBA1 variant burden predicts poor outcomes in VEXAS syndrome: a nationwide prospective study.

Background: While immune checkpoint inhibitor (ICI)-related pneumonitis (CIP) may relapse during or after steroid treatment, clinical factors associated with CIP relapse are unclear. This study explored risk factors potentially associated with CIP relapse. Methods: This single-center retrospective study included 1099 patients who received ICIs at our institution between April 2015 and March 2022. Among them, 39 patients who developed CIP and were treated with systemic steroids and tapered to prednisolone (PSL) ≤ 20 mg/day were analyzed. Patients were classified into relapse and non-relapse groups based on whether CIP recurred during or after steroid treatment. Patient characteristics, clinical features at onset, and treatment strategies were compared between the two groups. Results: Thirteen patients (33.3%) experienced relapse. Compared with the non-relapse group, the relapse group had a significantly higher proportion of non-smokers (30.8 vs. 3.3%, p = 0.035), a greater frequency of Common Terminology Criteria for Adverse Events (CTCAE) Grade 2 pneumonitis (92.3 vs. 53.8%, p = 0.029), and lower serum KL-6 levels (288 vs. 704 U/mL, p = 0.014). The relapse group also had a shorter duration of steroid therapy at the initial dose, ≥0.5 mg/kg/day, ≥15 mg/day, and ≥20 mg/day (p < 0.05) and lower cumulative steroid doses (1140 vs. 1902 mg, p = 0.015). Relapse tended to occur in patients with relatively mild pneumonitis who received lower steroid doses and shorter treatment durations. Conclusions: Non-smoking status, CTCAE Grade 2 pneumonitis, lower serum KL-6 levels, shorter duration of steroid therapy, and lower cumulative steroid dose were potentially associated with CIP relapse. Adequate steroid dosing and tapering may help prevent relapse.

Long-term data on the risk factors for fistulizing disease (FD) after ileal pouch-anal anastomosis (IPAA) for ulcerative colitis are limited. This study aimed to identify the predictors of FD after ileostomy closure and evaluate the impact of FD on pouch failure. We reviewed 374 patients who underwent mucosal restorative proctocolectomy with handsewn IPAA between 2004 and 2022. Cox proportional hazards regression and log-rank tests were used to assess the FD-free survival and risk factors. After excluding 59 patients, 315 patients were analyzed. FD developed in 20 (6.3%) of these patients. Multivariate analysis showed that a monthly prednisolone (PSL) dose ≥ 450mg before IPAA and chronic pouchitis were independent risk factors for FD (odds ratio [OR] 2.96, 95% CI: 1.15-7.64, p = 0.025; OR 3.85, 95% CI: 1.52-9.78, p = 0.0045). Patients with PSL ≥ 450mg or chronic pouchitis had significantly poorer FD-free survival (p = 0.0004 and p = 0.0002, respectively). Pouch failure occurred more frequently in patients with FD than in those without FD (30.0% vs. 1.0%; p < 0.0001). High steroid exposure before IPAA and chronic pouchitis were significant predictors of FD, which strongly increased the risk of pouch failure.

Clinical Characteristics and Treatment Outcomes of Autoimmune-Like Hepatitis After Allogeneic Hematopoietic Stem Cell Transplantation.

Cyclosporine A (CsA) is an effective steroid-sparing agent for steroid-dependent nephrotic syndrome (SDNS); however, long-term use can cause chronic kidney injury (CsA nephropathy). We previously reported that alternatively activated macrophages (M2-type) are linked to interstitial fibrosis in progressive kidney disease. This study aimed to investigate the potential involvement of M2-type macrophages in CsA nephropathy in SDNS. Thirty-three children with SDNS treated with CsA for more than 2years were investigated. Fourteen age-matched SDNS children without CsA served as controls. Kidney fibrosis was assessed by Masson staining. Sections were immunostained for α-SMA, type I collagen, CD68, CD163, and CCL2. Urine levels of CCL2 were measured using a cytometric bead array kit. The CsA-treated group showed greater interstitial fibrosis (12.2 ± 7.3 vs.7.6 ± 2.1%, p < 0.001) and increased CD163+CD68+ macrophages (10.8 vs.7.9/HPF; p < 0.001). Multivariate analysis identified CD163⁺ infiltration (β = 0.632, p < 0.0001), cumulative prednisolone (PSL) dose during CsA therapy (β = 0.015, p = 0.0031), and cumulative PSL dose from onset to biopsy (β = - 0.005, p = 0.030) as independent predictors of fibrosis. CCN2 and CCL2 co-localized with CD163+ macrophages in CsA biopsies. Urinary CCL2/creatinine ratio was higher in the CsA group than controls at relapse (1012 ± 641.2 vs. 239.9 ± 226.9pg/mg;p = 0.02) and at remission (202.0 ± 178.4 vs. 77.7 ± 127.3pg/mg;p = 0.04). CD163+ M2-type macrophages may contribute to CsA-induced interstitial fibrosis. Steroid treatment during CsA treatment appears to augment CsA nephrotoxicity via pro-fibrotic pathways.

Background Anifrolumab, a monoclonal antibody against the type I interferon receptor, has shown efficacy in systemic lupus erythematosus (SLE). However, sustained remission after drug discontinuation has been rarely reported. Case presentation We reported two females with SLE who achieved biologic-free clinical remission after discontinuation of anifrolumab, with no major adverse events observed during or after treatment. Case 1: A 25-year-old woman with newly diagnosed SLE presented with active disease (SLEDAI-2K 14), including arthritis, facial rash, alopecia, mucosal ulcers, hypocomplementemia, and anti–double-stranded DNA antibody positivity. She was initially treated with prednisolone (PSL) and hydroxychloroquine (HCQ); however, a flare occurred during glucocorticoid (GC) tapering, and anifrolumab was initiated with concomitant methotrexate (MTX). The patient achieved clinical remission according to the Definition of Remission In SLE (DORIS) criteria, despite persistent hypocomplementemia and anti–dsDNA antibody positivity. After sustained remission, PSL was discontinued, MTX was tapered, and anifrolumab was discontinued after 25 months due to sustained clinical remission according to the DORIS criteria and patient preference related to work-related difficulty with long-term regular follow-up. She has remained in clinical remission for 6 months on HCQ and low-dose MTX. Case 2: A 22-year-old woman with SLE diagnosed at age 16 presented after disease relapse following GC withdrawal at age 21. At relapse, her disease activity was moderate (SLEDAI-2K 11), characterized by facial rash, alopecia, mucosal ulcers, leukopenia, hypocomplementemia, and anti–dsDNA positivity. She was treated with anifrolumab in combination with HCQ. Her disease activity achieved and maintained clinical remission according to the DORIS criteria, despite persistent hypocomplementemia and anti–dsDNA antibody positivity. Dose spacing and subsequent discontinuation of anifrolumab were attempted because of patient preference related to work-related limitations to long-term follow-up, and future pregnancy plans, and sustained remission was maintained. Even after switching to tacrolimus (Tac), she has maintained remission without anifrolumab for 12 months. Conclusion These cases suggested that clinical remission could be maintained after discontinuation of anifrolumab in selected patients with SLE.

Biologic agents have demonstrated efficacy in the treatment of refractory Takayasu arteritis (TAK). Although vascular stenosis is a common manifestation of the chronic phase of TAK, evidence on the effects of biologic therapy on established vascular lesions remains limited. Here, we report a case series of TAK in which chronic arterial stenosis showed marked improvement following treatment with biologic agents. The first case was a 27-year-old woman with stenosis of the left common carotid artery. Initial treatment with prednisolone (PSL) led to clinical improvement; however, the vascular lesion progressed during tapering, despite the normalisation of inflammatory markers. Reinduction with a methylprednisolone pulse and methotrexate led to slight improvement. Subsequent therapy with tocilizumab, followed by golimumab, resulted in significant and sustained improvement in the stenosis. The second case was a 20-year-old woman with wall thickening of the right brachiocephalic artery. Although PSL and methotrexate were initially administered, the progression of left subclavian artery stenosis was detected on ultrasonography before symptom onset, despite normalised inflammatory markers. The introduction of tocilizumab, with increased PSL, led to a notable improvement in the vascular lesions. These cases and a literature review suggest that biologic agents may reverse vascular remodelling in chronic TAK, even in the absence of systemic inflammatory activity. Comprehensive disease assessment using imaging modalities, alongside serum biomarkers, is essential to guide therapeutic decisions and monitor vascular changes. These findings highlight the importance of imaging-based disease monitoring and raise the potential for targeted treatment strategies aimed at both inflammation control and vascular lesion modification.

A woman in her 50s contracted Coronavirus disease 2019 (COVID-19), initially presenting with mild symptoms, and managed conservatively. However, she developed a persistent low-grade fever and insidious joint pain for 1 month, prompting further evaluation. Chest computed tomography revealed bilateral pulmonary infiltrates, leading to hospitalization for COVID-19-associated pneumonia. Despite a 2-week course of ceftriaxone and azithromycin, her condition remained unchanged. Postadmission testing revealed elevated rheumatoid factor, anti-cyclic citrullinated peptide (CCP) antibodies, and matrix metalloproteinase-3, suggesting an inflammatory or autoimmune process. Given concerns for immune-mediated inflammation, she was treated with high-dose methylprednisolone. With pneumonia improvement, she was discharged on oral prednisolone (PSL) (20 mg/day) with a planned taper. Her joint symptoms resolved, and anti-CCP antibody levels normalized during steroid therapy. However, upon PSL tapering and discontinuation, her joint pain recurred, and anti-CCP antibodies became positive again. A rheumatology consultation confirmed rheumatoid arthritis (RA). This case provides rare longitudinal documentation of dynamic anti-CCP antibody changes that paralleled clinical disease activity, illustrating the progression from postviral reactive arthritis to classifiable RA. It underscores COVID-19's potential to trigger autoimmune dysregulation and highlights the need for long-term follow-up with serial autoantibody monitoring in patients with persistent musculoskeletal symptoms after infection.

BackgroundTanshinone IIA (TSN), a compound extracted from Salvia miltiorrhiza, has demonstrated a range of pharmacological activities. However, its therapeutic efficacy in glucocorticoid-induced osteoporosis (GIOP) remains insufficiently understood. This study was performed to investigate the protective effects of TSN against prednisolone (PN)-induced osteoporosis in zebrafish larvae.MethodsThe osteoprotective properties of TSN were assessed using alizarin red S staining, calcein staining, and alcian blue staining to evaluate bone mineralization, density, and cartilage development. Cardiovascular function and locomotor behavior were analyzed using transgenic zebrafish models and behavioral tracking systems. RNA sequencing was performed to identify differentially expressed genes and key signaling pathways. Protein-protein interaction networks were constructed to elucidate gene associations, and results were validated via quantitative reverse transcription polymerase chain reaction.ResultsTSN administration effectively attenuated PN-induced developmental toxicity, enhanced bone mineralization and density, and improved cartilage abnormalities in zebrafish larvae. Transcriptomic analysis identified 505 genes with reversed expression profiles between the TSN and PN treatment groups, primarily associated with skeletal system development, lipid metabolism, and fatty acid β-oxidation. Key affected pathways included PPAR signaling, lipid atherosclerosis, and reactive oxygen species. Notably, skeletal development was characterized by the upregulation of genes including col1a1b, col2a1b, col9a3, rdh1, and acana.ConclusionThese findings demonstrate the osteoprotective effects of TSN in mitigating glucocorticoid-induced bone loss and cartilage abnormalities, highlighting its potential as a therapeutic agent for GIOP. The transcriptomic insights provide novel perspectives on TSN’s role in regulating bone metabolism and skeletal development.

Recent reports have suggested an association between the pathogenesis of systemic lupus erythematosus (SLE) and various adipokines secreted by adipocytes. However, the impact of obesity on the susceptibility to SLE relapses remains unclear. This study investigated the association between obesity and relapse in patients with SLE using a large, multicenter cohort. Patients enrolled in LUNA, a nationwide, multicenter SLE registry in Japan, with a body mass index (BMI) ≥ 18.5 kg/m2 were included in the study. Participants were divided into two groups: the obese group (BMI ≥ 25 kg/m2) and the normal weight group (18.5 kg/m2 ≤ BMI < 25 kg/m2). The one-year relapse rate after LUNA registration was compared between the two groups. Relapses were defined as an increase in glucocorticoid dosage during follow-up. A multivariate analysis was performed using generalized linear models adjusted for age, sex, disease duration, education level, glycated hemoglobin level, disease activity (Systemic Lupus Erythematosus Disease Activity Index), organ damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), current prednisolone (PSL) dose, past maximum PSL dose, calcineurin inhibitor use, and incretin-related drug use as covariates. Furthermore, a sensitivity analysis was performed using the same statistical model and covariate adjustments to reclassify participants into the overweight (BMI ≥ 25 kg/m2 and < 30 kg/m2) and non-overweight (BMI ≥ 18.5 kg/m2 and < 25 kg/m2 or BMI ≥ 30 kg/m2) groups. Among the 1, 134 patients, 909 were classified as normal weight and 225 as obese. The multivariate analysis showed that obesity significantly reduced relapse rates (odds ratio [OR] 0.52, 95% confidence interval [CI] 0.30-0.90, p=0.02). The sensitivity analysis revealed that overweight was an independent factor associated with a reduced risk of SLE relapses, with an OR lower than that for obesity in the primary analysis (OR 0.43, 95% CI 0.22-0.82, p=0.01). Patients with mild obesity had lower relapse rates than those with normal BMI. The findings suggest that metabolic status may influence the disease course of SLE. Appropriate weight management guidance, including avoiding excessive dietary restriction, may help prevent SLE relapses.

Rationale:Hemoptysis is a known complication of lung cancer that may occur after antiplatelet or anticoagulant therapy, chemotherapy, or antiangiogenic agents. Although immune checkpoint inhibitors have improved outcomes in lung cancer, hemoptysis remains a rare but potentially fatal adverse event. We describe an autopsy-confirmed case of complete tumor remission with cavitation leading to fatal hemoptysis after chemoradiotherapy (CRT) followed by combined ipilimumab and nivolumab therapy for squamous cell carcinoma.Patient concerns:A 68-year-old man with a history of heavy smoking was found to have a 70-mm right hilar lung lesion and was diagnosed with stage IIIC squamous cell carcinoma with 50% programmed death ligand-1 expression. After CRT and subsequent combination chemotherapy plus immune checkpoint inhibitors, he developed pneumonitis requiring prednisolone (PSL). During PSL tapering, he experienced fever and a new right upper lobe cavitary lesion, which enlarged despite empiric antibiotics. After discharge under apparently stable conditions on a reduced PSL dose, he suddenly developed massive hemoptysis.Diagnoses:The primary diagnosis was stage IIIC squamous cell carcinoma of the lung. During the treatment course, he developed immune checkpoint inhibitor–related pneumonitis and a new cavitary lesion in the right upper lobe. Autopsy revealed a necrotic cavitary lesion with vascular destruction in the right upper lobe, dense adhesions between the lung and chest wall, and no residual carcinoma, infection, or distant metastases, confirming complete tumor remission and fatal hemoptysis due to treatment-related vascular injury.Interventions:The patient received definitive CRT with carboplatin and paclitaxel plus 60 Gy thoracic radiotherapy, followed by carboplatin and paclitaxel combined with ipilimumab and nivolumab, and subsequently maintenance ipilimumab and nivolumab. Immune-related pneumonitis was treated with systemic PSL, which was tapered and subsequently re-escalated when fever and a new cavitary lesion appeared. Empiric broad-spectrum antibiotics were also administered.Outcomes:CRT and combined immune checkpoint inhibitor therapy achieved complete tumor remission pathologically. However, the progressive cavitary change in the irradiated lung ultimately resulted in massive hemoptysis, cardiac arrest, and death. Autopsy confirmed a necrotic cavity with vascular destruction without residual malignancy or infection.Lessons:In patients receiving ipilimumab and nivolumab after thoracic CRT for centrally located squamous cell carcinoma, rapid tumor necrosis and cavitation within irradiated lung with fragile vasculature may markedly increase the risk of severe and fatal hemoptysis. When initiating or resuming combined immune checkpoint inhibitor therapy in this setting, clinicians should carefully consider CRT-associated vascular fragility and potential immune-mediated vascular injury, and ensure close imaging follow-up and airway surveillance.

Background. Patients with pleural mesothelioma and constitutional jaundice characterized by predominantly elevated direct bilirubin (DBL) are treated with a combination of nivolumab (Nivo) and ipilimumab (Ipi). Case. A 73-year-old man visited our hospital with a chief complaint of right pleural effusion. Constitutional jaundice with DBL predominance (total bilirubin [TBL] 3.3 mg/dl, DBL 2.2 mg/dl) had been observed for an extended time. A pleural biopsy confirmed the diagnosis of pleural mesothelioma. The tumor was judged to be unresectable, and combination therapy with Nivo and Ipi was initiated. Immediately prior to administration of therapy, TBL was 1.8 mg/dl, DBL was 1.6 mg/dl, aspartate aminotransferase (AST) was 13 U/l, and alanine aminotransferase (ALT) was 15 U/l. Two weeks after the administration, the patient complained of a fever and general fatigue. Hepatic impairment appeared on day 21 after administration. TBL was 16.4 mg/dl, DBL was 13.0 mg/dl, AST was 2,289 U/l, ALT was 1,594 U/l, prothrombin (PT) activity was 16%, and ammonia (NH3) was 151 μg/dl. The patient was diagnosed with immune checkpoint inhibitor (ICI)-induced liver injury. Oral prednisolone (PSL) 60 mg (1 mg/kg) was administered. On day 9 after ICI-induced liver injury, TBL rose to 26.1 mg/dl and DBL to 19.7 mg/dl. However, AST decreased to 236 U/l, ALT to 434 U/l, NH3 to 91 μg/dl, and PT activity rose to 31%. By day 38 of ICI-induced liver injury, AST, ALT, and PT levels were normal. A liver biopsy performed on day 125 of ICI-induced liver injury showed no dark melanin-like pigments in the hepatocytes. On day 211 following the onset of ICI-induced liver injury, the TBL and DBL levels returned to pre-treatment levels. Conclusions. A patient with DBL-predominant constitutional jaundice developed ICI-induced liver injury. After the administration of PSL, the AST and ALT levels improved immediately, but jaundice persisted.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory demyelinating disease primarily affecting the optic nerves and spinal cord. Polymyositis (PM) is an idiopathic inflammatory myopathy characterized by proximal muscle weakness. The anti-signal recognition particle (SRP) antibody is a myositis-specific autoantibody. We herein present a case of anti-SRP antibody-positive PM that developed 6years after the onset of NMOSD. A 52-year-old woman was diagnosed with NMOSD 6years previously based on left visual disturbance, a high signal intensity and contrast enhancement of the optic nerve on short τ inversion recovery (STIR)-magnetic resonance imaging (MRI), and anti-aquaporin 4 antibody positivity. She received methylprednisolone pulse therapy followed by oral prednisolone (PSL) at a starting dose of 40mg daily. Three years later, due to recurrent numbness in the left lower limb and difficulty in reducing the PSL dose to ≤10mg/day, satralizumab was initiated. At 52years old, she developed myalgia and muscle weakness in both thighs. PM was diagnosed based on an elevated serum creatine kinase level, anti-SRP antibody positivity, high signal intensities in the right triceps and bilateral adductor muscles on STIR-MRI, and muscle biopsy findings. Treatment with high-dose PSL and tacrolimus markedly attenuated her symptoms. Satralizumab was continued for NMOSD stabilisation. Previous studies reported the coexistence of NMOSD and autoimmune diseases; however, NMOSD with PM/DM is rare. We described a case of NMOSD with anti-SRP antibody-positive PM and provided a literature review.

Reactivation of cytomegalovirus (CMV) remains a significant concern in patients receiving immunosuppressive therapy. Although CMV infection and reactivation is associated with intrahepatic cholestasis in infants and immunosuppressive adults, intrahepatic cholestasis caused by CMV infection is rare in immunocompetent adults. Here, we report a case with CMV-induced intrahepatic cholestasis in an immunocompetent adult complicated by hemophagocytic syndrome (HPS). We excluded CMV infection from differential diagnosis of intrahepatic cholestasis based on serum CMV antibody profiles and immunocompetency at the initial assessment and then started oral administration of prednisolone (PSL). The development of CMV-induced lethal HPS after the treatment with PSL led us to reconsider the possibility of CMV infection as an etiology of cholestasis. Frozen serum before PSL administration exhibited a high copy number of CMV DNA and thus we reached the final diagnosis of CMV-induced intrahepatic cholestasis complicated by HPS. Resolution of cholestasis and HPS was achieved by ganciclovir. The patient exhibited elevated serum levels of IL-18, C-C motif chemokine ligand 2, and IL-13 with no alterations in serum levels of IFN-γ or TNF-α. This case underscores the importance of consideration of CMV reactivation as a differential diagnosis for intrahepatic cholestasis even in immunocompetent patients.

Factors affecting bone strength have not been elucidated in patients with rheumatoid arthritis (RA) undergoing silicone implant arthroplasty. In this observational study, we aimed to investigate factors affecting bone strength. We measured toughness in terms of bone strength, bone mineral density, and bone quality of 105 resected metacarpal heads (I:32, II:36, III:33, IV:2, V:2) obtained during silicone implant arthroplasty at the metacarpophalangeal (MP) joint in patients with RA. Bone strength was associated with all parameters in micro-computed tomography (µCT) and all parameters in bone histomorphometry, other than osteoid volume and osteoid surface. Clinical factors associated with bone strength included prednisolone (PSL) dose at the time of surgery, % young adult mean, T-score and the Rooney score. Bone volume fraction by µCT only influenced bone strength per multiple linear regression analysis. Compared to joints with higher bone strength, those with lower bone strength had a higher magnitude of implant subsidence, which assumed to the risk of reduced mobility and implant fracture after the operation. Suppressing the degradation of subchondral bone structures by continuously ameliorating disease activity without the use of PSL appeared to be crucial to maintain the radiological appearance of silicone implant arthroplasty at the MP joint.Supplementary InformationThe online version contains supplementary material available at 10.1038/s41598-025-31762-z.

ObjectivesIn systemic lupus erythematosus (SLE), a serologically active clinically quiescent (SACQ) state is defined as one in which high anti-DNA antibody levels or low complement levels persist, however, disease activity remains stable. Although treatment intensification is not recommended for the SACQ state under the Treat to Target strategy, SACQ has been reported to be a risk factor for flares. The present study aimed to evaluate the efficacy of adding belimumab (BLM) to hydroxychloroquine (HCQ), the standard treatment for SLE, for patients in the SACQ state.MethodsPatients treated with BLM were defined as the exposure group and those who did not were defined as the control group. Propensity score analysis with inverse probability of treatment weighting was used to analyze outcomes. The primary outcome was analyzed using the Kaplan-Meier method to determine time-to-event achievement to reduce prednisolone (PSL) dose to 7.5mg/day or 5mg/day. Secondary outcomes were as follows: (1) time to flares, analyzed using the Kaplan-Meier method, and (2) difference in median PSL dose at the last observation, analyzed using the Mann-Whitney U-test.ResultsOf the 146 patients in the SACQ state who received HCQ, 27 were included in the exposure group and 107 in the control group. The primary outcome, time-to-event achievement to reduce PSL dose to 7.5mg/day and 5mg/day had an HR value of 1.21 (95% confidence interval (CI) 0.78-1.89, p = 0.396) and 1.19 (95% CI 0.74-1.89, p = 0.471) in the two groups, respectively, with no significant difference between the two groups. No significant differences were observed in other outcomes.ConclusionsAdding BLM to HCQ in patients in the SACQ state did not demonstrate effects in preventing flares or reducing glucocorticoids (GC) dose.

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a systemic inflammatory disorder characterized by abundant infiltration of IgG4-positive lymphocytes and plasma cells with fibrosis in the involved organs. Periarterial lesions are relatively rare manifestations of IgG4-RD, and their clinical and imaging findings remain unclear. A 66-year-old man presented with persistent upper abdominal pain. Contrast-enhanced computed tomography (CECT) showed a gradually enhanced 47mm soft tissue mass lesion adjacent to the pancreatic uncinate and spreading around the superior mesenteric artery. Considering the imaging findings, pancreatic cancer could not be ruled out. We decided to perform an endoscopic ultrasound-guided fine-needle biopsy to confirm the histological diagnosis; however, we could not obtain a sufficient amount of tissue. Finally, the patient was diagnosed with IgG4-related periarteritis by performing a percutaneous biopsy of the lesion. The patient was treated with oral prednisolone (PSL) and approximately six months after PSL induction, CECT revealed marked shrinkage of the lesion. Although imaging findings are important for diagnosis, histopathological findings are essential for accurate clinical diagnosis. All possible means, including percutaneous targeted biopsy, should be attempted to acquire sufficient tissue to confirm the pathological diagnosis to enable appropriate treatment.