{"title":"Anti-striated muscle antibody positive anti-PD-1 antibody related myositis and cardiomyositis: an instructive autopsy case report with discussion for trigger of irAE.","authors":"Ayaka Kamada, Taichi Nomura, Terufumi Kubo, Takuto Ogasawara, Kazuki Yamada, Kaede Ishikawa, Yuki Oshima, Wataru Kawaharata, Tomoki Kikuchi, Masahiko Obata, Shigehisa Ura, Ichiro Yabe, Yoshihiko Hirohashi","doi":"10.1080/25785826.2026.2692148","DOIUrl":"https://doi.org/10.1080/25785826.2026.2692148","url":null,"abstract":"<p><p>Immune checkpoint inhibitors (ICIs) can cause severe immune-related adverse events (irAEs), including life-threatening myositis and myocarditis. Characterizing the immunopathological features of these conditions is essential for improving clinical recognition and management. We report a case of a 76-year-old man with metastatic renal cell carcinoma who developed fatigue, elevated creatine kinase, and complete atrioventricular block after receiving nivolumab. Despite pacemaker implantation and plasmapheresis, the patient died of respiratory failure. Notably, serum tests were positive for anti-Kv1.4 and anti-titin antibodies, although typical myasthenia gravis features were absent. Autopsy revealed CD8-positive T-cell and CD163-positive macrophage infiltration in the myocardium and skeletal muscles, while also confirming a pathological complete response of the carcinoma. Spatial analysis using digital pathology demonstrated that Human leukocyte antigen (HLA) class I expression in cardiomyocytes was closely apposed to infiltrating CD8-positiveT cells in a patchy distribution. This observation suggests a potential local feedback loop where activated T cells may induce HLA class I upregulation in adjacent muscle fibers, sustaining focal inflammation. This case provides important insights into the complex pathophysiology of fatal irAEs and underscores the value of postmortem examination in irAEs.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-7"},"PeriodicalIF":2.3,"publicationDate":"2026-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148273324","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical feature of anti-myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis with cerebrospinal fluid anti-N-methyl-D-aspartate receptor antibody co-positivity: a single center case series.","authors":"Kotaro Haruna, Junko Ishii, Kazuma Ota, Hajime Yoshimura, Satoru Fujiwara, Michi Kawamoto","doi":"10.1080/25785826.2026.2688713","DOIUrl":"https://doi.org/10.1080/25785826.2026.2688713","url":null,"abstract":"<p><p>Anti-myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis (MOG-CCE) occasionally exhibits cerebrospinal fluid (CSF) anti-N-methyl-D-aspartate receptor (NMDAR) antibody co-positivity; however, its clinical relevance remains unclear. This study examined the clinical characteristics of NMDAR antibody-positive and NMDAR antibody-negative MOG-CCE to clarify its clinical implications. Consecutive patients with MOG-CCE admitted to our hospital between January 2015 and July 2025 were included. Clinical, laboratory, and imaging findings, as well as treatments and outcomes, were descriptively analyzed according to the CSF anti-NMDAR antibody status. Ten cases with MOG-CCE were included, with five being NMDAR antibody-positive MOG-CCE. Compared to patients with NMDAR antibody-negative MOG-CCE, patients with NMDAR antibody-positive MOG-CCE appeared to have a higher frequency of psychiatric symptoms (80% vs. 20%), abnormal behavior (80% vs. 0%), and movement disorder (60% vs. 0%). Beyond fluid attenuated inversion recovery hyperintensity and gadolinium enhancement, cerebral perfusion imaging demonstrated regional hyperperfusion in most cases, irrespective of CSF anti-NMDAR antibody status. Functional outcomes were similar (median modified Rankin Scale at final follow-up 0 vs. 0). This study suggests that cases with NMDAR antibody-positive MOG-CCE may represent a synergistic overlap between MOG-CCE and NMDARE, characterized by NMDARE-like symptoms. Cerebral perfusion evaluation may serve as a supportive tool for the assessment of MOG-CCE.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-11"},"PeriodicalIF":2.9,"publicationDate":"2026-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148260002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Systemic lupus erythematosus complicated by hydrocephalus and neuromyelitis optica spectrum disorder presenting with complex neuropsychiatric manifestations: a case report.","authors":"Shinya Asatani, Masashi Uchikawa, Kiichi Sugito, Masahiro Nishihara, Miho Oshima, Kumiko Akiya, Hirotake Inomata, Noboru Kitamura, Hideki Nakamura","doi":"10.1080/25785826.2026.2688715","DOIUrl":"https://doi.org/10.1080/25785826.2026.2688715","url":null,"abstract":"<p><p>A 59-year-old woman with 45 years history of systemic lupus erythematosus (SLE) and antiphospholipid syndrome was admitted to our hospital with progressive disturbances in consciousness, cognitive impairment and paraparesis following pneumonia and cerebral infarction. Diagnostic workup revealed anti-aquaporin-4 antibody positivity and a longitudinally extensive transverse myelitis lesion, leading to a diagnosis of neuromyelitis optica spectrum disorder (NMOSD). Additionally, ventricular enlargement and cerebrospinal fluid (CSF) findings raised suspicion of normal pressure hydrocephalus (NPH). Treatment with methylprednisolone pulse therapy, hydroxychloroquine, and mycophenolate mofetil resulted in rapid clinical improvement. We propose that systemic inflammation and blood-brain barrier disruption, triggered by infection and infarction, facilitated the entry of autoantibodies and potentially exacerbated CSF dynamics. This case illustrates that neuropsychiatric symptoms in SLE can arise from the convergence of multiple distinct pathologies. Clinicians should maintain a high index of suspicion for overlapping conditions like NMOSD and NPH to ensure appropriate intervention, even when systemic lupus activity is not overtly high.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-8"},"PeriodicalIF":2.9,"publicationDate":"2026-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148253715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Utility of pretreatment GLOBE score as a non-invasive prognostic tool in primary biliary cholangitis.","authors":"Manabu Hayashi, Kazumichi Abe, Naoto Abe, Tatsuro Sugaya, Yosuke Takahata, Masashi Fujita, Hiromasa Ohira","doi":"10.1080/25785826.2026.2685924","DOIUrl":"https://doi.org/10.1080/25785826.2026.2685924","url":null,"abstract":"<p><p>Estimation of histological cirrhosis on diagnosis is useful for predicting prognosis in patients with primary biliary cholangitis (PBC). The pretreatment GLOBE score is associated with survival, but its relationship with histological cirrhosis remains unknown. We retrospectively investigated the pretreatment GLOBE score in PBC patients who had undergone liver biopsy. We analyzed the association between pretreatment GLOBE score and clinical findings, including histological cirrhosis and liver transplant (LT)-free survival rate. Among 183 patients with PBC, the pretreatment GLOBE score increased significantly with histological stage. The pretreatment GLOBE score demonstrated a high AUROC (0.868) than other noninvasive tools, with a cutoff value of 1.265 (95% CI: 1.195-1.585) for predicting histological cirrhosis. Patients with a pretreatment GLOBE score of < 1.265 had a significantly higher LT-free survival rate than those with a score of ≥ 1.265. A score of ≥ 1.265 was associated with lower LT-free survival rate independent of the presence of histological cirrhosis or GLOBE score 1 year after ursodeoxycholic acid treatment. Assessment of the pretreatment GLOBE score in PBC patients may be useful as a noninvasive prognostic tool.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-11"},"PeriodicalIF":2.9,"publicationDate":"2026-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148240412","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yinpeng Xu, Fang Li, Li Huang, Dan Mei, Pengqiang Du
{"title":"Comparative risk of Guillain-Barré syndrome recombinant and live zoster vaccines: a pharmacovigilance study using VAERS and EudraVigilance database.","authors":"Yinpeng Xu, Fang Li, Li Huang, Dan Mei, Pengqiang Du","doi":"10.1080/25785826.2026.2681258","DOIUrl":"https://doi.org/10.1080/25785826.2026.2681258","url":null,"abstract":"<p><p>This study aimed to compare the risk signals of Guillain-Barré syndrome (GBS) associated with recombinant zoster vaccine (RZV) and zoster vaccine live (ZVL) to improve understanding of herpes zoster vaccine safety. Disproportionality analyses were conducted using the Vaccine Adverse Event Reporting System (VAERS) and validated using the EudraVigilance database. Reporting odds ratio (ROR), proportional reporting ratio (PRR), and information component (IC) methods were applied, followed by time-to-onset and logistic regression analyses. A total of 482 GBS reports associated with RZV and 85 reports associated with ZVL were identified in VAERS. Significant disproportionality signals were detected for RZV but not for ZVL. Most GBS cases occurred within 30 days after RZV vaccination. Time-to-onset analysis indicated an early-failure pattern. Multivariable analysis showed that male sex and age ≥65 years were associated with an increased reporting risk of GBS following RZV vaccination. Sensitivity analyses restricted to cases occurring within 42 days of vaccination and to vaccines administered alone yielded consistent findings. Similar disproportionality signals for RZV were observed in EudraVigilance. This pharmacovigilance study identified a disproportionate reporting signal of GBS associated with RZV but not with ZVL. Although causality cannot be established, continued post-marketing surveillance of neurological adverse events following herpes zoster vaccination is warranted.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-13"},"PeriodicalIF":2.9,"publicationDate":"2026-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148227785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Joao Pedro M Lopes, Konrad Pisarczyk, Nicholas L Rider, Rachel Beckerman, Amanda Harrington
{"title":"Associations between disease factors and clinical outcomes in common variable immunodeficiency: a systematic literature review.","authors":"Joao Pedro M Lopes, Konrad Pisarczyk, Nicholas L Rider, Rachel Beckerman, Amanda Harrington","doi":"10.1080/25785826.2026.2678664","DOIUrl":"https://doi.org/10.1080/25785826.2026.2678664","url":null,"abstract":"<p><p>Common variable immunodeficiency (CVID) is associated with diverse clinical manifestations. This systematic review aimed to explore associations between disease factors and clinical outcomes in patients with CVID. Eligible studies included patients with CVID and reported associations between disease factors and clinical outcomes (e.g., autoimmune cytopenia, bronchiectasis, infections, lung damage, malignancy, and mortality). Quality assessment was conducted using the Newcastle-Ottawa Scale. Thirty-nine studies met inclusion criteria. The occurrence of lymphadenopathy or splenomegaly was associated with an increased risk of autoimmune cytopenia and lung damage specific to granulomatous-lymphocytic interstitial lung disease; splenomegaly was associated with increased risk of bronchiectasis. Higher levels of switched memory B (smB) cells and marginal zone B cells were associated with reduced risks of lung damage; higher levels of smB cells were associated with a reduced risk of autoimmune cytopenia. Elevated CD21<sup>low</sup> B cells were associated with increased risks of autoimmune cytopenia, lung damage, and infections. Higher total B cells were associated with lower risk of bronchiectasis and mortality. These findings suggest specific relationships are present between lymphoproliferation markers and B-cell subsets and clinical outcomes in CVID. Limitations including heterogeneity in study designs and low- to moderate-quality evidence underscore the need for additional research to validate these associations.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-17"},"PeriodicalIF":2.9,"publicationDate":"2026-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148144662","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Immunological MedicinePub Date : 2026-06-01Epub Date: 2025-10-18DOI: 10.1080/25785826.2025.2576285
Koji Suzuki, Yuko Kaneko
{"title":"Updates on the pathogenesis and molecular-targeted therapies of Still's disease.","authors":"Koji Suzuki, Yuko Kaneko","doi":"10.1080/25785826.2025.2576285","DOIUrl":"10.1080/25785826.2025.2576285","url":null,"abstract":"<p><p>Still's disease is a systemic inflammatory disorder involving both innate and adaptive immunity. Although its pathogenesis remains incompletely understood, immune cells such as neutrophils, macrophages, and T cells, along with pro-inflammatory cytokines, particularly interleukin (IL)-1, IL-6, IL-18, and interferon-γ (IFN-γ), play central roles in disease development. While glucocorticoids remain the first-line treatment, the advent of molecular-targeted therapies has revolutionized management. IL-1 and IL-6 inhibitors have shown efficacy in controlling disease activity and reducing glucocorticoid dependence. However, treatment responses vary among patients due to immunological heterogeneity. Recent studies have proposed clustering patients based on clinical features, immune cell profiles, and dominant cytokine signatures to help predict therapeutic responses. In addition, novel therapies targeting IL-18, IFN-γ, and Janus kinase (JAK) signaling pathways are emerging as promising options for refractory cases. This review summarizes current insights into the immunopathogenesis of Still's disease and explores the evidence supporting molecular-targeted therapies, paving the way toward personalized treatment strategies.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"130-146"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145318790","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Neural regulation of gut inflammation via autonomic nerves: therapeutic implications for inflammatory bowel disease.","authors":"Mai Hasegawa, Takaaki Kawaguchi, Hiroki Kiyohara, Toshiaki Teratani, Nobuhiro Nakamoto, Yohei Mikami, Takanori Kanai","doi":"10.1080/25785826.2025.2604347","DOIUrl":"10.1080/25785826.2025.2604347","url":null,"abstract":"<p><p>Inflammatory bowel disease (IBD) is a debilitating and treatment-refractory condition with a complex and incompletely understood etiology. Recent advances in neuroimmunology have revealed that intestinal homeostasis is regulated by bidirectional communication between the autonomic nervous system and the immune system. In particular, vagus nerve-mediated cholinergic anti-inflammatory signaling plays a central role in modulating intestinal immune responses and represents a key mechanism linking neural regulation to IBD pathogenesis and therapy. In parallel, the gut-brain axis has emerged as a critical regulator of intestinal inflammation and systemic disease. Psychological stress alters gut immune function through mechanisms involving enteric glial activation and microbiota-derived metabolites, contributing to treatment-resistant depression via immune activation and changes in short-chain fatty acid profiles. Moreover, gut microbiota dysbiosis has been implicated in neurodegenerative disorders such as Alzheimer's and Parkinson's diseases, potentially preceding central pathology through vagal signaling and systemic inflammation. Together, these findings position the gut microbiota as an immunological and neurological hub connecting intestinal, systemic, and brain health. This review summarizes recent advances in gut-brain axis-mediated immune regulation in IBD and highlights emerging bioelectronic neuromodulation strategies targeting autonomic circuits as promising non-pharmacological approaches to restore gut immune balance.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"164-187"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145811596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cutaneous sarcoidosis following complete remission of diffuse large B-cell lymphoma: a case report and literature review.","authors":"Shuhei Yoshida, Jumpei Temmoku, Kenji Saito, Haruki Matsumoto, Ryuto Mukaiyama, Erina Suzuki, Shotaro Ogawa, Yuya Sumichika, Tomoyuki Asano, Shuzo Sato, Yuko Hashimoto, Michiyuki Hakozaki, Toshiyuki Yamamoto, Kiyoshi Migita, Yasuhiro Shimojima","doi":"10.1080/25785826.2025.2576289","DOIUrl":"10.1080/25785826.2025.2576289","url":null,"abstract":"<p><p>Sarcoidosis is a multi-systemic granulomatous disease of unknown etiology. Its association with lymphoproliferative disorders is termed sarcoidosis-lymphoma syndrome, which is typically characterized by sarcoidosis preceding lymphoma. However, the development of sarcoidosis after lymphoma remission is rare. A 72-year-old woman with a history of diffuse large B-cell lymphoma, treated 11 years earlier, was admitted with a rash and weakness in the lower limbs. Multiple inflammatory lesions, predominantly in the lower limbs, were detected using computed tomography and fluorodeoxyglucose positron emission tomography, and a diagnosis of systemic sarcoidosis was established <i>via</i> biopsies of the skin lesion. We reviewed 11 cases of sarcoidosis with cutaneous manifestations after lymphoma treatment, including the present case. In addition, we emphasized the importance of considering cutaneous sarcoidosis in the differential diagnosis of post-treatment skin lesions alongside lymphoma recurrence, as well as the need for histopathological confirmation.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"244-252"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145401856","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Immunological MedicinePub Date : 2026-06-01Epub Date: 2025-11-27DOI: 10.1080/25785826.2025.2594828
Yuichiro Fujieda
{"title":"Neuropsychiatric manifestations in systemic lupus erythematosus and antiphospholipid syndrome: pathophysiology and current insights.","authors":"Yuichiro Fujieda","doi":"10.1080/25785826.2025.2594828","DOIUrl":"10.1080/25785826.2025.2594828","url":null,"abstract":"<p><p>Neuropsychiatric manifestations in systemic lupus erythematosus (SLE) and anti-phospholipid syndrome (APS) represent complex clinical challenges due to their diverse presentations and multifactorial pathogenesis. Neuropsychiatric SLE (NPSLE) affects approximately 30-40% of SLE patients, with symptoms ranging from cognitive dysfunction to severe conditions such as stroke, seizures, and psychosis. In APS, cerebrovascular events, including ischemic stroke and transient ischemic attacks, occur in ∼20% of patients, alongside non-thrombotic manifestations like migraine and chorea. The underlying mechanisms involve immune-mediated neuronal injury, vascular thrombosis, and neuroinflammation. In NPSLE, autoantibodies, inflammatory cytokines, and complement activation drive neuronal damage, while APS is primarily characterized by antiphospholipid antibody-mediated thrombosis, with additional direct neuronal effects. Advanced neuroimaging, including diffusion tensor imaging, functional MRI, and positron emission tomography, reveals subtle structural and functional brain alterations. Emerging biomarkers, such as neurofilament light chain and glial fibrillary acidic protein, show promise for detecting neural and glial injury. This review synthesizes current insights into the pathophysiology, diagnostic approaches to neuropsychiatric manifestations in SLE and APS, emphasizing the need for integrated clinical, imaging, and laboratory evaluations to improve diagnostic precision and patient outcomes.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"212-222"},"PeriodicalIF":2.9,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145640858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}