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Rapid response of eculizumab: a rescue therapy for ventilator-dependent refractory myasthenic crisis. eculizumab的快速反应:呼吸机依赖性难治性肌无力危象的抢救治疗。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-05-06 DOI: 10.1080/25785826.2025.2500698
Yohei Takenobu, Kazutoshi Ikeda, Sachiko Hasebe, Noriko Nomura, Shunsuke Tamaki, Kayoko Yukawa, Junichi Miyahara, Kentaro Yamakawa, Manabu Inoue
{"title":"Rapid response of eculizumab: a rescue therapy for ventilator-dependent refractory myasthenic crisis.","authors":"Yohei Takenobu, Kazutoshi Ikeda, Sachiko Hasebe, Noriko Nomura, Shunsuke Tamaki, Kayoko Yukawa, Junichi Miyahara, Kentaro Yamakawa, Manabu Inoue","doi":"10.1080/25785826.2025.2500698","DOIUrl":"10.1080/25785826.2025.2500698","url":null,"abstract":"<p><p>Myasthenic crisis (MC) represents the most severe and life-threatening complication of myasthenia gravis (MG). Some patients exhibit refractory responses to conventional immunotherapies, including intravenous immunoglobulin and plasma exchange. This report describes a patient with MC refractory to repetitive high-dose steroids and intravenous immunoglobulin, requiring ventilator support. Within 2 days of eculizumab administration, significant improvement enabled ventilator discontinuation. Subsequent doses further ameliorated limb and pharyngeal weakness, leading to independence. A literature review that identified ten cases reported across five publications highlighted the favorable outcomes achieved with eculizumab in refractory MC, while concomitant respiratory infection was shown to complicate the recovery from MG-related respiratory failure. Although the randomized controlled trials have excluded MC cases, eculizumab has emerged as a promising option for rescue therapy in refractory MC. Larger studies that specifically include MC cases are warranted.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"391-396"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144052695","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}
引用次数: 0
Immune checkpoint inhibitor-induced myocarditis and multiple adverse events with pre-existing rheumatoid arthritis: a case report and literature review. 免疫检查点抑制剂诱导的心肌炎和多重不良事件与预先存在的类风湿关节炎:1例报告和文献复习。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-06-05 DOI: 10.1080/25785826.2025.2515688
Shion Kachi, Mirei Shirakashi, Takashi Nomizo, Mei Onishi, Eri Toda Kato, Tomomi W Nobashi, Hideo Onizawa, Ryosuke Hiwa, Hideaki Tsuji, Shuji Akizuki, Ran Nakashima, Akira Onishi, Hajime Yoshifuji, Masao Tanaka, Kosaku Murakami, Akio Morinobu
{"title":"Immune checkpoint inhibitor-induced myocarditis and multiple adverse events with pre-existing rheumatoid arthritis: a case report and literature review.","authors":"Shion Kachi, Mirei Shirakashi, Takashi Nomizo, Mei Onishi, Eri Toda Kato, Tomomi W Nobashi, Hideo Onizawa, Ryosuke Hiwa, Hideaki Tsuji, Shuji Akizuki, Ran Nakashima, Akira Onishi, Hajime Yoshifuji, Masao Tanaka, Kosaku Murakami, Akio Morinobu","doi":"10.1080/25785826.2025.2515688","DOIUrl":"10.1080/25785826.2025.2515688","url":null,"abstract":"<p><p>Immune checkpoint inhibitors (ICIs) can trigger immune-related adverse events (irAEs) and rheumatoid arthritis (RA) reactivation in cancer patients with pre-existing RA. Studies indicate RA reactivation occurs in approximately 50% of these patients, while new irAEs develop in 25-50% of ICI-treated patients. Furthermore, ICI-induced myocarditis has been reported to have a high mortality rate, ranging from 25% to 50%. No prior reports have detailed the clinical course of ICI-induced myocarditis in patients with RA. We describe a 77-year-old man with RA who developed myocarditis, myositis, and RA flare following treatment with the PD-LI inhibitor, atezolizumab, for small-cell lung cancer. High-dose glucocorticoid (GC) therapy and intravenous immunoglobulin improved myocarditis and myositis. Corticosteroid tapering led to organizing pneumonia, necessitating a dosage adjustment. Once resolved, tapering resumed. During irAEs treatment, the patient maintained a partial response without cancer recurrence for ten months, and required no further cancer-specific therapy. To our knowledge, this is the first detailed report of ICI-induced myocarditis in a patient with pre-existing RA. Our findings emphasize the importance of vigilant monitoring of both irAEs and RA disease activity for optimal patient management.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"400-406"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144235463","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}
引用次数: 0
Ovariectomy-induced bone loss through inappropriate inflammatory response: an osteoimmunological perspective on postmenopausal osteoporosis. 卵巢切除术引起的骨质流失通过不适当的炎症反应:绝经后骨质疏松的骨免疫学观点。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-05-16 DOI: 10.1080/25785826.2025.2506870
Lilika Higuchi, Nozomi Ouchi, Yasuyuki Negishi, Munehiro Naruo, Maiko Kusano, Shunji Suzuki, Takahisa Okuda, Rimpei Morita
{"title":"Ovariectomy-induced bone loss through inappropriate inflammatory response: an osteoimmunological perspective on postmenopausal osteoporosis.","authors":"Lilika Higuchi, Nozomi Ouchi, Yasuyuki Negishi, Munehiro Naruo, Maiko Kusano, Shunji Suzuki, Takahisa Okuda, Rimpei Morita","doi":"10.1080/25785826.2025.2506870","DOIUrl":"10.1080/25785826.2025.2506870","url":null,"abstract":"<p><p>Postmenopausal osteoporosis (PO) is a prevalent condition that significantly impairs the quality of life in elderly women. While traditionally attributed to estrogen deficiency, emerging evidence suggests that immune dysregulation plays a critical role in its pathogenesis. This study investigates the osteoimmunological mechanisms underlying PO using an ovariectomy (Ovx) mouse model. Our findings indicate that Ovx mice exhibit substantial reductions in bone mineral density and bone volume, accompanied by a marked suppression of interleukin-4 (IL-4) and interferon-gamma (IFN-γ) production, particularly from natural killer T (NKT) cells. Lipidomic analysis of bone marrow further revealed an upregulation of omega-6 fatty acids, contributing to an inflammatory microenvironment that promotes excessive osteoclast activation. Notably, administration of the glycolipid OCH restored cytokine production and mitigated bone loss in Ovx mice, suggesting its therapeutic potential. These findings highlight the complex interplay between immune responses and lipid metabolism in PO and propose novel therapeutic strategies aimed at modulating immune function to prevent bone loss. This study offers valuable insights into the osteoimmunological mechanisms of PO and underscores the potential of immunomodulatory approaches for its management.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"357-370"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144081131","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}
引用次数: 0
Antibodies against Clostridium butyricum in the children of mothers at risk for gestational diabetes. 抗丁酸梭菌抗体的母亲的孩子在妊娠糖尿病的风险。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-05-23 DOI: 10.1080/25785826.2025.2504021
Celeste Peterson, Aili Tagoma, Kristi Alnek, Anu Bärenson, Tamara Vorobjova, Ija Talja, Helis Janson, Anne Kirss, Siiri Kõljalg, Aki Sinkkonen, Marja Irmeli Roslund, Raivo Uibo
{"title":"Antibodies against <i>Clostridium butyricum</i> in the children of mothers at risk for gestational diabetes.","authors":"Celeste Peterson, Aili Tagoma, Kristi Alnek, Anu Bärenson, Tamara Vorobjova, Ija Talja, Helis Janson, Anne Kirss, Siiri Kõljalg, Aki Sinkkonen, Marja Irmeli Roslund, Raivo Uibo","doi":"10.1080/25785826.2025.2504021","DOIUrl":"10.1080/25785826.2025.2504021","url":null,"abstract":"<p><p>Gestational diabetes mellitus (GDM) is linked to an imbalance in gut microbiota composition, which can be transferred to the mother's offspring. <i>Clostridium butyricum</i>, known for its health benefits in diabetes and allergy, lacks sufficient data regarding its effect on the immune system's development in the offspring of mothers with GDM. This study assessed antibody responses against <i>C. butyricum</i> T2F3 in children of mothers at risk for GDM, involving 88 children aged 1-6 years. Antibody responses were measured with flow cytometry and immunoblot. Lower IgG median fluorescence intensity (MFI) values and fewer IgA and IgG bands against <i>C. butyricum</i> were detected in children of mothers with GDM. Maternal body mass index was positively associated with children's IgG MFI and number of IgG bands. Fewer IgA bands were detected in children with higher IgE levels, atopic dermatitis, asthma, and allergic rhinitis. More IgG bands were detected in children with higher anti-β-lactoglobulin IgG levels. Children with autoimmune risk-related HLA-DR3/DQ2.5 had fewer IgA bands, while those with neutral HLA-DR1/DQ5 had higher IgA, but lower IgG MFI. These results indicate that maternal prenatal changes could affect their offspring's immune response against <i>C. butyricum</i>. Moreover, <i>C. butyricum</i> could have a protective role against allergic sensitization.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"346-356"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144129088","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}
引用次数: 0
The significance of tumor microenvironment for immunotherapy in melanoma and non-melanoma skin cancer. 肿瘤微环境在黑色素瘤和非黑色素瘤皮肤癌免疫治疗中的意义。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-08-25 DOI: 10.1080/25785826.2025.2550795
Taku Fujimura
{"title":"The significance of tumor microenvironment for immunotherapy in melanoma and non-melanoma skin cancer.","authors":"Taku Fujimura","doi":"10.1080/25785826.2025.2550795","DOIUrl":"10.1080/25785826.2025.2550795","url":null,"abstract":"<p><p>Immune checkpoint inhibitors (ICIs), such as anti-PD-1 antibody, have significantly changed the treatment landscape not only for unresectable melanoma but also for non-melanoma skin cancers. In addition, anti-PD-1 antibody administration methods have evolved and are now used in both the neoadjuvant and adjuvant settings. As these treatment strategies have been evaluated, it has become clear that understanding the role of the tumor microenvironment (TME) is critical to the success of anti-PD-1 antibody-based immunotherapy. For example, racial differences in the efficacy of immunotherapy in melanoma are influenced not only by tumor-related factors such as tumor mutational burden and microsatellite instability, but also by components of the TME, including tumor-associated macrophages, cancer-associated fibroblasts, and tumor-infiltrating lymphocytes (TILs), all of which can affect the therapeutic outcome of ICIs. Furthermore, studies conducted during the development of neoadjuvant therapies have shown that tumor-reactive TILs are densely localized within primary tumors and are closely associated with both treatment efficacy and the occurrence of immune-related adverse events. In this review, we discuss the therapeutic efficacy of currently available anti-PD-1 antibody-based immunotherapies for skin cancer and examine the role of the TME in influencing these therapeutic outcomes.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"307-317"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144972687","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}
引用次数: 0
Causal association of immune-related genes with mouth ulcers: findings from summary-based Mendelian randomization and transcriptome-wide association analysis. 免疫相关基因与口腔溃疡的因果关系:基于摘要的孟德尔随机化和转录组全关联分析的发现。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-05-05 DOI: 10.1080/25785826.2025.2498106
Mengru Shi, Tianqi Wang, Qi Xie, Guangwei Yuan, Juan Xia, Jingyun Yang, Weili Xie, Zetao Chen, Xiaobing Chen
{"title":"Causal association of immune-related genes with mouth ulcers: findings from summary-based Mendelian randomization and transcriptome-wide association analysis.","authors":"Mengru Shi, Tianqi Wang, Qi Xie, Guangwei Yuan, Juan Xia, Jingyun Yang, Weili Xie, Zetao Chen, Xiaobing Chen","doi":"10.1080/25785826.2025.2498106","DOIUrl":"10.1080/25785826.2025.2498106","url":null,"abstract":"<p><p>Oral ulceration is the most common ulcerative condition in humans, yet its underlying etiology remains poorly understood. To identify potential causal genes involved in the pathogenesis of mouth ulcers, we applied summary data-based Mendelian randomization (SMR) using eQTL data from GTEx and CAGE, along with a transcriptome-wide association study (TWAS). The SMR analysis of GTEx data identified 41 significant probes, with <i>LRRC37A4P</i>, <i>RP11-707O23.5</i>, and <i>RP11-259G18.3</i> standing out. In parallel, the CAGE SMR identified 67 probes corresponding to 58 genes, including <i>CCR2</i>, <i>MGC57346</i>, and <i>C17orf69</i>. TWAS further identified 181 significant genes, with 37 overlapping with GTEx SMR findings and 27 with CAGE SMR findings. Functional enrichment analysis revealed a strong involvement of immune-related pathways, especially those involving <i>HLA-DRB1</i> and <i>CCR2</i>. Differential expression analysis reinforced the relevance of <i>IL12RB1</i> and <i>HLA-DRB1</i>, which were consistently significant across both SMR and TWAS analyses. Collectively, these findings underscore the importance of immune-regulatory genes, particularly members of the CCR gene family and the HLA complex, in the genetic architecture of mouth ulcers. This integrative approach provides insights into potential therapeutic targets and advances our understanding of the genetic basis underlying this prevalent condition.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"335-345"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143989780","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}
引用次数: 0
Real-world effectiveness of belimumab and anifrolumab in systemic lupus erythematosus: comparable trends in disease activity and glucocorticoid reduction. belimumab和anifrolumab治疗系统性红斑狼疮的实际疗效:疾病活动性和糖皮质激素减少的可比趋势
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-07-08 DOI: 10.1080/25785826.2025.2528296
Ryota Sato, Masaru Shimizu, Yuya Kondo, Kazushi Maruo, Yuki Kuroda, Hiroshi Ebe, Mizuki Yagishita, Naoto Umeda, Seiji Mogi, Ayako Ohyama, Ayako Kitada, Saori Abe, Hiromitsu Asashima, Haruka Miki, Hiroto Tsuboi, Isao Matsumoto
{"title":"Real-world effectiveness of belimumab and anifrolumab in systemic lupus erythematosus: comparable trends in disease activity and glucocorticoid reduction.","authors":"Ryota Sato, Masaru Shimizu, Yuya Kondo, Kazushi Maruo, Yuki Kuroda, Hiroshi Ebe, Mizuki Yagishita, Naoto Umeda, Seiji Mogi, Ayako Ohyama, Ayako Kitada, Saori Abe, Hiromitsu Asashima, Haruka Miki, Hiroto Tsuboi, Isao Matsumoto","doi":"10.1080/25785826.2025.2528296","DOIUrl":"10.1080/25785826.2025.2528296","url":null,"abstract":"<p><p>Despite the proven efficacy of belimumab and anifrolumab for SLE, their relative effectiveness remains uncertain owing to conflicting indirect comparison studies. We conducted a comparative effectiveness study using multicenter health records to evaluate the adjusted 12-month changes in the SLE Disease Activity Index 2000 (SLEDAI-2K), glucocorticoid dose, anti-DNA antibody titers, and CH50 levels, along with the adjusted hazard ratios of discontinuation and adverse events. A total of 58 bionaïve lupus patients (belimumab: 36, anifrolumab: 22) who initiated belimumab or anifrolumab on or after January 1, 2022 were identified and followed for up to 2 years. Adjusted changes in the SLEDAI-2K, glucocorticoid dose, and anti-DNA antibody titers were similar, with between-group differences (belimumab - anifrolumab) of -0.4 SLEDAI-2K units (95% CI: -4.8, 4.0), 1.4 mg/day (95% CI: -5.1, 7.9), and -2.7 IU/mL (95% CI: -27, 21), respectively. Belimumab was associated with a greater increase in adjusted CH50 levels (between-group difference: 5.3 CH50/mL, 95% CI: -8.9, 20) and a lower adjusted hazard ratio of discontinuation (0.68, 95% CI: 0.12, 3.7). Infection was the most common adverse event (belimumab: 31%, anifrolumab: 41%). Our findings suggest both treatments provide similar effectiveness, with belimumab offering potential benefits in CH50 levels, treatment continuity, and infection risk.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"371-380"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144585162","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}
引用次数: 0
Current experience with manual push subcutaneous immunoglobulin (SCIg) in patients with immune deficiencies. 目前免疫缺陷患者使用手推皮下免疫球蛋白(SCIg)的经验。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-06-24 DOI: 10.1080/25785826.2025.2515333
Alex Richter
{"title":"Current experience with manual push subcutaneous immunoglobulin (SCIg) in patients with immune deficiencies.","authors":"Alex Richter","doi":"10.1080/25785826.2025.2515333","DOIUrl":"10.1080/25785826.2025.2515333","url":null,"abstract":"<p><p>Immunoglobulin G replacement therapy prevents infections in patients with antibody deficiencies. Subcutaneous immunoglobulin (SCIg) has typically been administered <i>via</i> infusion pump, but the manual push technique offers a simple, convenient alternative method. The manual push technique is efficacious, well tolerated, quick to administer, offers increased dosing flexibility, and does not rely on a pump. Having various administration options available to patients provides greater treatment satisfaction and feelings of self-empowerment, which may improve compliance. Currently available literature published before 10 February 2022, that reported patient and healthcare professional experience with SCIg administered <i>via</i> manual push, were reviewed. Literature searches were performed using PubMed, Google and ClinicalTrials.gov using key words 'manual push', 'rapid push', 'immunoglobulin', 'subcutaneous immunoglobulin', 'SCIg', and 'primary immunodeficiency'. Real-world evidence demonstrates all delivery techniques provide similar efficacy, so treatment administration becomes about patient preference, hospital resources, cost-effectiveness/recovery and clinician attitude. To establish newer administration modalities such as manual push or prefilled syringes, there needs to be patient awareness of these options, then education and finally confidence in recommending these options. Adoption of newer administration modalities will help ensure patients receive the widest range of choice, thus improving compliance and their risk of recurrent and severe infection.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"295-306"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144477146","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}
引用次数: 0
Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review. 系统性硬化症表现为TAFRO综合征样表现,包括肾小球微血管病变:1例报告及文献复习。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-03-21 DOI: 10.1080/25785826.2025.2481675
Hiroyuki Kawahara, Satoshi Hara, Noriko Iwaki, Hiroko Ikeda, Dai Inoue, Ichiro Mizushima, Hideki Nomura, Yasufumi Masaki, Yasunori Iwata, Mitsuhiro Kawano
{"title":"Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review.","authors":"Hiroyuki Kawahara, Satoshi Hara, Noriko Iwaki, Hiroko Ikeda, Dai Inoue, Ichiro Mizushima, Hideki Nomura, Yasufumi Masaki, Yasunori Iwata, Mitsuhiro Kawano","doi":"10.1080/25785826.2025.2481675","DOIUrl":"10.1080/25785826.2025.2481675","url":null,"abstract":"<p><p>TAFRO syndrome is a systemic inflammatory disorder of unknown etiology, and its diagnosis requires the exclusion of autoimmune diseases. A 42-year-old Japanese woman presented with TAFRO syndrome-like manifestations, but had undiagnosed limited-cutaneous systemic sclerosis preventing a definitive diagnosis of TAFRO syndrome. However, her clinical course and pathological findings, including renal glomerular microangiopathy, were consistent with TAFRO syndrome. We performed a systematic review of the literature to evaluate how autoimmunity affects the clinical characteristics of TAFRO syndrome/idiopathic multicentric Castleman disease (iMCD)-TAFRO. We reviewed 95 reported cases of TAFRO syndrome/iMCD-TAFRO and found that at least 41 (43.6%) had various autoantibodies. In particular, the positive rates of anti-nuclear antibody, anti-SS-A antibody, anti-SS-B antibody, PA-IgG, and direct Coombs test were high. Furthermore, we identified 14 cases of autoimmune diseases with TAFRO syndrome-like manifestations. We compared the clinical characteristics of these 14 with those of the autoantibody-positive and -negative cases among the 95 cases of TAFRO syndrome/iMCD-TAFRO. Apart from sex ratio, we found no significant difference in clinical presentation, treatment, or outcome among the groups. In conclusion, TAFRO syndrome/iMCD-TAFRO often accompanies autoantibodies and shares many clinical characteristics with other autoimmune diseases. Clinicians should be aware that some autoimmune diseases mimic TAFRO syndrome/iMCD-TAFRO.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"381-390"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143674660","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}
引用次数: 0
A case of early diagnosis of congenital multisystem langerhans cell histiocytosis with thymic involvement triggered by newborn screening. 新生儿筛查诱发胸腺受累的先天性多系统朗格汉斯细胞组织细胞增多症1例。
IF 2.9
Immunological Medicine Pub Date : 2025-12-01 Epub Date: 2025-06-13 DOI: 10.1080/25785826.2025.2519725
Yoji Uejima, Masayuki Iijima, Koichi Oshima, Ken Kawabata, Akihisa Nitta, Taiki Sato, Yoko Sato, Zenshiro Tamaki, Takahiro Hosokawa, Masaki Shimizu, Katsuyoshi Koh, Atsuko Nakazawa
{"title":"A case of early diagnosis of congenital multisystem langerhans cell histiocytosis with thymic involvement triggered by newborn screening.","authors":"Yoji Uejima, Masayuki Iijima, Koichi Oshima, Ken Kawabata, Akihisa Nitta, Taiki Sato, Yoko Sato, Zenshiro Tamaki, Takahiro Hosokawa, Masaki Shimizu, Katsuyoshi Koh, Atsuko Nakazawa","doi":"10.1080/25785826.2025.2519725","DOIUrl":"10.1080/25785826.2025.2519725","url":null,"abstract":"<p><p>T-cell receptor excision circle (TREC)-based newborn screening is a method for the early detection of severe combined immunodeficiency (SCID); however, undetectable TREC level results have also been reported in conditions other than SCID. To date, no studies have investigated the dynamics of TREC levels in patients with Langerhans cell histiocytosis (LCH). Here, we report a case of congenital multisystem LCH with thymic involvement diagnosed following a negative TREC result obtained through newborn screening. Partial remission was achieved with chemotherapy, and TREC levels subsequently recovered. LCH involving the thymus should be considered among the differential diagnoses for negative TREC results.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"407-410"},"PeriodicalIF":2.9,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144286704","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}
引用次数: 0
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