Immunological Medicine最新文献

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Overlapping Aicardi-Goutières and Singleton-Merten syndromes with a heterozygous gain-of-function mutation in IFIH1 mimicking juvenile idiopathic arthritis. 重叠aicardii - gouti<e:1>综合征和Singleton-Merten综合征伴IFIH1杂合功能获得突变,模拟幼年特发性关节炎。
IF 2.9
Immunological Medicine Pub Date : 2025-09-01 Epub Date: 2025-03-21 DOI: 10.1080/25785826.2025.2479148
Susumu Yamazaki, Shuya Kaneko, Asami Shimbo, Hitoshi Irabu, Ryo Ogino, Takayuki Miyamoto, Kazushi Izawa, Yuko Segawa, Jun Kakizaki, Masaaki Mori, Masaki Shimizu
{"title":"Overlapping Aicardi-Goutières and Singleton-Merten syndromes with a heterozygous gain-of-function mutation in <i>IFIH1</i> mimicking juvenile idiopathic arthritis.","authors":"Susumu Yamazaki, Shuya Kaneko, Asami Shimbo, Hitoshi Irabu, Ryo Ogino, Takayuki Miyamoto, Kazushi Izawa, Yuko Segawa, Jun Kakizaki, Masaaki Mori, Masaki Shimizu","doi":"10.1080/25785826.2025.2479148","DOIUrl":"10.1080/25785826.2025.2479148","url":null,"abstract":"<p><p>Aicardi-Goutières syndrome (AGS) and Singleton-Merten syndrome (SMS) are associated with heterozygous gain-of-function mutations in the interferon induced with helicase C domain 1 (IFIH1) gene. Recent reports describe patients exhibiting overlapping clinical features of AGS and SMS, along with marked type I interferon (IFN) overproduction. However, the clinical characteristics and optimal treatment strategies remain unclear. Herein, we present a patient with overlapping clinical features of AGS and SMS who was initially misdiagnosed with juvenile idiopathic arthritis. Surgical soft tissue release of the hip and knee joints improved joint deformities and spastic paraparesis. Baricitinib effectively treated refractory chilblains and skin ulcers while reducing IFN-stimulated gene overexpression in peripheral blood. These findings indicate that baricitinib may be a safe and effective treatment for AGS-SMS overlap, and surgical intervention may be a viable option for refractory joint deformities with spastic paraparesis.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"256-260"},"PeriodicalIF":2.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143674590","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
When ocular surface treatment fails: exploring neuropathic ocular pain in aqueous-deficient dry eye due to Sjögren's syndrome treated with mirogabalin: a case study. 当眼表治疗失败:探索因Sjögren综合征治疗的缺水型干眼症的神经性眼痛:一个案例研究。
IF 2.9
Immunological Medicine Pub Date : 2025-09-01 Epub Date: 2025-03-17 DOI: 10.1080/25785826.2025.2477321
Yoshiaki Tagawa, Yusuke Aihara, Susumu Ishida
{"title":"When ocular surface treatment fails: exploring neuropathic ocular pain in aqueous-deficient dry eye due to Sjögren's syndrome treated with mirogabalin: a case study.","authors":"Yoshiaki Tagawa, Yusuke Aihara, Susumu Ishida","doi":"10.1080/25785826.2025.2477321","DOIUrl":"10.1080/25785826.2025.2477321","url":null,"abstract":"<p><p>Dry eye disease can be classified as aqueous-deficient, evaporative and mixed-type. Recently, neuropathic pain has been indicated to be involved in the subjective symptoms of aqueous-deficient dry eye. However, the mechanism underlying neuropathic ocular pain in such patients remains elusive. Here, we present the case of a patient with aqueous-deficient dry eye disease and severe corneal and conjunctival epithelial damage due to Sjögren's syndrome. Despite considerable improvements in ocular surface conditions with local treatments, such as punctal plugs, autologous serum eye drops, rebamipide eye drops, and tacrolimus eye drops, the patient experienced severe ocular pain for two years, which was disproportionate to the objective clinical findings. The pain worsened during the exacerbations of systemic symptom, such as arthritis and interstitial pneumonia, without correlating with the ocular surface findings. The administration of mirogabalin, a neuropathic pain medication, effectively alleviated the patient's subjective ocular pain symptoms. Ocular pain in patients with Sjögren's syndrome is not necessarily linked to an aqueous deficiency or ocular surface findings. It can arise as neuropathic ocular pain due to inflammation spreading to the trigeminal nerve. Given the systemic inflammatory condition and characteristics of the ocular pain, administering neuropathic pain medications should be considered as a treatment option.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"261-265"},"PeriodicalIF":2.9,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143650820","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 : 2025-08-25 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":"https://doi.org/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":"1-11"},"PeriodicalIF":2.9,"publicationDate":"2025-08-25","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
Successful tocilizumab monotherapy for multicentric Castleman disease complicated by rheumatoid arthritis and Sjögren's disease: a case-based literature review. 托珠单抗单药治疗多中心Castleman病合并类风湿关节炎和Sjögren病的成功:一项基于病例的文献综述
IF 2.9
Immunological Medicine Pub Date : 2025-08-14 DOI: 10.1080/25785826.2025.2547444
Koji Suzuki, Mitsuhiro Akiyama, Koichi Saito, Kanako Shimanuki, Yuko Kaneko
{"title":"Successful tocilizumab monotherapy for multicentric Castleman disease complicated by rheumatoid arthritis and Sjögren's disease: a case-based literature review.","authors":"Koji Suzuki, Mitsuhiro Akiyama, Koichi Saito, Kanako Shimanuki, Yuko Kaneko","doi":"10.1080/25785826.2025.2547444","DOIUrl":"https://doi.org/10.1080/25785826.2025.2547444","url":null,"abstract":"<p><p>Multicentric Castleman disease (MCD) is a rare and complex lymphoproliferative disorder marked by multiple lymph node enlargement, characteristic histopathological features, and systemic inflammation driven by excessive interleukin-6 (IL-6) production. MCD can occasionally coexist with other autoimmune diseases, but the therapeutic approach in such cases remains poorly defined. Here, we present the first documented case of MCD complicated by both rheumatoid arthritis (RA) and Sjögren's disease (SjD), where all conditions were successfully treated with tocilizumab (TCZ) monotherapy, an IL-6 receptor antagonist. Furthermore, a literature review identified two previously reported cases of MCD with either RA or SjD, all of which showed favorable responses to TCZ. Our findings highlight the potential of TCZ as an effective therapeutic option for patients with MCD in the context of coexisting autoimmune disorders, offering new hope for managing these challenging clinical scenarios.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-8"},"PeriodicalIF":2.9,"publicationDate":"2025-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144856703","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
Rapid resolution of fever and panniculitis after anifrolumab in a patient with systemic lupus erythematosus refractory to standard immunosuppression. 对标准免疫抑制难治性系统性红斑狼疮患者进行无瘤单抗治疗后发热和全身炎的快速缓解。
IF 2.9
Immunological Medicine Pub Date : 2025-08-06 DOI: 10.1080/25785826.2025.2543612
Shunichiro Hanai, Yoshiaki Kobayashi, Taro Iwamoto, Daiki Nakagomi
{"title":"Rapid resolution of fever and panniculitis after anifrolumab in a patient with systemic lupus erythematosus refractory to standard immunosuppression.","authors":"Shunichiro Hanai, Yoshiaki Kobayashi, Taro Iwamoto, Daiki Nakagomi","doi":"10.1080/25785826.2025.2543612","DOIUrl":"https://doi.org/10.1080/25785826.2025.2543612","url":null,"abstract":"<p><p>Anifrolumab, a monoclonal antibody against type I interferon (IFN) receptor, has shown high efficacy against systemic lupus erythematosus (SLE) in clinical trials. Although rapid effects of anifrolumab against cutaneous manifestations of SLE have been reported, efficacy has still been considered to take a month or more, and shorter-term efficacy has not been described. A 29-year-old Japanese woman developed fever, erythema on the trunk and both upper and lower extremities, cytopenia, pericardial effusion, and acute confusional state and was diagnosed with SLE. Intravenous methylprednisolone pulse therapy followed by oral prednisolone at 50 mg/day improved her confusional state. Intravenous cyclophosphamide at 500 mg was added, and prednisolone was reduced to 40 mg/day. Fever and erythema on the upper extremities recurred shortly afterwards. Skin biopsy revealed panniculitis. After intravenously administering a single 300-mg dose of anifrolumab, fever resolved within a day, and erythema entirely disappeared within about 2 weeks. The serum IFN-α concentration decreased significantly after a single infusion of anifrolumab. Anifrolumab infusions every 4 weeks were continued, then prednisolone was tapered to 1 mg/day under anifrolumab therapy over 22 months. Anifrolumab may provide improvement on a daily basis even in patients refractory to the standard of care.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-5"},"PeriodicalIF":2.9,"publicationDate":"2025-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144790191","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
Muscle strength recovery in patients with idiopathic inflammatory myopathy with different myositis-specific autoantibodies. 具有不同肌炎特异性自身抗体的特发性炎性肌病患者的肌力恢复。
IF 2.9
Immunological Medicine Pub Date : 2025-07-29 DOI: 10.1080/25785826.2025.2537472
Naoki Mugii, Yasuhito Hamaguchi, Fujiko Someya, Pleiades Tiharu Inaoka, Sho Horie, Natsumi Fushida, Tasuku Kitano, Ko Fujii, Jiro Nishio, Kyosuke Oishi, Takashi Matsushita
{"title":"Muscle strength recovery in patients with idiopathic inflammatory myopathy with different myositis-specific autoantibodies.","authors":"Naoki Mugii, Yasuhito Hamaguchi, Fujiko Someya, Pleiades Tiharu Inaoka, Sho Horie, Natsumi Fushida, Tasuku Kitano, Ko Fujii, Jiro Nishio, Kyosuke Oishi, Takashi Matsushita","doi":"10.1080/25785826.2025.2537472","DOIUrl":"https://doi.org/10.1080/25785826.2025.2537472","url":null,"abstract":"<p><p>This study aims to assess muscle strength recovery in patients with idiopathic inflammatory myopathy (IIM) with three myositis-specific autoantibodies (MSAs). Forty-eight IIM patients (19 with anti-TIF1-γ Ab, 21 with anti-ARS Ab, and 8 with anti-SRP Ab) were included. Physical exercise began one week after starting medication. Muscle strength was measured using a hand held dynamometer. CK levels, muscle strength recovery, manual muscle test 8 (MMT8), and the Barthel index (BI) scores were evaluated before and after treatment among patients with anti-TIF1-γ, anti-ARS or anti-SRP Abs. CK levels decreased after one week of medication, and physical exercise did not worsen muscle involvement. Patients with anti-TIF1-γ and anti-ARS Abs exhibited rapid muscle strength improvement, while those with anti-SRP Ab had slower recovery. MMT8 followed a similar trend. BI scores significantly improved in patients with anti-TIF1-γ and anti-ARS Abs. All eight patients with anti-SRP Ab achieved a BI score of 100 despite no significant changes due to high variability. Muscle strength improved significantly, even in patients with a BI score ≤ 60. Muscle strength was recovered regardless of their MSA profile, and physical exercise may be safe for restoring muscle strength.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-9"},"PeriodicalIF":2.9,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144733679","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
Vogt-Koyanagi-Harada syndrome potentially associated with COVID-19 vaccination: a case report and literature review. Vogt-Koyanagi-Harada综合征可能与COVID-19疫苗接种相关:病例报告和文献综述
IF 2.7
Immunological Medicine Pub Date : 2025-07-09 DOI: 10.1080/25785826.2025.2528331
Zhiqiang Cui, Yan Luo, Yanli Yi, Xuli Guo, Yuqi Liu, Xiang Wang, Xiaonan Liu
{"title":"Vogt-Koyanagi-Harada syndrome potentially associated with COVID-19 vaccination: a case report and literature review.","authors":"Zhiqiang Cui, Yan Luo, Yanli Yi, Xuli Guo, Yuqi Liu, Xiang Wang, Xiaonan Liu","doi":"10.1080/25785826.2025.2528331","DOIUrl":"https://doi.org/10.1080/25785826.2025.2528331","url":null,"abstract":"<p><p>To investigate the potential association between COVID-19 vaccination and Vogt-Koyanagi-Harada (VKH) syndrome, offering novel insights for the diagnosis and management of vaccine-related ocular disorders. A case report combined with a literature review was conducted. A 19-year-old male developing VKH after receiving the second dose of an inactivated COVID-19 vaccine was analyzed. Clinical features, treatment outcomes (glucocorticoid therapy with 2-year follow-up), and literature-based comparisons were evaluated. PubMed-indexed cases of vaccine-associated VKH were systematically reviewed, and causality was assessed using the Naranjo Adverse Drug Reaction Probability Scale. The patient presented with bilateral blurred vision 14 days post-vaccination, diagnosed as VKH with retinal neuroepithelial detachment via fluorescein angiography (FFA) and optical coherence tomography (OCT). Oral prednisone (starting at 60 mg/day, tapered gradually)restored visual acuity to near-normal levels (OD: 20/40, OS: 20/33), consistent with the patient's reported baseline vision. Within 8 weeks, with no recurrence during follow-up. Literature analysis revealed vaccine-associated VKH symptoms typically emerged at a median of 8 days post-vaccination, aligning with the WHO's 40-day adverse event monitoring window. A Naranjo score of 4 indicated a probable vaccine-triggered immune response. COVID-19 vaccines may induce VKH via immune dysregulation mechanisms, particularly in genetically predisposed individuals. Although causality remains unconfirmed, clinicians should maintain vigilance for acute bilateral uveitis post-vaccination. Glucocorticoid therapy demonstrates efficacy in symptom resolution and relapse prevention. Enhanced active surveillance and mechanistic studies on vaccine-related ocular adverse events are warranted.</p>","PeriodicalId":37286,"journal":{"name":"Immunological Medicine","volume":" ","pages":"1-14"},"PeriodicalIF":2.7,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144592532","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 2.7
Immunological Medicine Pub 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":"https://doi.org/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":"1-10"},"PeriodicalIF":2.7,"publicationDate":"2025-07-08","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 2.7
Immunological Medicine Pub 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":"https://doi.org/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":"1-12"},"PeriodicalIF":2.7,"publicationDate":"2025-06-24","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
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 2.7
Immunological Medicine Pub 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":"https://doi.org/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":"1-4"},"PeriodicalIF":2.7,"publicationDate":"2025-06-13","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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