Dupilumab combined with corticosteroid therapy for Kimura disease with multiple systemic masses: a case report and literature review.

IF 5.7 2区 医学 Q1 IMMUNOLOGY Frontiers in Immunology Pub Date : 2024-10-24 eCollection Date: 2024-01-01 DOI:10.3389/fimmu.2024.1492547
Yansi Lyu, Yaqian Cui, Li Ma, Lvxin Guan, Ziping Wen, Jingkai Huang, Minglan Shi, Suchun Hou
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Abstract

To date, the pathogenesis of Kimura's disease remains unclear, there is no unified diagnostic criterion, the clinical phenotype shows considerable heterogeneity, and there is a lack of optimal treatment strategies. Due to its rarity, treatment strategies for KD are still under exploration. This paper reports a case of a 37-year-old Chinese female presenting with generalized erythematous papules and pruritic eruptions for 12 years, followed by the onset of limb swellings 3 years later, ultimately diagnosed as Kimura's disease. Considering the patient's multiple lymphadenopathies and limb swellings with concurrent atopic dermatitis, the treatment regimen included initial dupilumab dosage of 600 mg (300 mg administered in two injections), followed by subcutaneous injections of 300 mg every two weeks for four months. Concurrent oral corticosteroid therapy (methylprednisolone, initial dose 16 mg/kg/day, gradually tapered with tumor regression) was also administered. Following treatment, the patient did not experience severe adverse effects, and the multiple nodules markedly decreased in size. Additionally, serum IgE levels, eosinophil, and basophil counts showed significant reductions. These results demonstrate the significant efficacy of dupilumab combined with oral corticosteroids in treating Kimura's disease with concurrent atopic dermatitis.

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杜匹单抗联合皮质类固醇治疗伴有多发性系统性肿块的木村病:病例报告和文献综述。
迄今为止,木村氏病的发病机制仍不清楚,没有统一的诊断标准,临床表型表现出相当大的异质性,也缺乏最佳的治疗策略。由于其罕见性,KD 的治疗策略仍在探索之中。本文报告了一例37岁的中国女性患者,她出现全身红斑丘疹和瘙痒性糜烂12年,3年后出现肢体肿胀,最终被诊断为木村氏病。考虑到患者患有多发性淋巴结病和肢体肿胀,并同时患有特应性皮炎,治疗方案包括初始剂量为 600 毫克(300 毫克分两次注射),随后每两周皮下注射 300 毫克,持续四个月。患者还同时接受了口服皮质类固醇治疗(甲基强的松龙,初始剂量为 16 毫克/千克/天,随着肿瘤消退逐渐减量)。治疗后,患者没有出现严重的不良反应,多发性结节明显缩小。此外,血清 IgE 水平、嗜酸性粒细胞和嗜碱性粒细胞计数也显著下降。这些结果表明,dupilumab 联合口服皮质类固醇治疗并发特应性皮炎的木村氏病疗效显著。
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来源期刊
CiteScore
9.80
自引率
11.00%
发文量
7153
审稿时长
14 weeks
期刊介绍: Frontiers in Immunology is a leading journal in its field, publishing rigorously peer-reviewed research across basic, translational and clinical immunology. This multidisciplinary open-access journal is at the forefront of disseminating and communicating scientific knowledge and impactful discoveries to researchers, academics, clinicians and the public worldwide. Frontiers in Immunology is the official Journal of the International Union of Immunological Societies (IUIS). Encompassing the entire field of Immunology, this journal welcomes papers that investigate basic mechanisms of immune system development and function, with a particular emphasis given to the description of the clinical and immunological phenotype of human immune disorders, and on the definition of their molecular basis.
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