PDF(Pubmed)
Abstract：
Kimura\'s disease (KD) was first described in 1937. It is a rare, benign, and chronic immune-mediated inflammatory disorder affecting the subcutaneous tissue, salivary glands, and lymph nodes. The disease is more common in the second to third decades of life in middle-aged Southeast Asian countries. The cause of Kimura\'s disease remains unknown; some authors believe it is related to an autoimmune or delayed-type hypersensitivity reaction. It commonly presents as a solitary painless lymph node in the head and neck or generalized lymphadenopathy (67%-100%) associated with peripheral eosinophilia and elevated IgE levels. Renal involvement may occur in some patients. Diagnosis is made by histology. A 21-year-old Caucasian man with no relevant medical history presented with a non-tender swelling of the left hemiface without other associated symptoms. Laboratory investigations revealed a leukocyte count with eosinophilia (2.29×10^9/L- 26.5%) and elevated total IgE and IgG4. He had no renal dysfunction. He underwent surgical resection of the lesion in the jugal, infraorbital, and left nasolabial regions, and the anatomopathological examination revealed the characteristics of Kimura\'s disease. Three months after surgery, an MRI showed an increase in the size of the mass, and he was started on corticosteroids. Six months after surgery, he presented with a slight increase in the size of the mass and was started on Ciclosporin, which allowed a progressive reduction in the dose of corticosteroid with evidence of a progressive reduction in swelling. Ciclosporin was stopped due to toxic serum levels, and he was started on mycophenolate mofetil. The dose was increased because of the increase in facial mass; on mycophenolate mofetil 2500mg/day, the patient remains stable. KD is a chronic disorder of unknown etiology that mainly affects young people in Southeast Asia and is rare in Western countries, as in the case of this young man. Studies have shown no significant differences in region or race, complications, multiplicity, laterality, anatomical distribution, maximum size, eosinophil count, or IgE levels between age groups. There is no consensus on the optimal treatment for KD; several treatments have been used, including surgery, systemic corticosteroids, immunosuppressants, and radiation. Due to the tendency to relapse and the rarity of the disease, there is no consensus on treatment options for relapse.
摘要：
Kimura病（KD）于1937年首次被描述。这是一种罕见的，良性,和影响皮下组织的慢性免疫介导的炎性疾病，唾液腺,和淋巴结。这种疾病在中年东南亚国家的第二至第三个十年中更为常见。木村病的病因尚不清楚；一些作者认为它与自身免疫或迟发型超敏反应有关。它通常表现为头颈部的单发无痛淋巴结或与周围嗜酸性粒细胞增多和IgE水平升高相关的全身性淋巴结病（67％-100％）。某些患者可能发生肾脏受累。通过组织学进行诊断。一名无相关病史的21岁白种人，表现为左半边面无触痛性肿胀，无其他相关症状。实验室检查显示白细胞计数伴嗜酸性粒细胞增多（2.29×10^9/L-26.5％），总IgE和IgG4升高。他没有肾功能不全。他接受了jugal病变的手术切除，轨道下，和左侧鼻唇沟区域，解剖病理学检查揭示了木村病的特征。手术三个月后,MRI显示肿块的大小增加，他开始服用皮质类固醇。手术后六个月,他表现出肿块的大小略有增加，并开始服用环孢素，这使得皮质类固醇的剂量逐渐减少，并有证据表明肿胀逐渐减少。由于毒性血清水平，停用了环孢素，他开始服用霉酚酸酯。由于面部质量增加，剂量增加；霉酚酸酯2500mg/天，患者保持稳定。KD是一种病因不明的慢性疾病，主要影响东南亚的年轻人，在西方国家很少见，就像这个年轻人一样。研究表明，地区或种族没有显着差异，并发症,多重性,偏侧性,解剖分布,最大尺寸,嗜酸性粒细胞计数,或年龄组之间的IgE水平。对于KD的最佳治疗尚无共识；已经使用了几种治疗方法，包括手术,全身性皮质类固醇,免疫抑制剂,和辐射。由于这种疾病的复发趋势和罕见性，对于复发的治疗方案没有达成共识.
