Department of Health Care, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Department of General Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1, Shuaifuyuan, Wangfujing St., Beijing, 100730, China.
Clin Rheumatol. 2019 Dec;38(12):3661-3667. doi: 10.1007/s10067-019-04752-6. Epub 2019 Aug 22.
Kimura disease (KD) is a rare idiopathic inflammatory disorder of unknown etiology. Unusual presentations of KD might cause diagnostic difficulty or be misdiagnosed as malignancy if clinical suspicion is insufficiently high. Here, we aimed to determine the clinicopathological features of Chinese KD patients to reveal further insights into the natural history and treatment of this disease.
The clinical data of 46 cases of KD diagnosed at Peking Union Medical College Hospital from January 1980 to December 2018 were analyzed retrospectively through case record review.
Of 46 cases, 40 were male and six were female. The age at onset ranged from 2 to 56 years (median 27 years). All patients presented with either single (26.1%) or multi-focal (73.9%) subcutaneous masses. Twenty-nine (63.0%) cases presented with head and neck subcutaneous masses, and 9 cases (19.6%) involved different parts of the body. Parotid, submandibular, and lacrimal gland involvement occurred in 17 (37.0%), 3 (6.5%), and 2 cases (4.3%), respectively. Nephrotic syndrome was present in three cases (6.5%), and thromboembolism was present in five cases (10.9%). During follow-up, thirteen patients (13/28, 46.4%) relapsed over 1-13 years (median 8.5 years). The recurrence rate in patients receiving corticosteroids, surgery, and combined surgery and radiotherapy was 30.8%, 66.7%, and 50.0%, respectively. One patient was diagnosed with T cell lymphoma 1 year after diagnosis of KD.
KD is characterized by subcutaneous masses but it is also a systemic disease. Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.Key Points• Kimura disease (KD) is a rare inflammatory disorder of unknown etiology that is endemic in Asia.• Clinicians must regard and manage KD as a systemic disease.• There is no consensus on optimal treatments and further studies are necessary to improve outcomes.• Given the high rate of recurrence and reported association with lymphoma, patients require careful long-term follow-up.
木村病（KD）是一种病因不明的罕见特发性炎症性疾病。如果临床怀疑不足，KD 的不典型表现可能导致诊断困难或误诊为恶性肿瘤。在此，我们旨在确定中国 KD 患者的临床病理特征，以进一步了解该疾病的自然病程和治疗方法。
通过病历回顾，分析了 1980 年 1 月至 2018 年 12 月期间在北京协和医院诊断的 46 例 KD 患者的临床资料。
46 例患者中，男性 40 例，女性 6 例。发病年龄 256 岁（中位年龄 27 岁）。所有患者均表现为单发（26.1%）或多发（73.9%）皮下肿块。29 例（63.0%）患者表现为头颈部皮下肿块，9 例（19.6%）累及身体不同部位。17 例（37.0%）患者腮腺、颌下腺和泪腺受累，分别有 3 例（6.5%）和 2 例（4.3%）患者出现肾病综合征和血栓栓塞。随访期间，13 例（13/28，46.4%）患者在 113 年内复发（中位时间 8.5 年）。接受皮质类固醇、手术和手术联合放疗的患者复发率分别为 30.8%、66.7%和 50.0%。1 例患者在 KD 诊断后 1 年被诊断为 T 细胞淋巴瘤。
KD 的特征是皮下肿块，但也是一种全身性疾病。鉴于高复发率和报告的与淋巴瘤的关联，患者需要仔细的长期随访。
木村病（KD）是一种病因不明的罕见炎症性疾病，在亚洲流行。
临床医生必须将 KD 视为一种全身性疾病并加以处理。
对于最佳治疗方法尚无共识，需要进一步研究以改善预后。
鉴于高复发率和报告的与淋巴瘤的关联，患者需要仔细的长期随访。
