Fellow of Rheumatology, Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine Universitas Indonesia - Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia..
Acta Med Indones. 2024 Apr;56(2):210-217.
Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting histiocytic necrotizing lymphadenitis systemic disorder with unknown etiology. KFD has been known for half a century, but difficulties in distinguishing it remain. Its diagnostic significance is related to the increasing prevalence of KFD with autoimmune diseases in various timeframes. Systemic lupus erythematosus (SLE) is the most prevalent autoimmune connective tissue disease (AICTD) appearing alongside KFD. An 18-year-old female presented with acute muscle weakness, shortness of breath, fever, and significant weight loss for 5 months before admission. Pain and morning joint stiffness had been felt for 9 months. One year ago, she lumped her right neck and was diagnosed with KFD from the excision biopsy and immunohistochemical staining (CD68). Creatine-kinase enzymes and C-Reactive protein were elevated with a high anti-Ku and anti-Jo-1 negative level. There was a low level of complements, high anti-nuclear antibody titer, with positive anti-SS-A. Sialometry and Schirmer test showed reduced salivary and lacrimal gland production. We diagnosed this patient as having an overlap syndrome preceded by KFD. The AICTD involved was Sjögren's syndrome and SLE. Although KFD is considered a self-limiting disease, its occurrence should be noticed regarding the possibility of other autoimmune conditions. KFD usually coincides with AICTD, although it could also precede or occur afterward. This case is reported to raise awareness of the overlap syndrome preceded by KFD.
菊池古氏病（KFD）是一种良性、自限性组织细胞坏死性淋巴结炎全身性疾病，病因不明。KFD 已有半个世纪的历史，但鉴别仍存在困难。其诊断意义与 KFD 在不同时间范围内与自身免疫性疾病的发病率增加有关。系统性红斑狼疮（SLE）是最常见的自身免疫性结缔组织病（AICTD），与 KFD 同时出现。一名 18 岁女性因急性肌无力、呼吸急促、发热和显著体重减轻 5 个月而入院。9 个月前出现疼痛和晨僵。一年前，她发现自己的右颈有肿块，并通过切除活检和免疫组织化学染色（CD68）被诊断为 KFD。肌酸激酶和 C 反应蛋白升高，抗 Ku 和抗 Jo-1 阴性水平高。补体水平低，抗核抗体滴度高，抗 SS-A 阳性。唾液量和泪液分泌试验显示唾液和泪液分泌减少。我们诊断该患者为先发性 KFD 的重叠综合征。涉及的 AICTD 是干燥综合征和 SLE。尽管 KFD 被认为是一种自限性疾病，但应注意其发生其他自身免疫性疾病的可能性。KFD 通常与 AICTD 同时发生，但也可能先于或后于 AICTD 发生。本病例报告旨在提高对 KFD 先发性重叠综合征的认识。
