Abstract:
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.
摘要:
Kikuchi-Fujimoto病(KFD)是一种良性疾病,病因不明的自限性组织细胞坏死性淋巴结炎全身性疾病。KFD已经有半个世纪的历史了,但区分它的困难仍然存在。其诊断意义与不同时间范围内KFD合并自身免疫性疾病的患病率增加有关。系统性红斑狼疮(SLE)是与KFD同时出现的最常见的自身免疫性结缔组织疾病(AICTD)。一名18岁女性出现急性肌无力,呼吸急促,发烧,入院前5个月体重明显下降。疼痛和晨关节僵硬已经感觉到了9个月。一年前,她的右颈部结块,通过切除活检和免疫组织化学染色(CD68)诊断为KFD.肌酸激酶和C反应蛋白升高,具有较高的抗Ku和抗Jo-1阴性水平。补充水平很低,抗核抗体效价高,具有正的抗SS-A。Sialometry和Schirmer试验显示唾液和泪腺产生减少。我们诊断该患者患有KFD之前的重叠综合征。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,关于其他自身免疫性疾病的可能性,应注意其发生。KFD通常与AICTD一致,尽管它也可能在之前或之后发生。据报道,该病例可提高对KFD之前重叠综合征的认识。