鼹鼠儿科第一线

Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS.

菊池藤本病 （KFD） 通常是一种良性的自限性炎症性疾病。然而，一些患者可能有长期或复发的病程，或出现危及生命的并发症，例如巨噬细胞活化综合征 （MAS）。在这项研究中，我们旨在描述MAS在KFD中的发病率和临床特征，并获得诊断KFD相关MAS的潜在实验室标志物。

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is characterized by fever and cervical lymphadenopathy of unknown etiology. Systemic clinical manifestations may include night sweats, weight loss, fatigue, generalized lymphadenopathy, headaches, arthralgia, sore throat, rash, nausea, vomiting, and even neurological involvement. As the symptoms and laboratory findings are non-specific, differential diagnoses include infectious lymphadenitis, malignancies such as lymphoma, and autoimmune diseases such as systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA). Histopathological diagnosis by a lymph node biopsy is crucial to differentiate KFD from other etiologies.

KFD is typically a benign, self-limiting inflammatory process that resolves with supportive measures within months without any specific treatment. However, some patients may have a prolonged or recurrent disease course, or present with severe complications such as hemophagocytic Lymphohistiocytosis(HLH) . HLH is a life-threatening syndrome of cytokine storm, characterized by hemophagocytosis, intractable fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, and hypofibrinogenemia. Serum proinflammatory cytokines, such as interferon-γ(or CXCL9 and CXCL10, chemokines induced by interferon-γ), interleukin-6, interleukin-10, and soluble interleukin-2 receptor alpha, a marker of T-cell activation, are usually excessively produced. Secondary HLH occurs in the setting of a malignant, infectious or autoimmune stimulus. Macrophage activation syndrome (MAS) is the term used to describe HLH that develops secondary to rheumatological diseases such as lupus and juvenile idiopathic arthritis, among others . The presence of MAS in adult KFD patients may be associated with adverse clinical outcomes including higher steroid usage and worse hospitalization outcomes, including a much higher rate of intensive care unit admission or in-hospital mortality . The incidence and clinical features of MAS in KFD are not well understood in children. The potential markers for the diagnosis of MAS in children with KFD are still not well clarified.

菊池藤本病（KFD），也称为组织细胞坏死性淋巴结炎，其特征是发热和病因不明的颈部淋巴结肿大。全身临床表现可能包括盗汗、体重减轻、乏力、全身淋巴结肿大、头痛、关节痛、咽痛、皮疹、恶心、呕吐，甚至神经系统受累[1]。由于症状和实验室检查结果是非特异性的，鉴别诊断包括感染性淋巴结炎、恶性肿瘤（如淋巴瘤）和自身免疫性疾病（如系统性红斑狼疮 （SLE） 和幼年特发性关节炎 （JIA）。通过淋巴结活检进行组织病理学诊断对于鉴别 KFD 与其他病因至关重要。

KFD通常是一种良性的自限性炎症过程，在几个月内通过支持性措施消退，无需任何特异性治疗。然而，部分患者的病程可能延长或复发，或出现严重并发症，如噬血细胞性淋巴组织细胞增多症。HLH 是一种危及生命的细胞因子风暴综合征，其特征是噬血细胞增多、顽固性发热、肝脾肿大、血细胞减少、高甘油三酯血症和低纤维蛋白原血症。血清促炎细胞因子，如干扰素-γ（或CXCL9和CXCL10，干扰素-γ诱导的趋化因子）、白细胞介素-6、白细胞介素-10和可溶性白细胞介素-2受体α（T细胞活化的标志物）通常过量产生。继发性 HLH 发生在恶性、感染性或自身免疫性刺激的情况下。巨噬细胞活化综合征（MAS）是用于描述继发于风湿性疾病（如狼疮和幼年特发性关节炎等）的HLH的术语。成人KFD患者存在MAS可能与不良临床结局有关，包括类固醇使用率较高和住院结局较差，包括ICU收治率或院内死亡率更高。