Kellner David, Temelkovska Tijana, Greeley Adela, Saito Ashley
University of California, Los Angeles Medical Center.
University of California, Los Angeles David Geffen School of Medicine.
Fed Pract. 2023 Jan;40(1):22-27. doi: 10.12788/fp.0347. Epub 2023 Jan 16.
Kikuchi-Fujimoto disease (KFD) is a rare cause of rapidly evolving tender cervical lymphadenopathy. It is often initially misdiagnosed and managed as infectious lymphadenitis. Although most cases of KFD are self-limited and improve with antipyretics and analgesics, some are more refractory and may require corticosteroids or hydroxychloroquine therapy.
A 27-year-old White man presented for evaluation of fevers and painful cervical lymphadenopathy. He was found to have KFD on excisional lymph node biopsy. His symptoms proved challenging to manage with corticosteroids but eventually improved with hydroxychloroquine monotherapy.
KFD diagnosis should be considered irrespective of geographic location, ethnicity, or patient sex. Hepatosplenomegaly is a relatively rare manifestation of KFD that can make it especially difficult to distinguish from lymphoproliferative disorder, such as lymphoma. Lymph node biopsy is the preferred diagnostic approach to achieve a timely and definitive diagnosis. Although usually self-limited, KFD has been associated with autoimmune conditions, including systemic lupus erythematosus. Securing the diagnosis of KFD is therefore crucial to ensuring patients are monitored appropriately for the development of associated autoimmune conditions.
菊池-藤本病(KFD)是导致颈部淋巴结迅速肿大伴压痛的罕见病因。该病起初常被误诊为感染性淋巴结炎并按此治疗。尽管大多数KFD病例为自限性,使用退烧药和镇痛药后可好转,但有些病例更难治疗,可能需要使用糖皮质激素或羟氯喹治疗。
一名27岁白人男性因发热和颈部淋巴结疼痛前来就诊。经切除性淋巴结活检,确诊为KFD。事实证明,使用糖皮质激素治疗其症状颇具挑战性,但最终采用羟氯喹单药治疗后症状有所改善。
无论地理位置、种族或患者性别如何,均应考虑KFD的诊断。肝脾肿大是KFD相对罕见的表现,这使得它尤其难以与淋巴瘤等淋巴增殖性疾病相区分。淋巴结活检是实现及时、明确诊断的首选诊断方法。尽管KFD通常为自限性,但它与自身免疫性疾病有关,包括系统性红斑狼疮。因此,明确KFD的诊断对于确保对患者进行适当监测以预防相关自身免疫性疾病的发生至关重要。