组织细胞坏死性淋巴结炎合并系统性红斑狼疮1例分析并文献复习

Analysis of a case of histiocytic necrotizing lymphadenitis associated with systemic lupus erythematosus and review of the literature

目的：提高临床医生对组织细胞坏死性淋巴结炎（HNL）合并系统性红斑狼疮（SLE）的认识.方法：报告1例组织细胞坏死性淋巴结炎合并系统性红斑狼疮并复习相关文献.结果：患者为35岁女性,以发热、淋巴结肿大、关节炎、白细胞减少、贫血、脾大为主要临床表现,血沉、C反应蛋白升高,自身抗体检测：抗核抗体、抗SSA抗体及抗心磷脂抗体阳性,补体C3、C4下降,淋巴结活检示组织细胞坏死性淋巴结炎.最后诊断为组织细胞坏死性淋巴结炎合并系统性红斑狼疮.给予糖皮质激素及对症治疗,随访病情稳定.结论：HNL临床突出表现是发热、淋巴结肿大、疼痛,合并SLE临床少见,与SLE本身引起的淋巴结病变难以鉴别,病理检测有利于疾病的诊断,临床医师应引起注意并长期随访.

Objective To improve clinician＇s understanding of histiocytic necrotizing lymphsdenitis（HNL） associated with systemic lupus erythematosus（SLE）. Methods A case of necrotizing lymphadenitis complicated by systemic lupus erythematosus was reported and the related literatures were re- viewed. Results A 35--year--old famale presented with a Z--week history of fever associated with b/lateral cervical and axillary lymphadenopathy, in- flammatory polyarthritis, splenauxe. Laboratory data showed antinuclear antibodies（ANA） at titer of 1：640 dilution and positive tests for anti--SS-A （ELISA） and anti-cardiolipin, leucopenia （2100/ml）, anemia （hemoglobin 98g/1）, raised ESR （112mm/h）, and increased serum C-reactive protein （CRP） level （72. 1mg/dl）. Serum C3 and CA were descended. A diagnosis of SLE was consequently established. Lymph node histological analysis was compatible with a diagnosis of HNL. Treatment with prednisolone 30 mg/day was started. The patient became symptom free after few days. Conclusion HNL or Kikuchi--Fujimoto＇s disease（KFD） is a rare, benign condition of unknown etiology, which is usually characterized by lymphadenopatby and fever. This disease is rarely associated with SLE. It＇s very difficult to identify with lupus lymphadenopathy, and histology of nodes is conducive to diagnosis of HNL. Clinician should pay attention to it and encourage long--term follow--up in patients with this condition.