Castleman病合并系统性红斑狼疮的临床特征分析

Clinical Features of Patients with Castleman's Disease Complicated by Systemic Lupus Erythematosus

目的总结Castleman病（CD）合并系统性红斑狼疮（SLE）患者的临床特点。方法根据北京协和医院病案室1994年至2014年整理的诊断信息,收集诊断符合CD病及SLE患者。以＂Castleman’s disease＂、＂Systemic lupus erythematosus＂、＂SLE＂、＂lupus＂为关键词在Pub Med上检索1994至2014年间文献,筛选有详细临床资料记录的文献,临床资料不完善的病例被排除在本研究之外。结果共纳入9例患者,其中北京协和医院2例,占该院同期确诊SLE患者的0.03%（2/6502）,同期确诊CD患者的1.0%（2/100）。9例患者中,男2例,女7例,确诊CD时中位年龄39.0岁（21~60岁）。所有患者均为多中心型CD,病理检查提示浆细胞型3例,透明血管型3例,混合型3例。患者最常见症状为发热（88.9%,8/9）,最常累及的系统为血液系统（88.9%,8/9）,最常累及的脏器为肾脏（88.9%,8/9）。55.6%（5/9）的患者存在自身免疫性血小板减少,显著高于普通SLE患者的15.0%（P〈0.01）。所有患者均未出现中枢神经系统受累证据。结论 CD合并SLE是一种较为罕见的临床情况。与普通SLE患者相比,合并CD的SLE患者可能更易出现血小板减少,较少出现神经系统受累。

Objective To investigate the clinical features of patients with Castleman＇s disease（ CD）and systemic lupus erythematosus（ SLE）.Methods According to the diagnostic information between 1994 to2014 extracted from the database of the Medical Record Department of Peking Union Medical College Hospital（ PUMCH）,patients with CD and SLE were included.A thorough literature review utilizing the key words of＂Castleman＇s disease＂, ＂systemic lupus erythematosus＂, ＂SLE＂,and ＂lupus＂was performed in Pub Med during the same period.Cases with detailed clinical information were included while cases without detailed information were excluded from the analysis of this study.Results Nine patients worldwide were available for analysis [2 cases from PUMCH,accounted for 0.03%（ 2/6502） of all patients diagnosed as SLE and 1.0%（ 2/100） of patients diagnosed as CD during the same period]with a male-to-female ratio of 2∶7.The median age atdiagnosis of CD was 39.0 years（ range：21-60 years）.All patients were diagnosed as multicentric CD with generalized peripheral lymphadenopathy.Pathologic examination showed a balanced distribution：plasma cell variant：hyaline-vascular variant：mixed variant = 3∶3∶3.Fever was the most common symptom（ 88.9%,8/9）.Blood system was the most commonly involved system（ 88.9%,8/9） and kidneys were the most commonly involved organ（ 88.9%,8/9）.Autoimmune thrombocytopenia（ AITP） was observed in 55.6%（ 5/9） of patients,which was significantly higher than the general SLE patients（ 15.0%）（ P〈0.01）.None of the 9 patients had evidence of central nervous system involvement.Conclusions CD complicated by SLE is a rare clinical condition.Compared to the general SLE population,this subgroup of patients may have higher rate of AITP and lower rate of central nervous system involvement.