合并还是模拟:当Castleman病与结缔组织病共存时

Overlap or mimic:Castleman disease and connective tissue disease

目的探讨Castleman病与CTD共存时的关系,减少误诊误治。方法回顾性分析2001年1月至2017年5月北京协和医院收治的疑诊CTD最终诊断Castleman病患者的临床及实验室资料。结果①Castleman病合并或模拟CTD 11例,占同期Castleman病患者的5.7%(11/194),发病平均年龄(51±17)岁,男性6例,女性5例;②常见的临床表现依次为多发浅表淋巴结肿大(10/11)、发热(8/11)、浆膜腔积液(6/11)、关节肿痛(5/11)、脱发(2/11)、雷诺现象(1/11)、光过敏(1/11);③实验室检查表现为ESR增快(11/11)、低白蛋白血症(11/11)、hs-CRP升高(10/11)、IgG升高(7/11)、蛋白尿(5/11)、镜下血尿(5/11)、ANA阳性(5/11);④疑诊的CTD包括SLE 5例,IgG4相关性疾病2例,成人Still病2例;合并SS 2例;⑤11例均由活检病理明确诊断为多中心型Castleman病,浆细胞型10例,混合型1例。结论Castleman病可出现发热、多浆膜腔积液、关节肿痛、蛋白尿等合并或模拟CTD的表现,诊断和治疗需谨慎,必要时行淋巴结或肿物活检,减少误诊误治。

Objective To investigate the relationship of Castleman disease(CD)and connective tissue disease(CTD).Methods Clinical records and laboratory data of 11 patientsdiagnosed with CD and CTD were collected and retrospectively analyzed.All patients were diagnosed at Peking Union Medical College Hospital.Results①The proportion of CD associated with or mimicking CTD was 5.67%(11/194)in all CD patients during the same period.The average age of these cases at the diagnosis was(51±17)years and the ratio of male to female was 6∶5.②Lymphadenopathy(10/11),fever(8/11),serosal effusion(6/11),arthralgia(5/11),alopecia(2/11),Raynaud phenomenon(1/11)and photosensitivity(1/11)were the common clinical manifest-ations that could mimic CTD.③Elevated ESR(11/11),hypoalbuminemia(11/11),elevated CRP(10/11),elevated IgG(7/11),proteinuria(5/11),hematuria(5/11)and positive ANA(5/11)were commonly found in the patients'laboratory tests.④CD was inclined to mimic systemic lupus erythematosus(SLE)(5/11),IgG4-related disease(IgG4-RD)(2/11)and adult onset Still's disease(AOSD)(2/11),as well as 2 cases were associated with Sj?gren's Syndrome(SS)(2/11).⑤All cases were ultimately diagnosed as multicentric CD,the pathologic subtypes were plasma cell variant(10/11)and mixed(1/11)respectively.Conclusion CD maybe overlapped with or mimic a variety of clinical manifestations,such as fever,serosal effusion,arthralgia and proteinuria which could mimic CTD.Early biopsy is helpful for the diagnosis and to avoid misdiagnosis and mistreatment.