干燥综合征继发肺轻链沉积病的临床病理特点回顾分析  

Retrospective clinicopathological analysis of pulmonary light chain deposition disease secondary to Sjögren′s syndrome

作　　者：杨子萱 张卉[1] 吴焕文[1] 梁智勇[1] 张煜涵 庞钧译 冯瑞娥[1] Yang Zixuan;Zhang Hui;Wu Huanwen;Liang Zhiyong;Zhang Yuhan;Pang Junyi;Feng Rui′e(Department of Pathology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China;Department of Pathology,the Central Hospital of Wuhan,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430014,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院病理科,北京100730 [2]华中科技大学同济医学院附属武汉市中心医院病理科,武汉430014

出　　处：《中华结核和呼吸杂志》2024年第6期535-541,共7页Chinese Journal of Tuberculosis and Respiratory Diseases

摘　　要：目的分析干燥综合征(Sjögren′s syndrome,SS)继发的肺轻链沉积病(pulmonary light chain deposition disease,PLCDD)患者的临床、影像及病理特征,提高疾病认识水平。方法回顾性分析2009年至2023年中国医学科学院北京协和医学院北京协和医院病理诊断的23例PLCDD患者临床资料,筛选出其中11例SS继发的PLCDD,患者中位年龄51岁,年龄范围36~74岁,10例为女性,1例为男性,总结本病的临床、影像及病理特征。结果11例高分辨率CT(HRCT)均表现为双肺多发结节,10例可见囊性病变,6例囊壁或囊内可见血管伴行。11例患者显微镜下均表现为肺组织内无定形均质粉染物质沉积,刚果红染色阴性,血管壁受累常见,且伴多少不等的淋巴、浆细胞浸润及多核巨细胞反应,6例伴有囊形成,1例伴肺淋巴瘤。11例均未发现肺外脏器受累。10例接受激素和(或)免疫抑制剂治疗,1例1年后更换为硼替佐米联合地塞米松(BD)方案,平均随访50个月,5例临床和(或)HRCT稳定,2例HRCT缓解,2例HRCT进展。结论SS继发的PLCDD多见于中年女性,常局限于肺;HRCT以双肺多发结节和薄壁囊为特征;病理表现为刚果红阴性的非淀粉样物质沉积及SS相关肺间质改变;总体预后较好,少数需警惕淋巴瘤。Objective To analyze of the clinical,imaging,and pathological features of pulmonary light chain deposition disease(PLCDD)secondary to Sjögren′s syndrome(SS),and to improve the understanding of the disease.Methods We retrospectively analyzed the clinical data of 23 PLCDD cases diagnosed by pathology in Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College from 2009 to 2023,a total of 11 cases of PLCDD secondary to SS(SS-PLCDD)were selected,the median age was 51 years old(range:36~74),10 female and 1 male,the clinical,imaging,and pathological features were summarized.Results All 11 cases showed bilateral multiple pulmonary nodules on HRCT with 10 cases accompanied by cysts,6 cases with vessels in the cystic wall or crossing the cysts.Microscopically,all 11 cases showed amorphous eosinophilic material with negative Congo red staining deposited in the lung,vascular involvement was common,with variable lymphocyte,plasma cell infiltration and multinucleated giant cell reaction,cysts formation was observed in 6 cases,1 case combined with pulmonary lymphoma.No extrapulmonary involvement in all 11 cases.Ten patients were treated with glucocorticoid and(or)immunosuppressants,1 patient changed to bortezomib-dexamethasone chemotherapy after 1 year,with a mean follow-up of 50 months,5 cases were stable on clinical and(or)HRCT findings,2 cases showed remission on HRCT,2 cases progressed on HRCT.Conclusions SS-PLCDD affects predominantly middle-aged females with lesions confined to the lung;HRCT showed bilateral multiple nodules and thin-walled cysts.Pulmonary pathology presented as non-amyloid material with negative Congo red staining and interstitial changes associated with SS.The overall prognosis was good,but caution is advised regarding the underlying lymphoma.

关 键 词：肺 轻链沉积病 干燥综合征 囊 

分 类 号：R563[医药卫生—呼吸系统]