摘要
目的总结结缔组织病(CTD)合并淋巴管受累患者的临床特征及治疗经验。方法回顾性收集2008年1月至2020年12月间,首都医科大学附属北京世纪坛医院住院患者中CTD合并淋巴管梗阻患者的临床资料、实验室检查、影像学资料、治疗及转归等数据。将本研究中样本量较多的SLE和RA患者,按照性别、年龄1∶2匹配,随机选取同时期入院的不合并有淋巴管回流障碍的SLE、RA患者作为对照组。2组之间进行比较时连续变量采用t检验或Wilcoxon秩和检验,分类变量采用χ^(2)检验或Fisher确切概率法。结果共有44例CTD合并淋巴管梗阻患者,男性7例,女性37例;其中类风湿关节炎(RA)14例,系统性红斑狼疮(SLE)21例,原发性干燥综合征(pSS)8例,系统性硬化病(SSc)1例。CTD发病年龄14~68岁,平均(37±15)岁;CTD病程1~480个月,中位病程66个月。出现淋巴管梗阻症状如肢体水肿或者胸腹腔积液的平均年龄(42±17)岁,有淋巴管梗阻症状病程3~480个月,中位病程12个月。59%患者(26/44)先诊断CTD后出现淋巴管梗阻并发症,41%患者(18/44)以淋巴管梗阻症状为CTD首发表现。44例患者均行淋巴管相关影像学检查,分别表现为胸导管梗阻(64%,28/44),胸导管畸形或者变异(36%,16/44),肢体淋巴回流障碍(34%,15/44),小肠淋巴管扩张或肠蛋白质丢失(18%,8/44)。RA患者主要表现为肢体肿胀(12/14),与无淋巴管受累的对照组相比,有淋巴管受累患者发病年龄较低[(34±14)岁和(44±13)岁,t=-2.15,P=0.037],有更长的RA病程[(17±11)个月和(7±7)个月,t=3.38,P=0.002];而SLE患者则表现为乳糜性多浆膜腔积液(20/21)。与对照组相比SLE合并淋巴管梗阻患者,出现狼疮肾炎[60%(12/21)和86%(36/42),χ^(2)=4.87,P=0.027]和狼疮脑病[0(0/21)和16.7%(7/42),χ^(2)=6.11,P=0.013]的比例较低,但更易出现多浆膜腔积液[95%(20/21)和62%(25/42),χ^(2)=7.63,P=0.006)。27%(12/44)患者经积极激素联合免疫抑制剂治疗后好转,54%(24/44)的患者需要在内科治疗的基础上行淋巴管重建手术,5例失访,死亡2例。结论CTD可累及淋巴管导致淋巴管梗阻,也可以淋巴管梗阻为CTD的首发症状,应引起风湿科医生的重视。育龄期女性出现难治性多浆膜腔积液或者淋巴水肿应警惕是否合并CTD。淋巴管梗阻可能与CTD长期慢性炎症相关,激素和免疫抑制剂联合外科手术是治疗CTD淋巴管受累策略之一。
ObjectiveTo investigate the clinical characteristics and treatment strategies of patients with connective tissue disease(CTD)related lymphatic duct obstruction.MethodsThe clinical data,laboratory tests results,imaging data,and treatment of CTD patients associated with lymphatic vessel obstruction were retrospectively collected from January 2008 to December 2020 at Beijing Shijitan Hospital.Lymphatic duct obstruction was confirmed by thoracic duct ultrasound or thoracic duct MRI or lymphoscintigraphy or direct lymphangiography.SLE and RA patients were matched with gender and age in a 1∶2 ratio,and SLE and RA patients without lymphatic reflux disorder admitted at the same time were randomly selected as the control group.When comparing the data between the two groups,t-test or rank sum test was used to test continuous variables,and chi-square test or Fisher′s exact probability method was used to test categorical variables.ResultsForty-four patients with CTD complicated with thoracic duct obstruction were included,with a male-to-female ratio of 7∶37,including 14 cases of rheumatoid arthritis(RA),21 cases of systemic lupus erythematosus(SLE),8 cases of primary Sjogren's syndrome(pSS),and 1 case of systemic sclerosis(SSc).The onset age of CTD ranged from 14 to 68 years,the mean age was(37±15)years and the median duration of CTD was 66(range 1~480)months.The median age at the onset of lymphatic duct obstruction such as limb edema or thoracoabdominal effusion was(42±17)years,and the median duration of lymphatic duct obstruction symptoms was 12(range 3~480)months.59%(26/44)of patients were diagnosed with CTD followed by the diagnosis of thoracic duct obstruction,and 41%(18/44)of patients had lymphatic duct obstruction symptoms as the initial presentation of CTD.Thoracic duct-related imaging was performed in 44 patients and showed thoracic duct obstruction(64%,28/44),thoracic duct malformation or variation(36%,16/44),limb lymphatic reflux disorder(34%,15/44),and small bowel lymphatic duct dilatation or intestinal protein loss(18%,8/44),respectively.Compared with the control group,among these patients,patients with RA complicated with lymphatic involvement had a younger onset age[(34±14)years old vs.(44±13)years old,t=-2.15,P=0.037)]and longer RA course[(17±11)months vs.(7±7)months,t=3.38,P=0.002]and presented with limb swelling(12/14).While compared with the control group,SLE patients complicated with lymphatic duct obstruction presented with celiac multi-plasmatic effusion(20/21),more patients presented with multiple serous cavity effusion[95%(20/21)vs.62%(25/42),χ^(2)=7.63,P=0.006],but the prevalence of lupus nephritis[(60%(12/21)vs.86%(36/42),χ^(2)=4.87,P=0.027]and lupus encephalopathy[0%(0/21)vs.16.7%(17/42),χ^(2)=6.11,P=0.013]was lower.27%(12/44)of patients improved with aggressive glucocorticoids combined with immunosuppressive therapy,54%(24/44)of patients were performed with lymphatic duct reconstruction surgery on top of medical treatment,5 patients were lost of follow-up,and 2 patients deceased.ConclusionCTD patients may develop lymphatic duct obstruction during the disease course,while lymphatic duct obstruction can also be the initial presentation of CTD.Rheumatologists and surgeons should be alert to this rare situation.Young women with refractory polyserositis or lymphedema should be examined for the possibility of combined CTD.Lymphatic duct obstruction may be associated with long-term chronic inflammation in CTD.Glucocorticoids combined with immunosuppressive agents and surgery can be used to treat lymphatic duct obstruction in patients with CTD.
作者
张令令
高兰
张国华
罗俊丽
信建峰
沈文彬
王玉华
Zhang Lingling;Gao Lan;Zhang Guohua;Luo Junli;Xin Jianfeng;Shen Wenbin;Wang Yuhua(Department of Rheumatology and Clinical Immunology,Beijing Shijitan Hospital,Capital Medical University,Beijing 100038,China;Department of Lymphatic Surgery,Beijing Shijitan Hospital,Capital Medical University,Beijing 100038,China)
出处
《中华风湿病学杂志》
CAS
CSCD
2024年第9期631-639,共9页
Chinese Journal of Rheumatology
基金
北京市教育委员会科技计划一般项目(KM201910025004)
北京市医院管理中心第五期"青苗"计划(QMS20190705)。
关键词
结缔组织疾病
淋巴管
梗阻
Connective tissue disease
Lymphatic duct
Obstruction
