The clinical characteristics of connective tissue disease complicated with lymphatic duct obstruction
10.3760/cma.j.cn141217-20230712-00002
- VernacularTitle:结缔组织病合并淋巴管梗阻临床特征分析
- Author:
Lingling ZHANG
1
;
Lan GAO
;
Guohua ZHANG
;
Junli LUO
;
Jianfeng XIN
;
Wenbin SHEN
;
Yuhua WANG
Author Information
1. 首都医科大学附属北京世纪坛医院风湿免疫科，北京 100038
- Keywords:
Connective tissue disease;
Lymphatic duct;
Obstruction
- From:
Chinese Journal of Rheumatology
2024;28(9):631-639
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics and treatment strategies of patients with connective tissue disease (CTD) related lymphatic duct obstruction.Methods:The 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. Results:Forty-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), χ2=7.63, P=0.006], but the prevalence of lupus nephritis [(60%(12/21) vs. 86%(36/42), χ2=4.87, P=0.027] and lupus encephalopathy [0%(0/21) vs. 16.7%(17/42), χ2=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. Conclusion:CTD 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.
