Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome A Case-Control, Single Center Study

被引:11
作者
Zhang, Lili [1 ,2 ]
Wu, Xiuhua [1 ,2 ]
Wang, Laifang [3 ]
Li, Jing [1 ,2 ]
Chen, Hua [1 ,2 ]
Zhao, Yan [1 ,2 ]
Zheng, Wenjie [1 ,2 ]
机构
[1] Chinese Acad Med Sci, Dept Rheumatol & Clin Immunol, Peking Union Med Coll Hosp, Beijing 100730, Peoples R China
[2] Peking Union Med Coll, Beijing 100021, Peoples R China
[3] Henan Univ, Huaihe Hosp, Dept Rheumatol, Kaifeng, Henan, Peoples R China
基金
北京市自然科学基金; 中国国家自然科学基金;
关键词
CLASSIFICATION CRITERIA; THROMBOCYTOPENIC PURPURA; HEMOLYTIC-ANEMIA; T-CELLS; DERIVATION; RITUXIMAB; CYTOKINES; DISEASE;
D O I
10.1097/MD.0000000000003279
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
The aim of the study was to investigate the clinical features of systemic lupus erythematous (SLE) complicated with Evans syndrome (ES). We conducted a retrospective case-control study to compare the clinical and laboratory features of age-and gender-matched lupus patients with and without ES in 1: 3 ratios. In 5724 hospitalized SLE patients, we identified 27 (0.47%, 22 women and 5 men, average age 34.2 years) SLE patients complicated with ES. Fifteen patients (55.6%) presented with hematologic abnormalities initially, including 6 (22.2%) cases of isolated ITP, 4 (14.8%) cases of isolated AIHA, and 5 (18.5%) cases of classical ES. The median intervals between hematological presentations the diagnosis of SLE was 36 months (range 0-252). ES developed after the SLE diagnosis in 4 patients (14.8%), and concomitantly with SLE diagnosis in 8 patients (29.6%). Systemic involvements are frequently observed in SLE patients with ES, including fever (55.6%), serositis (51.9%), hair loss (40.7%), lupus nephritis (37%), Raynaud phenomenon (33.3%), neuropsychiatric (33.3%) and pulmonary involvement (25.9%), and photosensitivity (25.9%). The incidence of photosensitivity, hypocomplementemia, elevated serum IgG level, and lupus nephritis in patients with ES or without ES was 25.9% vs 6.2% (P = 0.007), 88.9% vs 67.1% (P = 0.029), 48.1% vs 24.4% (P = 0.021), and 37% vs 64.2% (P = 0.013), respectively. Twenty-five (92.6%) patients achieved improvement following treatment of glucocorticoids and immunosuppressants as well as splenectomy, whereas 6 patients experienced the relapse and 1 patient died from renal failure during the follow-up. ES is a relatively rare complication of SLE. Photosensitivity, hypocomplementemia, and elevated serum IgG level were frequently observed in ES patients, but lupus nephritis was less observed. More than half of patients presented with hematological manifestation at onset, and progress to typical lupus over months to years. Therefore, monitoring with antoantibodies profile as well as nonhematological presentations are necessary for patients with ITP and (or) AIHA.
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页数:5
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