title: Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome: A Case-Control, Single Center Study.
abstract: 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.
authors.affiliation: From the Department of Rheumatology and Clinical Immunology (LZ, XW, JL, HC, YZ, WZ), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing; and Department of Rheumatology (LW), Huaihe Hospital of Henan University, Henan, China.
authors.forename: Lili
authors.initials: L
authors.lastname: Zhang
authors.forename: Xiuhua
authors.initials: X
authors.lastname: Wu
authors.forename: Laifang
authors.initials: L
authors.lastname: Wang
authors.forename: Jing
authors.initials: J
authors.lastname: Li
authors.forename: Hua
authors.initials: H
authors.lastname: Chen
authors.forename: Yan
authors.initials: Y
authors.lastname: Zhao
authors.forename: Wenjie
authors.initials: W
authors.lastname: Zheng
mesh_terms: D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000276:immunology / Q000503:physiopathology; D016022:Case-Control Studies; D002681:China / Q000453:epidemiology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015166:Monitoring, Immunologic / Q000379:methods; D011379:Prognosis; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000276:immunology / Q000503:physiopathology
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents