Liao Yi-Xuan, Guo Yan-Fei, Wang Yu-Xia, Liu Ai-Hua, Zhang Chun-Li
Department of Pulmonary and Critical Care Medicine, Beijing Hospital, National Center of Gerontology; Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China.
Department of Rheumatology and Immunology, Beijing Hospital, National Center of Gerontology; Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing 100730, China.
World J Clin Cases. 2021 Mar 16;9(8):2008-2014. doi: 10.12998/wjcc.v9.i8.2008.
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by systemic involvement and multiple autoantibodies in the serum. Patients with protein C (PC) and protein S (PS) deficiency are prone to thrombosis. In contrast, patients with primary hyperfibrino-lysis tend to bleed.
A 52-year-old female patient with bilateral pleural effusion was diagnosed with "tuberculous pleurisy" and treated with anti-tuberculosis drugs and prednisone. The coagulation-related laboratory results showed decreased fibrinogen, PC activity, PS activity, and antithrombin Ш activity. The immune-related laboratory results showed positive antinuclear antibody, anti-Smith antibody, anticardiolipin antibody (ACL), anti-β2-glycoprotein I antibody (aβ2GPI) and direct Coomb's test and decreased complement 3 and complement 4. Thoracoscopy was performed and bloody pleural fluid was drained. Pathology of the pleural biopsy showed lymphocytes, plasma cells, and a few eosinophils in adipose and fibrous connective tissue. Results of whole exome sequencing of blood showed no genetic mutations suggesting the presence of hereditary hematological diseases. The patient was finally diagnosed with SLE and primary hyperfibrinolysis, and was treated with prednisolone, hydroxychloroquine, and compound cyclophosphamide.
PC and PS deficiency in SLE might be related to ACL and aβ2GPI. SLE and primary hyperfibrinolysis can coexist in one patient, with both a risk of thrombosis and a risk of bleeding.
系统性红斑狼疮（SLE）是一种自身免疫性疾病，其特征为全身受累及血清中多种自身抗体。蛋白C（PC）和蛋白S（PS）缺乏的患者易发生血栓形成。相反，原发性纤维蛋白溶解症患者倾向于出血。
一名52岁双侧胸腔积液的女性患者被诊断为“结核性胸膜炎”，并接受抗结核药物和泼尼松治疗。凝血相关实验室检查结果显示纤维蛋白原、PC活性、PS活性及抗凝血酶Ⅲ活性降低。免疫相关实验室检查结果显示抗核抗体、抗史密斯抗体、抗心磷脂抗体（ACL）、抗β2糖蛋白I抗体（aβ2GPI）阳性及直接抗人球蛋白试验阳性，补体3和补体4降低。进行了胸腔镜检查并引流出血性胸腔积液。胸膜活检病理显示脂肪和纤维结缔组织中有淋巴细胞、浆细胞及少量嗜酸性粒细胞。血液全外显子测序结果未显示提示遗传性血液病存在的基因突变。该患者最终被诊断为SLE和原发性纤维蛋白溶解症，并接受泼尼松龙、羟氯喹和复方环磷酰胺治疗。
SLE中PC和PS缺乏可能与ACL和aβ2GPI有关。SLE和原发性纤维蛋白溶解症可在同一患者中共存，既有血栓形成风险又有出血风险。
