系统性红斑狼疮合并胃肠道表现

Systemic Lupus Erythematosus with Gastrointestinal Manifestations

摘要: 系统性红斑狼疮(Systemic Lupus Erythematosus, SLE)是一种以抗体和免疫复合物形成，并介导器官组织损伤的自身免疫性疾病，临床上常存在多系统受累表现。患者血清中存在大量抗体，免疫复合物沉积在小血管，引起血管炎的病理损害导致多器官功能受损。其发病机制不十分清楚，目前发现感染、免疫功能紊乱、遗传和环境等多因素共同在发病机制中起重要作用。其典型特征是体内产生多种致病性自身抗体，形成大量自身免疫复合物(IC)，经循环沉积于各个组织、器官中，继而出现皮肤、心脏、肺、肾脏、血液系统和中枢神经系统等多个器官和系统损伤。SLE患者的临床表现及严重程度各不相同，其并发症是导致死亡的重要原因之一，如狼疮性肾炎(LN)、神经精神狼疮(NPSLE)、心血管疾病、血栓事件等。目前，治疗系统性红斑狼疮的基石药物为激素(GC)和免疫抑制剂，仍有部分患者疗效不佳、病情反复，且长期使用上述药物可能导致一些严重的副作用，如感染增加、骨质疏松、肝肾损害、类固醇性糖尿病等，因此SLE的治疗仍旧具有挑战性。蛋白丢失性肠病(Protein-loss enteropathy, PLE)为循环免疫复合物沉积或肠系膜、肠血管的血管炎导致的肠道毛细血管通透性增加，由于胃肠道蛋白丢失导致全身水肿及严重低蛋白血症的一种病理状态，表现为腹胀，腹泻与肾脏蛋白丢失不成比例的严重低蛋白血症、伴有水肿、胸腹水。SLE合并肠道病变患者临床症状多样且不典型，特别是以肠道症状首发时鉴别困难，本文为我院收治的1例系统性红斑狼疮导致的蛋白丢失性肠病的报告如下。

Abstract: Systemic Lupus Erythematosus (SLE) is an autoimmune disease that induces organ and tissue damage due to the formation of antibody and immune complex. A large number of antibodies are present in the serum of patients, and immune complexes are deposited in small blood vessels, causing pathological damage of vasculitis and impaired function of multiple organs. The pathogene-sis of the disease is not very clear, but many factors such as infection, immune dysfunction, genetics and environment have been found to play an important role in the pathogenesis. Its typical charac-teristics are the generation of a variety of pathogenic autoantibodies in the body, the formation of a large number of autoimmune complexes (IC), circulation deposition in various tissues and organs, and then skin, heart, lung, kidney, blood system and central nervous system and other organs and systems damage. The clinical manifestations and severity of SLE patients vary, and its complications are one of the important causes of death, such as lupus nephritis (LN), neuropsychiatric lupus (NPSLE), cardiovascular disease, thrombotic events, etc. At present, the cornerstone drug for the treatment of systemic lupus erythematosus (SLE) is hormone (GC) and immune inhibitor, there are still some poor efficacy and patient again and again, and the long-term use of these drugs can lead to some serious side effects, such as infection, osteoporosis, kidney damage, steroid diabetes, etc., so the treatment of SLE remains challenging. Protein-loss enteropathy (PLE) is a pathological condition in which intestinal capillary permeability is increased due to deposition of circulating immune complexes or vasculitis of mesentery and intestinal vessels. The loss of gastrointestinal protein leads to systemic edema and severe hypoproteinemia, manifested as abdominal distention, diar-rhea, severe hypoproteinemia, with edema and pleural effusion, disproportionate to renal protein loss. The clinical symptoms of SLE patients with intestinal lesions are varied and atypical, especially when intestinal symptoms are first identified with difficulty. In this paper, a case of protein-loss en-teropathy caused by systemic lupus erythematosus admitted to our hospital is reported as follows.