系统性红斑狼疮继发视神经脊髓炎谱系疾病1例并文献回顾

<正>病例女,63岁,因“突发腰部麻木不适感伴双下肢麻木1月,加重伴左下肢活动不灵2天”就诊。神经科查体：左下肢肌力及肌张力减弱,左侧膝反射增强,左侧Babinski征可疑阳性。既往史：白细胞减低病史10余年。实验室检查：(1)血常规、血液生化检查：白细胞2.64×109L-1,血红蛋白100 g/L,淋巴细胞0.73×109L-1,余均为阴性;(2)风湿免疫指标：ANA 1∶3 200+阳性、SM抗体弱阳性、ds-DNA抗体357.08 IU/mL、抗β2糖蛋白1Ig G抗体118.6 CU;     

视神经脊髓炎合并系统性红斑狼疮及干燥综合征1例报告

<正>神经精神性狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE)是系统性红斑狼疮(systemic lupus erythematosus,SLE)常见的并发症之一,有报道称其发生率高达14%~81%[1],并可作为SLE的首发症状。典型的SLE中枢神经系统受累包括癫痫、抑郁、多发性单神经炎,但也可出现横贯性脊髓炎、脱髓鞘或颅神经受累等少见临床表现。以中枢神经系统损害为首发症状的SLE容易被误诊、延误治     

视神经脊髓炎一例误诊并文献复习

1病例资料 女,49岁.因右侧肢体麻木、无力1个月,进行性加重伴四肢活动受限1周入院.患者曾在当地用中草药治疗,症状缓解不明显,近1周自觉症状明显加重,并出现右手、足阵发性痉挛性抽搐,无规律性,每次发作持续时间不等,伴双下肢无力、站立不稳、行走困难、胸腹部束带感,无头痛、头晕、视物旋转及复视,尿、便正常,为进一步检查到我院就诊.     

系统性红斑狼疮合并弥漫性肺泡出血1例并文献复习

弥漫性肺泡出血(diffuse alveolar hemorrage,DAH)是系统性红斑狼疮(systemic lupus erythematosus,SLE)的严重并发症之一,临床上少见,病死率高,并且临床表现不特异,常常漏诊误诊。其疗效和预后多取决于及时的诊治。现报道SLE并发DAH经激素冲击等疗法成功救治1例并做文献复习。1病历摘要患者,女,29岁。因发热、关节痛3个月,咳嗽伴痰中带血1个月,于2006年9月20日入院。查体:脉搏106次/min,呼吸23次/min,端坐呼吸,双下肺可闻及细小水泡音。血常规:白细胞(WBC)3.65×109/L,血红蛋白(Hb)80g/L,血小板(Plt)50×109/L。尿常规:蛋白( ),红…     

系统性红斑狼疮合并尿路梗阻2例及合并肠梗阻1例并文献复习

系统性红斑狼疮（systemic lupus erythematosus,SLE）是一种系统性自身免疫性疾病,可累及全身多种脏器或器官,临床表现复杂多样。但SLE合并尿路梗阻和（或）肠梗阻较少见,临床报道较少,易被人们忽视,造成误诊、漏诊。现报告我院近5年来收治的30例SLE患者中SLE合并尿路梗阻2例及合并肠梗阻1例,并复习相关文献,以提高临床医师对此两种并发症的认识。     

1例系统性红斑狼疮合并急性横贯性脊髓炎患者的护理

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种自身免疫性结缔组织病[1].由于体内有大量致病性自身抗体和免疫复合物,造成组织损伤,临床可以出现各个系统和脏器损害的症状.     

系统性红斑狼疮患者并白血病漏诊1例并文献复习

系统性红斑狼疮（systemic lupus eryhematosis，SLE）是一种系统性自身免疫性疾病。SLE患者细胞免疫功能低下，且因长期应用激素和（或）免疫抑制剂治疗，增加了肿瘤发生的几率。而SLE可累及全身各器官系统，临床表现为发热、关节疼痛、贫血、腹痛等多种症状，合并白血病的SLE患者早期往往缺乏典型症状，较难与活跃期SLE鉴别，易造成误诊和漏诊。现将我院收治的1例SLE并白血病漏诊患者的病例进行分析，以期望引起临床医师对同类病例的重视。     

1例系统性红斑狼疮合并急性横贯性脊髓炎患者的护理

系统性红斑狼疮(systemiclupuserythematosus,SLE)是一种自身免疫性结缔组织病[1]。由于体内有大量致病性自身抗体和免疫复合物,造成组织损伤,临床可以出现各个系统和脏器损害的症状。本病女性约占97%,多为育龄妇女。横贯性脊髓炎是指非特异性,局限于数个节段的脊髓炎症[2]。表     

系统性红斑狼疮合并急性胰腺炎1例并文献复习

患者,女,19岁。发现雷诺现象5年,关节疼痛2年,1个月前开始发热,20天前腹痛入院。5年前,患者在双手受凉或受到刺激后出现变白、变紫,再变红,未检查和治疗。2年前,患者开始出现双腕、近指、掌指、膝、踝等多个关节疼痛,同时有四肢近端肌痛,在当地诊断为类风湿性关节炎,给予中药治     

表现为超长节段横贯性脊髓炎的视神经脊髓炎谱系疾病1例并文献复习

目的：探讨表现为超长节段横贯性脊髓炎（u-LETM）的视神经脊髓炎谱系疾病（NMOSD）的临床特 点及预后。方法：报道我院1例表现为u-LETM的NMOSD并结合文献进行回顾性临床分析。结果：本例患 者以不伴视神经受累的u-LETM为主要表现,且血清抗甲状腺抗体阳性（ATAbs）的NMOSD,该患者首次发 病未出现视神经炎、极后区综合征等表现,病情进展迅速,预后差。搜索既往报道的表现为 u-LETM 的 NMOSD患者44例,大部分患者核心临床症状表现为横贯性脊髓炎（TM）,复发率高达78%。其中血清自身 免疫抗体中ATAbs阳性的患者共4例。结论：表现为u-LETM的NMOSD的患者往往短期复发率高。自身 免疫性抗体阳性的患者常提示病情较重,预后差。     

Aplastic anemia associated with systemic lupus erythematosus in children – case report and review of the literature

Systemic lupus erythematosus should be included in the differential diagnosis of every adolescent with pancytopenia. An accurate diagnosis with the appropriate therapy is vital and can cause lasting reversal of this condition.     

Immunotherapies application in active stage of systemic lupus erythematosus in pregnancy: A case report and review of literature

BACKGROUNDPregnancy in the setting of systemic lupus erythematosus can worsen the condition from the stable to active stage, with quality of life and fertility desire being particular concerns. Pregnancy in the active stage of systemic lupus erythematosus (ASLE), although rare and complicated to manage, can be treated favorably with immunotherapies ifs used properly. Here we report such a success case.CASE SUMMARYA 31-year-old primigravida patient, diagnosed with SLE seven years ago, was induced ASLE after a cold at 21 + weeks. The patient’s vital signs on presentation were normal. Her laboratory exam was remarkable for significant proteinuria, liver and renal dysfunction, and low C3 and C4 levels. Infectious work-up was negative. The patient was diagnosed with ASLE. She was given immunosuppressive agents (methylprednisolone, gamma globulin and azathioprine etc.) and plasma adsorption therapy, monitoring blood pressure every 8 h, fetal heart rate twice a day, and liver and renal function at least twice a week. Successful maternal and fetal outcomes are presented here.CONCLUSIONChild-bearing in ASLE has become more promising, even for this difficult case of ASLE with multiple organ damage. Thorough antepartum counseling, cautious maternal-fetal monitoring, and multi-organ function monitoring by multidisciplinary specialties are keys to favorable pregnancy outcomes.     

Acquired C1-inhibitor deficiency in a patient with systemic lupus erythematosus: a case report and review of the literature

The C1-inhibitor (C1-INH) is an important member of the serpin family which inhibits the first component of the human complement system and controls contact activation of the coagulation and kinin system. An acquired form of C1-INH deficiency was recognized and classified as type I, which is characterized by accelerated catabolism of C1-INH, whereas type II is defined by the presence of an autoantibody directed against the C1 inhibitor molecule. This study reports the case of a 32-year-old woman with systemic lupus erythematosus (SLE) who experienced recurrent angioedema because of an acquired C1-INH deficiency. The relevant literature is reviewed.     

贝利尤单抗联合环孢素治疗难治性系统性红斑狼疮继发免疫性血小板减少1例并文献复习

目的 探讨贝利尤单抗治疗难治性系统性红斑狼疮(systemic lupus erythematosus, SLE)合并顽固性血液系统损害的疗效。方法 回顾性分析贵州省人民医院收治的1例以顽固性血液系统损害为主要表现的SLE病例的诊治过程。结果 患者为青年女性,SLE病史1年余,因皮下瘀斑伴腹痛10天入院,住院后明确诊断SLE合并难治性血液系统损害,在环孢素等基础治疗上加用贝利尤单抗维持治疗后,患者病情明显改善,且未见不良反应。随访1年余,患者激素用量已减至最低维持量,无复发。结论 贝利尤单抗联合环孢素治疗为难治性SLE继发免疫性血小板减少的治疗提供了新选择,更多的临床应用证据有待长期的大样本病例研究观察、评估。     

抗染色质抗体检测在SLE诊断及病情发展中的意义

目的检测SLE患者血清中抗染色质抗体(anti-chromatin antibody,AchA)水平,评价其在SLE诊断和病情发展中的意义。方法选取SLE患者154例,疾病对照组155例(类风湿关节炎46例,干燥综合征49例,骨关节炎10例,系统性硬化症20例,肾病综合征30例),健康人对照107名。用ELISA检测AchA水平,并探讨其与SLE患者发病年龄、病程、疾病活动指数(SLEDAI)评分、其他自身抗体、补体、免疫球蛋白(IgG)、ESR、24 h尿蛋白、肾功能指标等的关系。结果 SLE患者血清中AchA的阳性率为63.6%(98/154),明显高于疾病对照组5.8%(9/155)及健康人对照组0%(P均0.01)。AchA诊断SLE的特异性为96.6%,敏感性为63.6%,阳性预测值和阴性预测值分别为91.6%和81.9%。SLE患者血清中AchA水平明显高于疾病对照组和健康人对照组(P均0.000 1)。与AchA阴性SLE患者比较,AchA阳性SLE患者SLEDAI评分、24 h尿蛋白水平、ESR升高,C3、C4、WBC降低,抗核小体抗体(AnuA)、抗dsDNA抗体、抗核糖体P蛋白抗体水平升高(P均0.05)。相关性分析显示,AchA水平与ESR(r=0.218,P=0.01)、C3(r=0.451,P0.000 1)、Alb(r=0.295,P0.000 1)、AnuA(r=0.349,P0.000 1)、抗dsDNA抗体(r=0.311,P0.000 1)、24 h尿蛋白(r=0.243,P=0.003)、SLEDAI评分(r=0.202,P=0.015)均具有相关性。结论 AchA对SLE的诊断具有较高的敏感性和特异性,并且与疾病活动及病情严重程度相关,与狼疮肾损害强相关,提示其在狼疮肾发病及病理过程中的重要作用。     

系统性红斑狼疮合并带状疱疹的护理

目的 总结系统性红斑狼疮（SLE）合并带状疱疹（HZ）的临床特点及护理方法。方法 给予36例SLE合并HZ患者改善全身血液循环，抗病毒治疗，止痛，调节免疫等，并根据SLE患者疱疹的部位、性质和伴随症状，分别给予换药及预防感染等护理干预。结果 36例患者均痊愈出院，病程在2—3周。结论 指导患者预防皮肤感染，密切观察疱疹特点并给予针对性的换药处理，及时处理皮疹侵犯头面部神经而出现头痛、面瘫、视力模糊等，加强心理护理及疾病相关知识教育，对于缩短病程、减少复发、提高治愈率起着重要的作用。     

Concurrent monoclonal gammopathy and systemic lupus erythematosus in a known case of ulcerative colitis: A case report

Our patient had previously been diagnosed with Ulcerative colitis. The clinical manifestations of the patient along with laboratory tests such as anti‐dsDNA and proteinuria were also positive. Therefore, the clinical manifestation was consistent with SLE. In the following work up, monoclonal gammopathy in serum electrophoresis was also detected.     

Systemic lupus erythematosus complicated by noncirrhotic portal hypertension: A case report and review of literature

A 48 year-old Chinese woman suffering from polyarthritis, irregular fever and trichomadesis was admitted to the hospital. A diagnosis of systemic lupus erythematosus (SLE) was made based on polyarthritis, pancytopenia, reduced complement 3, multiple positive autoantibodies, a positive Coomb’s test and protein in her urine. In addition, splenomegaly was detected during physical examination and confirmed by abdominal ultrasonography and magnetic resonance imaging, indicating that the patient had SLE and portal hypertension. Further negative investigations ruled out the possibility of cirrhosis. The patient was diagnosed with active SLE complicated by noncirrhotic portal hypertension (NCPH) without liver histopathology, due to the patient’s refusal for liver biopsy. Portal vein diameter and splenomegaly decreased following treatment with methylprednisolone, hydroxychloroquine and metoprolol tartrate. To date, SLE complicated by NCPH has rarely been reported, as it is under-recognized clinically as well as pathologically. Here we describe a case of SLE complicated by NCPH and review the literature for its characteristics, which may contribute to improving the recognition of NCPH and reducing missed and delayed diagnosis of this disorder.     

Refractory autoimmune hemolytic anemia in a systemic lupus erythematosus patient: A clinical case report

Warm autoimmune hemolytic anemia (AIHA) is a hematologic disorder with an incidence of 1–3 per 10⁵ individuals/year. Patients with systemic lupus erythematosus (SLE) develop AIHA in 3% of adult cases and 14% of pediatric cases. We report a case of AIHA refractory to multiple lines of treatment in a patient with SLE, who eventually responded to a proteasome inhibitor‐based combination. A patient with systemic lupus erythematous was diagnosed with symptomatic autoimmune hemolytic anemia. The patient was refractory to multiple lines of treatment including prednisone, intravenous immune globulin, methylprednisolone, rituximab, cyclophosphamide, mycophenolate mofetil, and splenectomy. She eventually had a beneficial response to a proteasome inhibitor‐based combination with bortezomib plus mycophenolate mofetil. The treatment of refractory autoimmune hemolytic anemia can be challenging. Patients with AIHA refractory to primary or secondary treatments must resort to receiving novel therapeutic modalities including combinations targeting plasma cell, T‐ and B‐cell proliferation.     

以红斑肢痛为首发表现的系统性红斑狼疮1例

患者,女,44岁。双足疼痛2年,伴颜面浮肿半年,加重1天。患者于2年前无明显诱因出现双足疼痛,以双足底疼痛,伴烧灼感,疼痛以夜间为甚,疼痛时轻时重,严重时伴有血压升高,自觉遇冷时疼痛可缓解,但运动及保暖时疼痛加重,伴口干、视物模糊,无晨僵,无畏寒发热,无夜间盗汗、午后低热,无口腔溃疡、蝶形红斑、颜面部皮疹,