狼疮肾病终末期患者肾移植的临床分析

目的：对6例系统性红斑狼疮（SLE）终末期肾病患者的肾移植情况进行总结。方法：对6例终末期狼疮肾病肾移植患者的临床、实验室资料和手术后随访情况进行分析。结果：6例患者均为女性，平均病程4.4年，进行移植手术时全身病情稳定；术前均使用激素和/或免疫抑制剂控制SLE病情，手术后免疫抑制剂使用情况差异无显著性，术后平均随诊时间为24.2个月，无1例发生急、慢性排异反应及狼疮肾病复发，移植肾功能良好。结论：终末期狼疮肾病患者的移植肾存活及功能状况良好，无狼疮肾病复发，肾移植是治疗终末期狼疮肾病的有效方法之一。     

系统性红斑狼疮相关可逆性后部脑病综合征

目的:分析系统性红斑狼疮(SLE)合并可逆性后部脑病综合征(PRES)的临床特点、治疗及预后。方法:回顾性分析SLE合并PRES患者的临床表现、实验室检查及影像学资料,探讨其发病机制、危险因素、治疗及预后特点。结果:本研究共纳入16例SLE患者,女性14例、男性2例,平均年龄(22. 8±8. 5)岁,16例均有狼疮重度活动,SLE活动性指数为(20. 9±3. 7)分。发生PRES前15例(93. 8%)有高血压,12例(75%)合并肾功能不全、淋巴细胞计数低下、高脂血症,16例患者均使用激素或免疫抑制剂。临床症状有癫痫发作(16例,100%)、头痛(5例,31%)、视觉障碍(5例,31%),恶心呕吐(6例,38%)。CT及磁共振成像(MRI)显示病灶多位于双侧顶枕叶,也可累及颞叶、额叶、脑室、基底节、小脑及皮层。降压、抗癫痫和小剂量激素控制狼疮活动后症状可缓解。结论:SLE合并PRES是一种临床-影像综合征,发病与高血压、肾功能不全、狼疮重度活动等因素相关,临床表现有头痛、视力障碍、癫痫发作和恶心呕吐,脑部影像学表现为双侧顶枕区的皮层下血管源性水肿。SLE合并PRES时若能及时诊断并治疗,病变可逆,预后良好。     

系统性红斑狼疮患者的用药选择及其与肾脏损害相关性的回顾性分析

目的 了解系统性红斑狼疮(SLE)患者用药的基本情况,探讨规范化应用免疫抑制剂治疗的重要性,为改善SLE患者预后提供临床依据.方法 采用流行病学现况调查的研究方法,随机调查252例SLE患者,记录患者自发病以来的用药情况,并分析用药以及就诊情况等因素与肾脏损害之间的关系.结果 在252例SLE患者中药物治疗以糖皮质激素(99.2%)、免疫抑制剂(75.8%)、羟氯喹(61.5%)为主.免疫抑制剂以环磷酰胺为常用药(75.9%).应用免疫抑制剂6个月以上的患者仅有128例(50.8%).因惧怕不良反应而擅自停药仍为患者停用免疫抑制剂、羟氯喹的主要原因.通过单因素及多因素Logistic回归分析表明,规范应用激素、免疫抑制剂、羟氯喹治疗可以显著降低狼疮肾脏损害的发生率;并且应用免疫抑制剂治疗时间超过3个月,其肾损害以及肾功能不全的发生率明显降低(χ2=3.996,P＜0.05;χ2=13.196,P＜0.01),尤其是达到6个月以上的患者,这种差异更加明显(χ2=4.505,P＜0.05;χ2=8.453,P＜0.01).结论 SLE患者治疗主要是应用糖皮质激素联合免疫抑制剂、羟氯喹.尽早及规范应用糖皮质激素联合免疫抑制剂或羟氯喹治疗,可以显著降低狼疮肾脏损害的发生率.     

泰它西普治疗狼疮足细胞病1例

狼疮足细胞病(LP)是狼疮肾炎的一种特殊类型, 特征是SLE通过介导非免疫复合物沉积使得足细胞广泛损伤。激素对LP大多能起效, 但治疗后容易复发, 目前缺乏一种方案降低LP复发率。本例LP患者常规治疗后病情反复, 经加用泰它西普治疗后, 随访6个月患者临床完全缓解, 泼尼松顺利减量到10 mg/d, 未有病情复发。     

系统性红斑狼疮合并乙型肝炎病毒感染临床分析

目的 了解系统性红斑狼疮(SLE)合并乙型肝炎病毒(HBV)感染患者临床特点.方法 回顾性分析2003年2月至2011年6月在北京协和医院住院的40例合并HBV感染的SLE患者临床资料,并与性别、发病年龄相匹配的60例无HBV感染的SLE患者比较.统计学方法采用t检验和x2检验.结果 40例合并HBV感染者中男性4例,女性36例,平均年龄(32±13)岁;其中23例患者表现为慢性乙型肝炎,5例为HBV携带状态,12例为既往无症状感染.21例慢性乙型肝炎患者肝功能轻度异常主要为丙氨酸转氨酶升高,2例严重肝功能异常.临床表现合并HBV感染组和对照组比较,发热、肝脏受累、肾脏受累、血小板减少、巨细胞病毒感染更常见.肾病变主要为Ⅳ型或Ⅴ型狼疮肾炎,2例合并HBV相关性肾炎.血清乙型肝炎病毒表面抗原(HBsAg)阳性的SLE患者在接受免疫抑制剂治疗时同时使用拉米夫定抗病毒治疗.患者出院好转率为98％ (39/40),1例预后不佳者有严重肝脏病变.结论 应重视SLE合并HBV感染患者临床特点:SLE合并HBV感染患者发热、肝脏受累、肾脏受累、血小板减少、巨细胞病毒感染者增多,可合并HBV相关性肾炎;肝脏病变严重者预后差;血清HBsAg阳性的SLE患者在接受免疫抑制剂治疗时应同时使用抗HBV药治疗.     

老年狼疮肾炎发病机制探讨

<正>狼疮肾炎(LN)是系统性红斑狼疮(SLE)的肾脏损害,约50%以上SLE患者有肾损害的临床表现,肾活检显示肾脏受累几乎为100%。肾衰竭是SLE患者死亡的常见原因。目前针对LN治疗以激素联合免疫抑制剂为主,在治疗期间老年患者更易继发感染〔1〕。SLE患者体内出现多种自身抗体。这些抗体主要与核抗原结合,自身抗体与抗原结合形成免疫复合物后沉积于肾脏,逐步发展为LN。突破免疫耐受是SLE及LN发生的     

系统性红斑狼疮患者合并假性肠梗阻

目的:观察分析系统性红斑狼疮(SLE)患者合并假性肠梗阻(IPO)的临床特点及预后。方法:2004年1月至2014年4月南京军区南京总医院肾脏科住院确诊为SLE合并IPO患者30例,回顾性分析其临床表现、实验室检查结果、影像学特点、治疗及预后。结果:30例SLE合并IPO患者中,女性26例,男性4例,发生IPO时平均年龄(26.1±11.2)岁,SLE中位病程为27.5月,狼疮活动性指数(SLE-DAI)评分(11.2±7.4)分。6例有明确诱因(5例不洁饮食、1例上呼吸道感染)。IPO临床症状依次为恶心呕吐(76.6%)、腹痛(66.7%)、腹泻(56.7%)和腹胀(30%)。腹部平片及腹部CT可辅助诊断肠梗阻,阳性率75%,7例患者同时合并肾盂、输尿管积水。96.7%患者存在贫血,9例患者血小板减少,8例患者白细胞减少,4例直接Coombs试验阳性。96.7%的患者补体C3降低,ANA阳性率89.7%,抗ds-NDA抗体阳性率51.7%,抗心磷脂抗体阳性率30.4%,狼疮抗凝物阳性率43.8%,抗C1q抗体阳性率47.4%。29例患者接受激素为主[泼尼松0.6~1 mg/(kg·d)]的免疫抑制剂治疗(其中10例甲泼尼龙冲击治疗,11例丙种免疫球蛋白治疗),病情均好转出院,IPO好转中位数时间12d。24例患者长期随访(1~60月),2例死亡,4例行腹膜透析,5例反复发生IPO,至随访终点,肾脏完全缓解率25%,部分缓解率41.7%。结论:SLE累及消化道导致IPO临床相对罕见,但症状较重,容易误诊,早期识别,并及时给予免疫抑制剂治疗。     

泰它西普治疗狼疮足细胞病1例

狼疮足细胞病（LP）是狼疮肾炎的一种特殊类型,特征是SLE通过介导非免疫复合物沉积使得足细胞广泛损伤。激素对LP大多能起效,但治疗后容易复发,目前缺乏一种方案降低LP复发率。本例LP患者常规治疗后病情反复,经加用泰它西普治疗后,随访6个月患者临床完全缓解,泼尼松顺利减量到10 mg/d,未有病情复发。     

含汞化妆品相关肾病的临床病理特征及预后

目的:探讨使用含汞化妆品后出现肾脏疾病的临床病理特征、治疗及预后。方法:回顾性分析2013年1月至2019年12月间结合病史、临床、肾活检和尿汞检测诊断为含汞化妆品相关肾病患者的临床表现、实验室指标、肾脏病理特点、治疗及预后。结果:共收集41例患者,均为女性,平均年龄39.4±6.6岁,使用含汞化妆品中位时间为6月,平均尿汞水平为66.80±38.55μg/(g·Cr),临床表现以蛋白尿为主,多数患者(32/41,78.05%)为肾病综合征。肾活检病理:膜性肾病(MN)22例(53.65%)、微小病变肾病(MCD)13例(31.71%)、IgA肾病(微小病变型)(IgAN-MCD)5例(12.20%)、局灶节段肾小球硬化(FSGS)1例(2.44%)。病理表现为MN的患者发病前含汞化妆品使用的中位时间更长、合并自身抗体阳性的(以ANA为主)比例(7/22)更高,且血抗磷脂酶A2受体抗体(PLA2R-Ab)及肾组织抗磷脂酶A2受体(PLA2R)均阴性。接受激素和(或)免疫抑制剂治疗36例、血管紧张素受体拮抗剂(ARB)治疗5例,其中9例同时接受驱汞治疗。中位随访时间9月,尿检达完全缓解32例(78.05%),8例(19.51%)部分缓解,1例(2.44%)未缓解,激素减量后复发1例,15例成功停用免疫抑制剂及保肾药物。结论:含汞化妆品相关肾病临床主要表现为肾病综合征,肾小球病变多样,以MN为主,其次为MCD,激素或免疫抑制剂治疗后缓解率高,预后佳。     

系统性红斑狼疮合并Evans综合征临床特点

目的总结系统性红斑狼疮(systemic lupus erythematosus,SLE)合并Evans综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月SLE合并Evans患者的临床表现及实验室特点及治疗和预后。结果 SLE并发Evans综合征患者22例,占同期SLE住院患者3400例的0.65%。其中男3例,女19例,平均35.1岁(16~53岁)。22例患者中以血液系统受累[特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)或自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)]为首发表现的11例(50%),确诊SLE后诊断Evans综合征者6例,二者同时诊断的5例。SLE并发Evans综合征时,患者往往有多系统受累,表现为肾脏受累13例(59.1%),皮肤黏膜受累、关节炎各9例(40.9%),神经系统受累4例(18.2%),胃肠道、肺部受累各2例等。Evans综合征多发生于SLE活动期,患者平均狼疮活动指数评分(11.45±7.6)分(3~30分)。伴发其他结缔组织病5例(22.7%)。经激素联合免疫抑制剂治疗后,20例好转,2例无效者应用利妥昔单抗后好转。结论 SLE合并Evans综合征罕见,发生于SLE多系统受累及活动期。部分患者以ITP或AIHA为SLE首发表现,应及时筛查多种自身抗体,并定期随访密切观察,以期早期诊治。     

系统性红斑狼疮合并血栓性血小板减少性紫癜14例临床分析

 目的 探讨系统性红斑狼疮（SLE）合并血栓性血小板减少性紫癜 (TTP) 的临床特点、诊断及治疗。方法 回顾性分析14例SLE合并TTP者的临床表现、实验室检查结果、诊治和转归。结果 14例SLE合并TTP者中,男4例,女10例,中位年龄23（17～69）岁。5例以SLE首发,9例两者同时发病。所有患者均有血小板减少、微血管病性溶血性贫血,发热、神经系统异常、肾功能损害的发生比例分别为12/14、11/14、11/14,其中8例患者具有上述五联征。糖皮质激素（单用或联合静脉注射人免疫球蛋白和环磷酰胺）治疗6例,糖皮质激素联合血浆置换8例,有效率分别为2/6、6/8。8例患者存活,6例死亡。对6例存活者进行了随访,均无复发。结论 SLE与TTP临床表现相似,多次重复血涂片检查对早期明确诊断SLE合并TTP十分重要。SLE合并TTP者肾功能损害程度比单纯TTP或SLE者更为严重,早期诊断并及时应用糖皮质激素联合血浆置换有助于改善患者的预后。     

Two cases of acute lupus peritonitis]

We report two cases of systemic lupus erythematosus (SLE) diagnosed when acute peritonitis was appeared. Case 1 was a 20 year-old woman suffering from stomachache and right lower abdominal pain. Case 2 was a 40 year-old woman with diarrhea, epigastralgia, pollakisuria. In both cases, their peritoneal fluids were exudative with positive autoantibodies. After high dose steroid therapy, abdominal symptoms and ascites improved promptly. However, due to the complication of lupus nephritis, additional therapy was necessary. To characterize the feature of lupus peritonitis (LP), we examined the clinical and laboratory findings of LP from the literature. In patients with acute LP, abdominal pain, vomiting, diarrhea were significantly more common compared with chronic LP patients (P < 0.05), and fever, arthritis, central nervous system involvement and cystitis were more common. In patients with chronic LP, pleural effusion and pericardial effusion were more common compared with acute LP patients. Gastrointestinal manifestations such as abdominal pain, vomiting and diarrhea were more common in patients with acute LP compared with patients with chronic LP. Most patients with chronic LP were asymptomatic, ascites and serositis being the only clinical findings. The response to steroid therapy was better in acute LP.     

Human immunodeficiency virus infection and systemic lupus erythematosus. An unusual case and a review of the literature

Systemic lupus erythematosus (SLE) and infection with the human immunodeficiency virus (HIV) are rarely seen in the same patient. Both diseases share clinical and serological features and the differential diagnosis is difficult, with renal manifestations being of special interest. To date, 29 cases of association between the two diseases have been reported, but the diagnosis was simultaneous in just two of these and only 18 fulfilled the ARA criteria for the diagnosis of SLE. Most patients experienced an improvement in their SLE after development of their HIV associated immunosuppression and a reactivation of lupus manifestations has also been noted after immunological recovery secondary to antiretroviral therapy. We present the case of a woman in whom HIV and SLE with renal involvement were diagnosed simultaneously.     

Accuracy of cerebrospinal fluid IL-6 testing for diagnosis of lupus psychosis. A multicenter retrospective study

Psychiatric manifestations are relatively common in systemic lupus erythematosus (SLE) patients. Since there are factors causing psychiatric manifestations other than SLE, the diagnosis of lupus psychosis (LP) is often difficult. Previous studies disclosed that cerebrospinal fluid (CSF) IL-6 was elevated in SLE patients with neuropsychiatric manifestation. The current studies were therefore designed to examine the efficacy of CSF IL-6 in diagnosis of LP. Multicenter retrospective study was performed with 45 SLE patients who showed psychiatric manifestations between 1993 and 2000. The diagnosis of LP and psychosis due to causes other than SLE (non-LP) was confirmed by retrospective review of the clinical records. Thirty-two of the 45 patients were reconfirmed as LP in the retrospective study. Receiver operating characteristic curve analysis revealed that the sensitivity and specificity of CSF IL-6 for diagnosis of LP were 87.5% and 92.3%, respectively, at the cut-off value of 4.3 pg/ml. These results indicate that CSF IL-6 might be an effective measure in diagnosing LP, although exclusion of infectious meningoencephalitis and cerebrovascular accident is necessary.     

蛋白丢失性肠病61例临床分析

目的 通过对蛋白丢失性肠病临床资料的总结分析,提高对本病的认识.方法 对北京协和医院1997至2009年诊断的61例蛋白丢失性肠病进行分析总结.结果 男26例,女35例,年龄16～77(40±15)岁.水肿为首发症状51例;腹水为主要症状41例;合并双侧胸腔积液23例;腹痛16例,腹泻33例;所有患者均有显著的低蛋白血症.37例患者经核素99Tcm标记白蛋白显像证实存在肠道蛋白丢失,24例为临床诊断.原发病主要为系统性红斑狼疮(28例),先天性淋巴管扩张(12例).治疗上以原发病治疗为主.结论 蛋白丢失性肠病临床并非罕见,以严重的低蛋白血症和多浆膜腔积液为特征,核素99Tcm标记白蛋白显像是特异性的诊断方法之一,治疗上以原发病治疗为主,预后与原发病控制与否相关.

Abstract：

Objective To increase the understanding in protein-losing enteropathy (PLE).Methods Sixty-one PLE patients were enrolled in the study and the clinical characteristics, complicated disease, diagnosis and treatment were analyzed. Results The age of the patients was 16-77 (40±15)years, and the gender ratio was 35:26 (female: male). The main clinical manifestations were bilateral lower limb edema in 51 cases, ascites in 41 cases, bilateral pleural effusion in 23 cases, pericardial effusion in 13cases, abdominal pain in 16 cases and diarrhea in 33 cases. The prominent abnormality in laboratory examinations was hypoalbuminemia. The underlying diseases include systemic lupus erythematosus (SLE) in 28 cases, intestinal lymphangiectasia in 12 cases, hepatic cirrhosis in 5 cases, heart diseases in 5 cases,Crohn's disease in 3 cases, membranous nephropathy in 2 cases, Budd-Chiari syndrome in 1 case. Four cases happened after abdominal operation and 1 case after radiation therapy of gastric cardia cancer. Thirtyseven cases were diagnosed by 99Tcm-labelled human serum albumin scintigraphy and 24 cases were diagnosed clinically. Treatment was focused on underlying diseases. The clinical manifestations in 21 cases of SLE improved after SLE was controlled. In 2 cases of intestinal lymphangiectasia and one with Crohn's disease, the clinical manifestations improved after surgery. The other patients had no improvement.Conclusions PLE was not uncommon in clinical practice. Its predominant characteristics were severe hypoalbuminemia, edema and dropsy of serous cavity. PLE can complicate other diseases such as SLE,intestinal lymphangiectasia. Treatment should be focused on primary disease.     

Seronegative systemic lupus erythematosus: etiology of nephrotic syndrome and acute renal failure in early postpartum period

Systemic lupus erythematosus (SLE) is an autoimmune syndrome that occurs most commonly in women during their reproductive years. Nephritis is known to be one of the most serious complications of SLE. Lupus nephropathy is frequently associated with ANA and anti-dsDNA antibodies. Rarely, serological markers may be initially absent, and in many cases, they become positive after sometime. We present a 28-year old, otherwise healthy female who admitted to our clinic with edema, hypertension, proteinuria and acute renal failure following her fourth delivery. Serum immunological markers were negative and renal biopsy showed histopathological changes consistent with systemic lupus erythematosus as the etiology of nephrotic syndrome. A dramatic therapeutic response was achieved by pulse steroid and cyclophosphamide treatment following oral steroid therapy. In women with new onset nephrotic syndrome or renal function deterioration in postpartum period, even if the patient is asymptomatic or seronegative, it is crucial to exclude SLE for a rapid diagnosis and prompt treatment in the case of lupus nephritis. Renal biopsy is of diagnostic importance in such cases in which there is no other clinical, biochemical and serological evidence of the disease.     

Pemphigus vulgaris and systemic lupus erythematosus in a 46-y-old man.

Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many organs in the body. Skin manifestations are frequent and sometimes vesiculobullous lesions may apper such as in bullous lupus erythematosus. SLE may also be exceptionally associated with other blistering diseases such as pemphigus vulgaris (PV). We describe the case of a male diagnosed as having SLE, who one year later developed PV. Only three cases (all in women) of SLE associated with PV have been reported in the literature. Our case is noteworthy in that this exceptional association developed in a middle-aged male.     

系统性红斑狼疮患者发病及就医行为的现况调查

目的 回顾性分析系统性红斑狼疮(SLE)的发病形式、临床特点以及患者发病后的就诊情况.方法 采用流行病学现况调查的研究方法,随机调查了300例SLE患者,了解其自发病以来的临床表现以及发病后的就诊情况,并采用SPSS 13.0统计软件包进行统计学分析.结果 ①在300例SLE患者中,男女之比为1:13.②首发临床表现以关节痛/炎最多,其次为皮疹、发热,分别占46.3%、34.0%、32.7%.与女性患者相比,男性更容易于发病时即出现肾脏损害.60.9%的患者于发病起1年内出现肾脏损害,提示自SLE发病起1年为肾脏损害的高发期.③患者首次就诊选择风湿免疫科者较少,仅占35.3%(106/300),但风湿免疫科的确诊比例最高,为99.1%(105/106).从出现临床症状到患者就诊的中位时间为半个月,从患者出现症状到确诊SLE的中位时间为3个月,有23.7%的患者需要1年以上方能确诊.结论 关节痛/炎、皮疹、发热为SLE最常见的首发临床表现.与女性患者相比,男性患者更容易出现肾脏损害.发病后不能正确选择科室就诊、诊断延误的SLE患者仍占相当大比例.     

<Emphasis Type="Italic">Listeria monocytogenes</Emphasis> infection in patients with systemic lupus erythematosus

Listeria monocytogenes infection (LMI) is a rare complication in systemic lupus erythematosus (SLE) patients, and it is associated with nonspecific clinical manifestations and is often mistaken with SLE flares. Several cases of LMI in SLE patients have been reported, with high mortality rates. This article describes five new cases of LMI in patient with SLE in a cohort of 174 patients (2.8%). All patients were women, with a mean age of 19.4 years (range, 5–29 years). Mean duration of SLE before clinical LMI was 2.8 years (range, 2–4 years). Recurrent infection was not evidenced. At the time of LMI, all patients had an inactive disease, receiving steroids and immunosuppressive treatment. Clinical picture of meningitis was present in two patients. All patients were treated with ampicillin, with resolution of clinical manifestations without sequels. In order to eliminate the intracellular forms of L. monocytogenes, trimethoprim–sulfamethoxazole was initiated, and an allergic skin reaction was presented in all but one patient. Our report highlights the unspecific clinical manifestations of LMI and these characteristics are initially challenging and may be interpreted as lupus flares. An accurate diagnosis and an early antibiotic treatment are essential to improve the outcome in these patients.