系统性红斑狼疮患者合并妊娠的临床研究

目的 探讨系统性红斑狼疮(SLE)患者妊娠的安全性、妊娠结局及对子代的影响.方法 回顾性分析1999年6月至2009年10月我院收治的SLE合并妊娠的患者的妊娠情况,比较选择性妊娠和非选择性妊娠组患者的SLE疾病活动情况、产科并发症情况、胎儿情况.并对SLE患者的子代进行随访.统计学处理采用x2检验和t检验.结果 SLE合并妊娠的患者共62例,选择性妊娠组43例,非选择性妊娠组19例;选择性妊娠组中10例(23%)患者在妊娠过程中出现疾病活动,8例(19%)流产,35例(81%)活胎分娩,其中低体质量儿7例,早产7例;非选择性妊娠组中16例(84%)出现疾病活动,13例(68%)流产,6例(32%)活胎分娩,均为低体质量儿,4例早产,3例合并胎儿生长受限.选择性妊娠组的妊娠过程中疾病活动率、流产率均显著低于非选择性妊娠组(P＜0.05).22例子代随访未发现SLE患儿.结论 选择性妊娠组与非选择性妊娠组患者均面临妊娠过程中SLE疾病活动及妊娠结局不良的风险,但是选择性妊娠组患者妊娠期间疾病稳定状况、母婴的预后均优于非选择性妊娠组.     

系统性红斑狼疮伴发急腹症20例分析及文献复习

目的 提高对系统性红斑狼疮（SLE）伴发急腹症（AA）临床多样性的认识。总结治疗此类病例的经验。方法 对20例SLE伴发AA的病例做回顾性分析。并复习35年相关文献。结果 AA多数是SLE病情活动的表现（70%），也可能由独立于SLE的其他疾病引直民（30%），病变广泛多变，容易误诊，早期确定采用保守或手术治疗是提高生存率的关键。结论 SLE伴发AA预示病情危重，应尽早明确AA的原因，恰当选择治疗措施。     

Cutaneous lupus erythematosus: Diagnosis and treatment

Cutaneous lupus erythematosus (CLE) encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several sub-types, including acute CLE (ACLE), sub-acute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain cutaneous lupus and lupus tumidus. The diagnosis of these diseases requires proper classification of the sub-type, through a combination of physical examination, laboratory studies, histology, antibody serology and occasionally direct immunofluorescence, while ensuring to exclude systemic disease. The treatment of cutaneous lupus consists of patient education on proper sun protection along with appropriate topical and systemic agents. Systemic agents are indicated in cases of widespread, scarring or treatment-refractory disease. In this chapter, we discuss issues in classification and diagnosis of the various sub-types of CLE, as well as provide an update on therapeutic management.     

系统性红斑狼疮患者外周血单个核细胞骨调素表达及临床意义

目的 探讨骨调素在系统性红斑狼疮（ＳＬＥ）患者中的表达及临床意义。方法 用流式细胞仪和Ｎｏｒｔｈｅｒｎ ｂｌｏｔ杂交方法分别检测ＳＬＥ患者外周血单个核细胞（ＰＢＭＣ）ＯＰＮ蛋白和ｍｔｎＡ的表达。结果 活动期和稳定期ＳＬＥ患者ＰＢＭＣ ＯＰＮ蛋白和ｍＲＮＡ的表达均高于正常人（Ｐ〈０．０１）,ＳＬＥ患者ＯＰＮ蛋白主要在淋巴细胞表达,而活动期患者ＰＢＭＣ ＯＰＮ蛋白及ｍＲＮＡ的表达高于稳定期患者（Ｐ〈０     

Systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a typical autoimmune disease that leads to multiple organ damage. For over half a century, SLE has been treated mainly with nonspecific glucocorticoids and immunosuppressants, and the development of molecular target drugs with few adverse reactions is awaited. The treatment goal is remission without systemic symptoms or organ damage. An anti-B-cell activating factor antibody belimumab and an anti-type I interferon receptor antibody anifrolumab are used for patients with active SLE who respond poorly to standard of cares. Additionally, as many susceptibility genes for SLE are associated with signal transduction of dendritic and B cells, cytokines and signaling molecules that bridge the innate and adaptive immune systems are the current focus of attention. Promising approaches include the development of a Janus kinase inhibitors targeting tyrosine kinase deucravacitinib, plasmacytoid dendritic cell-targeted drugs, proteasome inhibitors (e.g., iberdomide), type II anti-CD20 antibody, and obinutuzumab.     

系统性红斑狼疮患者妊娠与分娩过程处理的初步探讨

目的 探讨系统性红斑狼疮(SLE)患者的妊娠时机、妊娠期并发症处理、终止妊娠时机、分娩过程中及娩后治疗问题。方法 前瞻性地观察29例SLE患者妊娠结果，其中23例患者病情控制1年以上妊娠。2例妊娠前1年内有病情活动，4例妊娠期首发SLE。结果 10例发生妊娠高血压综合征，6例妊娠晚期伴发心力衰竭．3例妊娠晚期伴发广泛性肺间质炎。29例患者均于孕30～37周终止妊娠，出生婴儿29名．无新生儿狼疮．无母婴死亡。6例分娩后狼疮加重。结论 SLE患者病情控制1年以上妊娠母婴安全度显著提高．疾病活动、妊娠过程及产后适当应用激素控制并发症是成功的关键，有并发症者应适时终止妊娠以确保母婴安全。     

Lupus erythematosus and lupus valgaris: a case report and historical remarks

Lupus erythematosus and lupus valgaris, i.e., cutaneous tuberculosis, are confusing skin lesions in the history. Here, we reported on a Chinese lupus patient manifested with multiple nodular erythematous rashes on both upper extremities, which turned out to be cutaneous mycobacterial infection.     

系统性红斑狼疮合并获得性血友病A一例并文献复习

目的总结系统性红斑狼疮(SLE)合并获得性血友病A的临床表现及治疗,以提高对SLE合并获得性血友病A的认识。方法对1例SLE合并获得性血友病A的临床、实验室资料及治疗进行分析,并进行文献复习。结果患者为25岁女性患者,SLE病史5年,此次以腹痛为主诉,实验室检查活化部分凝血活酶时间(APTT,135．3s)明显延长,因子Ⅷ促凝活性(FⅧ：C,0．9%)减低,因子Ⅷ抑制物滴度26．1U/ml(Bethesda法),B超及核磁共振均发现子宫后血肿,治疗上给予激素、因子Ⅷ制品、大剂量丙种球蛋白等,临床症状及实验室指标明显好转,出院后给予激素及环磷酰胺治疗,随访10个月患者一般情况可。结论SLE合并血友病A在临床上应引起高度的重视,在治疗上目前尚无标准方案。     

系统性红斑狼疮细胞与其病情活动性的相关性研究

目的 观察系统性红斑狼疮（SLE）患者外周血狼疮细胞（LEC）形态的变化，探讨其与SLE疾病活动性的关系。方法 采用经典改良血块法观察了50例SLE疾病活动期和30例SLE非活动期患者外周血LEC形态，同时与血清自身抗体、补体及SLE疾病活动指数（SLEDAI）对比研究。结果 特殊形态的LEC与自身抗体的抗dsDNA抗体和抗核小体抗体（AnuA）（r=0．588，P=0．056；r=0．759，P=0．135），补体C3和C4（r=-0．648，P=0．058；r=-0．589，P=0．057）及SLEDAI（r=0．686．P〈0．05）具有明显相关性。SLE活动期组特殊型LEC、自身抗体和补体与SLE非活动期组差异有统计学意义（P〈0．01）。当自身抗体和补体与SLEDAI无相关性时，特殊型LEC仍具有良好的相关性（r=0．786，P〈0．05）。结论 特殊型LEC与自身抗体、补体及SLEDAI有明显的相关性，可作为判断SLE疾病活动性的独特指标。     

小剂量利妥昔单抗治疗系统性红斑狼疮的临床观察

目的 评价小剂量利妥昔单抗治疗系统性红斑狼疮(SLE)的疗效和安全性.方法 对既往激素和多种免疫抑制剂治疗无效或复发的10例SLE患者,予利妥昔单抗100mg,使用4次,同时仅联合使用激素,观察疗效和不良反应.结果 利妥昔单抗对狼疮肾炎、神经精神性狼疮、顽固性血小板减少以及自身免疫性溶血性贫血均显示有良好的疗效,且起效迅速.10例患者中仅1例出现了泌尿系感染,1例在首剂利妥昔单抗治疗1周后猝死,但不能确定死亡原因与利妥昔单抗相关.结论 小剂量利妥昔单抗治疗SLE有良好的疗效和安全性,并且降低了治疗费用.     

Evaluation of visfatin in patients with systemic lupus erythematosus: Correlation with disease activity and lupus nephritis

IntroductionRenal involvement affects about 50% of SLE patients accounting for significant morbidity and mortality in these patients. The adipokine “visfatin” acting as a growth factor for B-lymphocyte-precursors, exerts several proinflammatory functions. It was demonstrated as a marker of endothelial dysfunction (ED) in chronic kidney disease (CKD) thus could be a factor linking inflammation in SLE and kidney disease.Aim of the workTo assess serum visfatin level in SLE patients and its correlation to disease activity and lupus nephritis (LN) in these patients.Patients and methodsSerum level of visfatin using enzyme-linked immunosorbent assay (ELISA), chemical and immunological markers of SLE and LN were measured in 40 SLE patients and 40 age and sex matched healthy controls. Disease activity and renal involvement were assessed using SLE Disease Activity Index (SLEDAI) and Renal SLEDAI respectively further dividing patients into active versus inactive and LN versus non-LN respectively. Renal biopsies were taken from LN subgroup and were classified according to the modified WHO classification.ResultsA significantly higher serum visfatin level was found on comparing SLE patients (mean 109 ± 180 ng/ml, median18) with controls (mean 9.4 ± 11 ng/ml, median2.5) with statistically highly significant difference (z = 5.2, P < 0.001). Also there was a statistically significant difference as regards serum visfatin level between active SLE patients (mean 173 ± 111 ng/ml, median 14) and inactive patients (mean 139 ± 88 ng/ml, median 5) (z = 2.1, P < 0.05) as well as between patients with LN (mean 226 ± 180 ng/ml, median18) and patients with no LN (mean 101 ± 140 ng/ml, median 8(2-229)) (z = 2.1, P < 0.05). Visfatin had a highly significant positive correlation with disease duration (r = 0.48, P < 0.001), SLEDAI (r = 0.62, P < 0.001) as well as ESR, CRP and, renal score (r = 0.45, 0.35, and 0.65, respectively) while inverse correlation with estimated GFR (r = ?0.614) and C3 and C4 titre (r = ?0.26, r = ?0.35, respectively) was recorded. Visfatin showed high sensitivity in detecting active SLE and LN 83% and 85%, respectively.ConclusionSerum visfatin is strongly associated with LN in SLE patients and is a promising biomarker for prediction of renal involvement in these patients. It reflects SLE activity specially LN activity namely renal score and GFR decline. Further prospective studies are required to confirm visfatin as a destructive mediator of predictive and prognostic value in active lupus nephritis.     

活性DNA诱导抗核抗体及系统性红斑狼疮样综合征

目的 寻找系统性红斑狼疮真正的核成分免疫原。方法 从ＣｏｎＡ活化的脾淋巴细胞中提取ＤＮＡ,然后同系免疫ＢＡＬＢ／ｃ小鼠,用ＥＬＩＳＡ方法测定ＩｇＧ类抗ｄｓＤＮＡ,抗组蛋白抗体,用免疫荧光法检测抗核抗体核型和免疫复合物沉积,用免疫印迹法测定抗可溶性抗原抗体,用考马斯亮蓝法检测抗体阳性的动物尿蛋白含量。结果 活性ＤＮＡ能诱导抗ｄｓＤＮＡ、抗组蛋白等多种抗核抗体生成,且能诱发同系小鼠产生ＳＬＥ样综合征。     

系统性红斑狼疮伴发假性肠梗阻十例分析

目的了解系统性红斑狼疮(SLE)伴发假性肠梗阻(IPO)的临床特征,提高对该病的诊治水平。方法收集武汉同济医院2001年7月至2005年7月收治随访的10例SLE伴发IPO的临床资料,按1:2比例选择20例同期收治性别、年龄相配,无IPO伴发的SLE病例对比分析。并搜寻国内、国外(仅限于英文)相关病例40例一并综述。结果10例IPO中,以IPO为首发表现的病例数、IPO合并泌尿系统损害及治疗后稳定病例数分别为1、5和4例,综述文献三者比例分别为46%,64%和46%。与不伴有IPO的单纯SLE病例相比,伴发IPO者其外周血白细胞数相对较高(8.1±5.2vs4.0±1.8,P<0.05),而SLE疾病活动指数(SLEDAI)评分则较低(6.1±2.2vs9.6±1.8,P<0.01),其他病情观察指标差异均无统计学意义。结论IPO可为SLE的首发表现,但往往也是SLE病程中的一种并发症,它常与泌尿系统的病变相关联。激素和免疫抑制剂能够迅速改善病人的临床症状和预后。     

系统性红斑狼疮伴结核菌感染临床特点分析

目的 分析系统性红斑狼疮(SLE)并发结核菌感染的临床特点。方法 分析我院自1991年1月至2004年1月共收治的426例SLE住院患者。结果 426例SLE患者中18例伴有结核，患病率为4．2％，死亡7例，病死率为39％。明显高于普通人群。其中肺结核12例，结核性脑膜炎3例，淋巴结核3例。18例做结核菌素试验纯蛋白衍生物(PPD)强阳性2例(12．5％)，阴性16例(87，5％)，结核菌素试验PPD阴性率明显高于正常人群。15／18(83％)患者在应用免疫抑制剂6个月-3年内患结核。结论 SLE患者由于长期服用免疫抑制剂，易发生结核菌感染且临床表现不典型。其患病率及病死率均明显高于普通人群．因此对结核菌感染的早期诊治对降低病死率十分重要。     

羟氯喹治疗系统性红斑狼疮合并妊娠24例临床研究

目的 评价羟氯喹治疗系统性红斑狼疮(SLE)患者合并妊娠的疗效与安全性.方法 回顾性分析2006年5月至2011年2月中日友好医院风湿免疫科收治的24例妊娠期间使用羟氯喹治疗SLE患者的疾病控制和新生儿安全情况,并对其子代进行随访.结果 24例患者中22例在妊娠期间持续应用羟氯喹治疗,21例患者SLE病情平稳,无复发.2例患者妊娠后停用羟氯喹,在妊娠中期出现SLE活动,增加糖皮质激素剂量并再度加用羟氯喹,SLE病情稳定至分娩和产后.3例患者诊断妊娠高血压,3例患者早产.副作用:2例患者在妊娠期加用羟氯喹后,分别出现一过性厌食和脱发,未予特殊治疗,症状大约持续3周后自行缓解.所有患者均未出现眼部不适及视野缺损症状.至目前为止,患者在我院眼科门诊定期复查视野及眼底均无异常发现.新生儿听力筛查和身高、体重均在正常范围内,未发现心肺异常及生长发育异常.结论 羟氯喹对SLE妊娠期妇女及胎儿具有良好的安全性,应在妊娠期间持续使用.     

亚临床狼疮性肾炎的病理报告(附37例初发SLE肾活检病理)

目的 研究初发、临床及实验室无任何肾损害证据的系统性红斑狼疮（ＳＬＥ）患者肾脏病理改变及临床意义。方法 选择３７例初发确诊为ＳＬＥ，血、尿实验室检查无肾脏损害的病例，行肾穿刺活检，观察其肾单位的病理改变。结果 ３７例研究对象肾脏病理学上均有损害。小球病损率为１００％，其中ＷＨＯⅡａ型５例（１３．５％），ＷＨＯⅡｂ型１４例（３７．８％），ＷＨＯⅢ型７例（１８．９％），ＷＨＯⅣ型９例（２４．３％），Ｗ     

Cutaneous warts in patients with lupus erythematosus

The aim of this study was to determine the frequency of cutaneous warts (CW) in patients with lupus erythematosus (LE) and evaluate the effect of immunosuppressants on the appearance of CW. Fifty-eight patients with LE, 74 with rheumatoid arthritis (RA), and 105 healthy individuals were interviewed and examined for the presence of CW. All were questioned to find out whether their CW developed before or after onset of the disease or during treatment. Cutaneous warts occurred considerably more often in patients with LE than in healthy control and RA patients (P<0.003 and P<0.04, respectively). The presence of CW did not correlate with the taking of immunosuppressive drugs. The findings suggest that the high prevalence of CW in patients with LE is probably due to defects in some immune mechanisms, independently of immunosuppressive drugs.     

Maternal and fetal outcomes of 72 pregnancies in Argentine patients with systemic lupus erythematosus (SLE)

The purpose of the following study was to analyze maternal and fetal outcomes in pregnant patients with systemic lupus erythematosus (SLE) and the influence of SLE exacerbations on those pregnancies. Seventy-two pregnancies in 61 SLE patients treated between January 1986 and February 2004 in Hospital de Clínicas “José de San Martin” were reviewed retrospectively. Patient age was 28.1 ± 6.2 years (mean±standard deviation [SD]). Mean SLE duration was 4.5 ± 3.2 years (range 6 months–10 years). No patient acquired the disorder during gestation. Four (5.5%) patients had signs of active disease at the beginning of her pregnancy. Sixteen patients, accounting for 20 pregnancies, had a history of lupus nephritis. Nine patients met secondary antiphospholipid syndrome criteria and had 13 pregnancies. There were 14 exacerbations of the disease during pregnancy (19.4%), with most flares being mild. The most common obstetric complications were gestational hypertension in 15 pregnancies (20.8%) and preeclampsia in 8 pregnancies (11%). Forty-six percent of pregnancies ended in preterm deliveries. There were 62 live births (1 twin birth; 85%), 6 stillbirths (8%), and 5 spontaneous abortions (7%). Thirty-nine percent of newborns had low birth weight. Adequate pregnancy follow-up and delivery care by an interdisciplinary team in Argentine SLE patients with no pre-gestational preparation resulted in maternal and fetal outcomes similar to those seen in world reference centers.     

系统性红斑狼疮合并冠心病临床特点

目的总结系统性红斑狼疮（SLE）患者合并冠心病的临床特点。方法对1970—2006年北京协和医院院诊断为SLE合并冠心病11例患者传统危险因素、第一次心脏事件发作时的情况、SLE诊断、治疗及活动情况进行了回顾性分析。结果SLE患者发生冠心病的年龄较小为（53．9±8．1）岁;冠心病传统危险因素较少为（1．3±0．8）个／例;糖皮质激素使用前后各项血脂指标均有显著增高（P〈0．05）;发生心脏事件时的狼疮活动评分（SLEDAI）较高为（12．0±10．3）分;SLE患者冠状动脉（冠脉）病变程度较重,表现为弥漫狭窄、重度钙化。结论SLE患者早发冠心病不能完全用传统危险因素来解释,而且冠脉病变程度往往较重,预后差,需要尽早干预,尽量延缓疾病进程,并使用无创检查加强早期检出率,从而改善预后。     

系统性红斑狼疮合并心肌受累病例分析

目的 通过对系统性红斑狼疮(SLE)心肌受累病例的分析,提高对本病的认识.方法 总结分析北京协和医院近10年共13例SLE心肌受累病例临床资料及预后.结果 SLE心肌受累发生率0.52%,糖皮质激素治疗前后左心室射血分数分别为(37.7±5.8)%和(40.9 ±7.1)%(P=0.002),抗rRNP抗体与治疗后左心室射血分数改善显著相关(r=0.843,P=0.001).cTnI与左心室舒张末期内径改善相关(r=0.656,P=0.036).结论 SLE心肌受累仍属罕见病,预后较差,心脏超声可早期诊断并监测病情,激素治疗可以改善症状,一些血清学指标与病情相关.