SLE患者血清催乳素水平检测及与SLEDAI积分的相关性

目的探讨催乳素在系统性红斑狼疮（SLE）发病中的作用。方法采用ELISA法检测64例SLE患者（SLE组）和20例查体健康者（对照组）血清催乳素水平,并评定SLE患者SLE疾病活动度指数（SLEDAI）积分,分析SLEDAI积分与血清催乳素水平的相关性。结果 SLE组血清催乳素水平明显高于对照组（P〈0.01）,SLE组SLEDAI积分为（11±6）分,其中≥10分（活动期）28例,〈10分（缓解期）36例。活动期血清催乳素水平明显高于缓解期（P〈0.001）;血清催乳素水平与SLEDAI积分呈正相关（r=0.490,P〈0.01）。结论催乳素在SLE发病中起重要作用;其水平变化有助于SLE的诊断和病情监测。     

系统性红斑狼疮合并冠心病的临床特点及治疗现状

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种累及多脏器的自身免疫性炎症性结缔组织病。近20年来,SLE全因病死率在不断下降,心血管疾病已成为SLE患者死亡最主要的原因。SLE合并冠心病(coronary hearty disease,CHD)是心血管事件主要表现形式,往往起病早、病变重、病死率高。积极控制危险因素、药物治疗和血运重建是降低其病死率的有效措施。     

血清铁蛋白水平与成人系统性红斑狼疮疾病活动的关系

目的探讨成人系统性红斑狼疮(SLE)患者疾病活动度与血清铁蛋白(SF)的关系。方法初次确诊为SLE的患者40例依据SLE疾病活动指数(SLEDAI)评分分为稳定组与活动组。同期确诊为其他自身免疫性疾病的患者38例纳入其他对照组,40例健康体检者纳入正常对照组。电化学发光法检测SF水平,酶联免疫吸附(ELISA)法检测抗ds DNA抗体,同时回顾性收集SLE患者C反应蛋白(CRP)、红细胞沉降率(ESR)、补体及尿蛋白等实验室检查结果。结果 SLE患者SF水平高于其他对照组和正常对照组(P<0.05),且活动组SF水平高于稳定组(P=0.044)。SLE患者SF与SLEDAI评分(r=0.48,P<0.05)、抗ds DNA(r=0.34,P<0.05)、补体C3(r=-0.367,P=0.02)、C4(r=-0.371,P=0.018)均有相关性。另发现尿蛋白阳性的SLE患者SF水平较尿蛋白阴性者高(P<0.001)。结论 SF与SLE疾病活动密切相关,与SLEDAI评分、抗ds NDA抗体滴度及补体C3、C4等指标联合检测可判断成人SLE疾病活动程度,并指导临床治疗。     

系统性红斑狼疮患者CD4~+CD25~+调节性T细胞及TGF-β1表达的临床意义

目的探讨系统性红斑狼疮(SLE)患者CD4+CD25+调节性T细胞(Treg)及转化生长因子(TGF)-β1表达的临床意义。方法 SLE患者88例,其中活动期者35例(活动组),稳定期25例(稳定组),肾病期28例(肾病组),另选同期在医院实施健康体检的志愿者30例为对照组,检测并对比各组CD4+CD25+Treg及TGF-β1水平,各组SLE病情活动度积分(SLEDAI)、红细胞沉降率(ESR)及补体C3水平,分析指标间的相关性。结果肾病组及活动组CD4~+CD25~+Treg及TGF-β1水平均明显低于稳定组和对照组,且肾病组明显低于活动组(均P<0.05)。肾病组及活动组SLEDAI积分及ESR水平明显高于稳定组和对照组,补体C3水平明显低于稳定组和对照组,且肾病组SLEDAI积分及ESR水平明显高于活动组,补体C3水平明显低于活动组(均P<0.05)。根据Spearman相关性分析,患者CD4+CD25+Treg及TGF-β1与SLEDAI积分和ESR均呈负相关,与补体C3呈正相关。结论 SLE患者CD4+CD25+Treg及TGF-β1水平可随疾病的进展而下调表达,且可以预测患者病情进展。     

系统性红斑狼疮患者磷脂酶C-γ1与磷脂酶C-γ2表达的研究

目的 探讨系统性红斑狼疮(SLE)患者外周血单个核细胞(PBMCs)中磷脂酶C-γ1、磷脂酶C -γ2的表达水平及其与SLE疾病活动性的相关性.方法 运用半定量聚合酶链反应(RT-PCR)法,检测30例SLE患者与25名健康对照人的磷脂酶C-γ1与磷脂酶C-γ2 mRNA表达水平,并采用Pearson相关性检验分析它们与补体C3、C4、抗双链DNA (dsDNA)抗体及SLE疾病活动指数(SLEDAI)积分的相关性.结果 ①SLE组与健康对照组相比,磷脂酶C-γ1与磷脂酶C-γ2 mRNA表达显著增高(分别为P＜0.01);②磷脂酶C-γ1与磷脂酶C-γ2 mRNA表达水平之间呈正相关(r=0.726,P＜0.01);③SLE组磷脂酶C-γ1 mRNA表达水平与补体C3、C4、抗dsDNA抗体均无相关性,而与SLEDAI积分呈正相关(r=0.002,P＜0.05).磷脂酶C-γ2与补体C3、C4呈显著负相关(r=-0.914,P＜0.01;r=-0.829,P＜0.05),与抗dsDNA抗体正相关(r=0.171,P＜0.05),与SLEDAI积分相关性不明显.结论 本研究发现SLE患者磷脂酶C-γ1与磷脂酶C-γ2的表达水平在SLE患者中增高,磷脂酶C-γ1与SLEDAI积分呈正相关,磷脂酶C-γ2与补体C3、C4呈负相关,与抗dsDNA抗体正相关,可能对SLE患者的诊断和病情评估有较大价值.     

系统性红斑狼疮患者干扰素诱导基因的表达及其与疾病活动性的相关性研究

目的 探讨系统性红斑狼疮(SLE)患者外周血单个核细胞(PBMCs)中5个干扰素(IFN)诱导基因(IFIT1、IFIT4、OAS1、OASL、ISG15)的表达及其与SLE疾病活动度的相关性.方法 运用SYBRgreen dye I实时定量聚合酶链反应(RT-PCR)方法检测76例SLE患者与54名健康对照人的IFIT1、IFIT4、OASL、OASL及ISG15 mRNA表达水平(以-AACt值表示),并且与红细胞沉降率(ESR)、C反应蛋白(CRP)、补体C3、C4、抗核抗体(ANA)及抗dsDNA抗体等指标比较,分析IFIT1、IFIT4、OAS1、OASL、ISG15 mRNA表达水平以及上述常规检测指标与SLE疾病活动指数(SLEDAI)积分之间的相关性.结果 ①SLE组与正常对照组相比,IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达显著增高(P＜0.01);SLE活动组与SLE缓解组比较,IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达增高(P＜0.05);SLE组IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达水平之间呈正相关(r＞0.5,P＜0.05).②SLE组IFIT1、IFIT4、OAS1、OASL及ISG15 mRNA表达水平均与SLEDAI积分呈显著正相关(r＞0.5,P＜0.05).③ESR、CRP、补体C3、C4、ANA与IFIT1、IFIT4、OAS1、OASL、ISG15 mRNA表达水平及SLEDAI积分均无相关性,抗dsDNA抗体与IFIT1、IFIT4、OAS1、OASL、ISG15 mRNA表达水平及SLEDAI积分呈正相关(r＞0.5,P＜0.05).结论 SLE患者IFIT1、IFIT4、OAS1、OASL及ISG15的表达水平在SLE患者中显著增高,并且与SLEDAI积分呈显著正相关,对SLE患者病情活动度和病情严重度的判断均有较大价值,抑制这5个基因的表达可能为SLE的治疗提供新的靶点.     

系统性红斑狼疮合并急性胰腺炎2例并文献复习

正系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种自身免疫性疾病,可以累及全身多器官系统,SLE合并急性胰腺炎(acute pancreatitis,AP)相对少见,且病死率高~[1,2]。本文总结2例SLE合并AP患者的临床特征、治疗及转归,以期提高对本病的认识。病例资料病例1女性,维吾尔族,已婚未生育,16岁,厨     

80岁以上老年急性重症胆管炎的个体化治疗体会

急性重症胆管炎(acute cholangitis se-veretype,ACST)是胆道感染性疾病的严重阶段,是良性胆道疾病死亡的首要原因;其起病急、病情变化快、病死率高。高龄患者并存疾病多,全身重要器官功能减退,机体防御应激能力低下,一但出现ACST,病情更加危重,死亡率极高,因此,选择何种治疗方式降低病死率至关重要。我院自2006年6月至2012年9月根据患者的具体情况,对62例≥80岁老年ACST患者采用个体化的外科干预治疗,有效降低了病死率,现总结分析如下。     

系统性红斑狼疮患者血清基质金属蛋白酶水平变化

目的探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)患者血清基质金属蛋白酶(matrix metalloproteinase,MMP)-3及MMP-9水平变化与疾病的关系和意义。方法测定SLE患者及健康对照者血清MMP-3,MMP-9水平,将SLE患者分为SLE活动期组与SLE非活动期组,且根据有无累及神经系统、肾脏及关节分为不同病损组,将同时无神经系统、肾脏及关节炎者定义为无器官损害组。结果共纳入SLE组患者85例,将其中临床资料相对充分的59例患者分为SLE活动期组22例,SLE非活动期组37例。SLE患者血清MMP-3水平明显高于对照组(P0.01),SLE活动期和非活动期患者之间无明显差异(P0.05);SLE活动期患者血清MMP-9水平低于SLE非活动期患者和对照组,差异有统计学意义(P0.05,P0.01)。神经系统损害组、肾损害组及关节炎组患者血清MMP-9水平明显低于无器官损害组(P0.05)。结论血清MMP-3可能参与了SLE的发病,其水平下降可能与狼疮病情改善有关,可作为治疗后病情好转的信号。血清MMP-9水平和疾病活动或进行性脏器损害有关,有望作为评判SLE疾病活动和预后的指标。     

抗C1q抗体在狼疮性肾炎诊断及预后判断中的价值

系统性红斑狼疮(SLE)是一种累及多系统、多脏器，临床表现复杂、多样，病程迁延反复的自身免疫性疾病。肾脏是SLE最常累及的脏器之一，有些狼疮性肾炎(LN)患者病情进展迅速，短期内发展至肾功能不全，而有些患者在经过恰当的治疗后病情可趋于稳定或完全缓解。此外很多患者起病时临床及组织学特点相似，但是肾脏病复发的严重程度、稳定期的长短等临床特点却完全不同。目前尚不清楚哪些免疫学参数有助于LN复发的诊断，抗ds—DNA抗体滴度增高及补体水平的降低提示狼疮活动，     

Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus

OBJECTIVE: To investigate potential differences between childhood-onset and adult-onset systemic lupus erythematosus (SLE). METHODS: An inception cohort with childhood-onset SLE (n = 67) was compared with an inception cohort with adult-onset SLE (n = 131), each of whom was diagnosed between 1990 and 1998 and followed up until February 1999. Prospective information included data on medications, laboratory markers, and disease activity and damage as measured by the SLE Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), respectively. RESULTS: Eighty-five percent of patients with childhood-onset SLE and 88% of patients with adult-onset SLE were female; the mean duration of followup was 3.2 and 3.5 years, respectively. On average, the children had more-active disease than did the adults at the time of diagnosis and during followup. There was a higher incidence of renal disease in those with childhood-onset SLE (78% versus 52% in adults; P = 0.0005), and the adjusted mean SLEDAI renal score was higher in the children than in the adults (2.37 versus 0.82; P < 0.0001). Treatment with steroids (97% versus 72%; P < 0.0001) and immunosuppressive drugs (66% versus 37%; P = 0.0001) was used significantly more often in children with SLE. Four adult patients with SLE, but none of the children, died during the followup. At the end of the followup, the mean SDI scores in those with childhood-onset SLE were higher than those with adult-onset SLE (1.70 versus 0.76; P = 0.008). CONCLUSION: Children with childhood-onset SLE have more active disease at presentation and over time than do adults with SLE, especially active renal disease. Compared with adults with SLE, children receive more intensive drug therapy and accrue more damage, often related to steroid toxicity.     

Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus.

INTRODUCTION: Systemic lupus erythematosus (SLE) in children usually follows a more severe course than in adults, but sometimes in the previous studies reported there are many confounding factors. OBJECTIVE: To analyse the immunological and clinical characteristics of SLE juvenile onset and SLE adult onset. METHODS: We studied 179 patients with SLE, 49 patients were aged 6-18 yrs at onset of disease. Anti-dsDNA antibodies were detected by radioimmunoassay and antibodies to extractable nuclear antigens (ENA): anti-nRNP, anti-Sm, anti-Ro/SS-A and anti-La/SS-B antibodies by ELISA, counterimmuno-electrophoresis and immunoblotting. RESULTS: Juvenile-onset SLE shows a higher frequency of cutaneous vasculitis (44.8% vs 27.6%; P < 0.05), seizures (18.3% vs 7.6%; P < 0.05) nephropathy (67.3% vs 48.4%; P < 0.025), and discoid lupus erythematosus (26.5% vs 13.8%; P < 0.05). The incidence of articular manifestations is lower than in adults (85.7% vs 96.1%; P < 0.025). No significant differences were found between the two groups in relation with the prevalence of antinuclear antibodies. CONCLUSIONS: Juvenile-onset SLE has more frequent neurological and renal manifestations than adult-onset SLE, but immunological markers are similar in both groups. These features suggest the most severe clinical manifestations in the juvenile-onset SLE group are not related with the presence of studied antibodies by different methods.     

Childhood-onset Systemic Lupus Erythematosus: clinical presentation and prognosis in 31 patients.

OBJECTIVE: To determine the clinical features at onset, the disease course, and prognostic factors in children with SLE. METHODS: The medical records of 31 patients with childhood-onset SLE were reviewed. Signs and symptoms at onset and during the course of the disease were documented as well as survival and SLICC/ACR damage index. The disease course was compared to 135 consecutive adult-onset SLE patients. RESULTS: Childhood-onset SLE most frequently presented with fatigue, arthritis, fever, weight loss, and malar rash. During follow-up, the frequency of the presence of malar rash, anemia, leukocytopenia, and anti-dsDNA antibodies was significantly higher in childhood-onset than in adult-onset patients. Mean SLICC/ACR damage index was 2.6 after 4.7 years of follow-up. The presence of arthritis, anemia, and seizures at the onset of disease resulted in a 2.6 to 3.9 times higher chance of a severe disease course. CONCLUSION: Patients with childhood-onset SLE suffer from substantial morbidity. Arthritis, anemia, and seizures at onset may be indicators of poor prognosis.     

Systemic lupus erythematosus in children and adolescents

Children and adolescents represent 15-20% of all systemic lupus erythematosus (SLE) patients. Although the clinical presentation and immunological findings are similar to those of adult SLE, children usually have a more severe disease at onset with higher rates of organ involvement. Rapid diagnosis and subsequent therapy are necessary to prevent major organ damage. The survival of children with SLE has improved dramatically over the past decades due to the introduction of steroids and immunosuppressive drugs. New strategies to improve the long-term course of the disease and to reduce potential drug toxicities are necessary. A common concept does not exist. There are some promising new drugs. This review article summarizes the epidemiology, pathogenesis, clinical manifestations and therapy of childhood and adolescent-onset SLE.     

Systemischer Lupus erythematodes im Kindes- und Jugendalter

Children and adolescents represent 15–20% of all systemic lupus erythematosus (SLE) patients. Although the clinical presentation and immunological findings are similar to those of adult SLE, children usually have a more severe disease at onset with higher rates of organ involvement. Rapid diagnosis and subsequent therapy are necessary to prevent major organ damage. The survival of children with SLE has improved dramatically over the past decades due to the introduction of steroids and immunosuppressive drugs. New strategies to improve the long-term course of the disease and to reduce potential drug toxicities are necessary. A common concept does not exist. There are some promising new drugs. This review article summarizes the epidemiology, pathogenesis, clinical manifestations and therapy of childhood and adolescent-onset SLE.     

Clinical features and long-term outcomes of systemic lupus erythematosus: comparative data of childhood,adult and late-onset disease in a national register

Systemic lupus erythematosus (SLE) affects predominantly women at reproductive age but may present at any age. Age at disease onset has a modulating effect on presentation and course of disease, but controversies persist regarding its impact on long-term outcome. Our aims were to characterize clinical features, co-morbidities and cumulative damage in childhood-onset, adult-onset and late-onset SLE. Patients with childhood-onset SLE fulfilling ACR 1997 criteria were identified in a nationwide register-Reuma.pt/SLE (N = 89) and compared with adult-onset and late-onset counterparts matched 1:1:1 for disease duration. 267 SLE patients with mean disease duration of 11.9 ± 9.3 years were analyzed. Skin (62 %), kidney (58 %), neurological (11 %) and hematologic involvement (76 %) were significantly more common in childhood-onset SLE and disease activity was higher in this subset than in adult- and late-onset disease (SLEDAI-2K 3.4 ± 3.8 vs. 2.2 ± 2.7 vs. 1.6 ± 2.8, respectively; p = 0.004). Also, more childhood-onset patients received cyclophosphamide (10 %) and mycophenolate mofetil (34 %). A greater proportion of women (96 %), prevalence of arthritis (89 %) and anti-SSA antibodies (34 %) were noted in the adult-onset group. There was a significant delay in the diagnosis of SLE in older ages. Co-morbidities such as hypertension, diabetes and thyroid disease were significantly more frequent in late-onset SLE, as well as the presence of irreversible damage evaluated by the SLICC/ACR damage index (20 vs. 26 vs. 40 %; p < 0.001). Greater organ involvement as well as the frequent need for immunosuppressants supports the concept of childhood-onset being a more severe disease. In contrast, disease onset is more indolent but co-morbidity burden and irreversible damage are greater in late-onset SLE, which may have implications for patients’ management.     

Late-onset systemic lupus erythematosus: a personal series of 47 patients and pooled analysis of 714 cases in the literature

Systemic lupus erythematosus (SLE) is uncommon after the age of 50 years, and studies of elderly patients with SLE are scarce. We conducted the current study to analyze characteristics and outcome of patients with late-onset SLE in a French tertiary referral center, and to compare them with those of younger patients with SLE. From 1980 to 2000, 47 patients were identified as having late-onset SLE, defined as SLE diagnosed at or over the age of 50 years. These patients were compared with a group of 114 randomly selected patients aged younger than 50 years at SLE diagnosis. We compared clinical characteristics, laboratory data, therapy, and course.The female to male ratio was smaller in the late-onset SLE group (p = 0.0012). Some manifestations occurred less frequently in late-onset SLE: arthritis (p = 0.009), malar rash (p = 0.013), and nephropathy (p = 0.009). High-dose corticosteroids (p = 0.0016) and immunosuppressive drugs (p = 0.006) were less commonly used in the elderly. Deaths occurred more frequently in late-onset SLE (p = 0.019), with a 10-year survival rate of 71% versus 95% in early-onset SLE (p < 0.01). In patients with late-onset SLE, causes of death were usually unrelated to SLE.Analysis of pooled data from the literature, based on 714 old and 4700 young SLE patients, confirmed that late-onset SLE was characterized by a smaller female to male ratio (4.4:1 vs. 10.6:1; p = 3.10); a higher occurrence of serositis (36.7% vs. 28.6%; p = 7.10) and pulmonary involvement (21.2% vs. 11.3%; p = 6.10); and a lower occurrence of malar rash (31.1% vs. 62.4%; p = 10), photosensitivity (26.2% vs. 38.2%; p = 6.10), purpura/cutaneous vasculitis (13.4% vs. 25.9%; p = 9.10), alopecia/hair loss (24% vs. 44.9%; p = 3.10), Raynaud phenomenon (24.8% vs. 37.2%; p = 3.10), neuropsychiatric manifestations (15.3% vs. 20.2%; p = 0.025), lymphadenopathy (9.1% vs. 19.6%; p = 2.10), nephrotic syndrome (8.1% vs. 24.3%; p = 0.015), and nephritis (28.6% vs. 42.7%; p = 2.10). Regarding laboratory features, rheumatoid factor positivity was more frequent (32.7% vs. 20.1%; p = 3.10), whereas anti-RNP positivity (10.4% vs. 20.9%; p = 9.10), anti-Sm positivity (9.1% vs. 17.1%; p = 0.001), and a low CH50 complement fraction (45% vs. 64.9%; p = 0.002) were less frequent in old compared with young SLE patients.In conclusion, the clinical pattern of late-onset SLE is characterized by a lower disease severity. The reduced survival observed in this group seems to result mainly from the consequences of aging.     

Health-related quality of life in Italian patients with systemic lupus erythematosus. I. Relationship between physical and mental dimension and impact of age

OBJECTIVE: To examine health-related quality of life (HRQOL) in Italian patients with systemic lupus erythematosus (SLE) and compare it with that of healthy people, and to investigate relationships among different dimensions and subscales of a generic health status measure. METHODS: The Medical Outcomes Study Short Form-36 (SF-36) was applied in a cohort of 126 consecutive SLE patients and 96 healthy controls. At the time of HRQOL testing, all patients underwent clinical and laboratory evaluation. RESULTS: Both physical (PCS) and mental (MCS) component summary scores of the SF-36 were reduced in patients compared with controls. In SLE great variability in all the subscales was observed. Significant correlations between PCS and MCS and between many different subscales were observed in patients but not in controls. The PCS was higher than MCS more frequently in controls than in SLE patients (81 vs 48.4%, P<0.00001). In SLE, HRQOL tended to worsen with age. CONCLUSION: Both PCS and MCS contribute to the decrease in HRQOL in SLE patients. In SLE the mutual interaction between these two dimensions seems to be more relevant than in healthy people.     

Impact of C1q deficiency on the severity and outcome of childhood systemic lupus erythematosus

Objective: To delineate the clinical and laboratory features of systemic lupus erythematosus (SLE) in C1q‐deficient Saudi children and to compare them with sporadic SLE patients with respect to their clinical and laboratory features and disease outcome. Methods: The C1q‐deficient SLE patient group comprised 14 patients, while the comparative group comprised 11 patients selected by systemic sampling from our pediatric lupus clinic database. The two groups were compared with respect to: demographic, clinical and laboratory variables and outcome. Results: The C1q‐deficient SLE patients had an earlier age of onset of disease (P = 0.003); 43% had familial SLE and none of the comparative group had family histories of SLE. The two groups were comparable with respect to gender, disease duration and follow‐up. Scarring alopecia, discoid rash and nail changes were more frequent in the C1q‐deficient SLE patient group. However, there were no significant differences. The mean white blood cell count was lower (P = 0.04) and the level of anti‐Sm and anti‐phospholipid antibodies were higher (P = 0.04) in the C1q‐deficient SLE patients. Other variables did not show significant differences. Two patients from the C1q‐deficient SLE patient group died due to infection. All patients from the control group are alive. Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index mean was higher in the C1q‐deficient SLE patients group but was not statistically significant. Conclusion: C1q‐deficient SLE patients tend to be younger and more likely to have familial disease with severe cutaneous manifestations. The mortality among them is more frequent, which may reflect disease severity.