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系统性红斑狼疮继发纯红细胞再生障碍一例

【摘要】　目的　提高对系统性红斑狼疮继发纯红细胞再生障碍（pure red cell aplasia，PRCA）的诊断和治疗认识。　方法　报道系统性红斑狼疮继发PRCA病例1例，观察患者对甲泼尼龙联合环孢素治疗的疗效。　结果　使用甲泼尼龙联合环孢素治疗后，系统性红斑狼疮继发PRCA患者血红蛋白恢复良好。　结论　大多数系统性红斑狼疮继发PRCA患者对糖皮质激素和免疫抑制剂联合治疗反应良好。

系统性红斑狼疮患者使用免疫抑制剂后卵巢贮备功能的临床评估

目的研究抗苗勒管激素（AMH）与系统性红斑狼疮（SLE）女性患者使用免疫抑制剂的关系，评估SLE患者卵巢贮备功能。 方法选取2009年1月-2013年12月72例年龄在18～40岁，符合美国风湿病学会诊断标准的初诊SLE女性患者，使用酶联免疫吸附测定法对患者用药前后血清中AMH进行检测，选取同期20例同年龄段健康女性作为正常对照。比较使用环磷酰胺（CTX）患者（A组，n=41）与未使用CTX患者（B组，n=31）血清AMH的差异，了解AMH水平与CTX累积剂量及年龄之间的关系。 结果72例SLE患者使用免疫抑制剂后血清中AMH水平[（2.39±1.12）ng/mL]较使用前[（3.12±1.38）ng/mL]及正常对照组[（2.95±0.86）ng/mL]低，差异均有统计学意义（P＜0.05）。治疗后A组患者血清中AMH水平[（2.24±1.23）ng/mL]低于治疗前[（2.97±1.31）ng/mL]，且低于B组患者血清中AMH水平[（2.92±0.82）ng/mL]，差异有统计学意义（P＜0.05）；B组治疗前差异无统计学意义（P＞0.05）。AMH水平与使用CTX累积剂量及年龄呈负相关（P＜0.05）。 结论AMH可以作为评价SLE女性患者在使用CTX后造成卵巢损伤的一个指标。

Expression of B-cell maturation antigen mRNA in Peripheral Blood Mononuclear Cells in Patients with Systemic Lupus Erythematosus

【摘要】 目的 检测B细胞成熟抗原（BCMA）mRNA在系统性红斑狼疮（SLE）患者外周血单个核细胞（PBMC）的表达水平，探讨BCMA在SLE发病中的意义。 方法 纳入2006年1-11月收治的36例SLE患者，同期17例健康志愿者作为对照组，采用半定量RT-PCR法检测外周血单个核细胞中BCMA mRNA的表达，并与SLE疾病活动指数（SLEDAI）进行相关性分析。 结果 SLE患者组BCMA mRNA表达水平（0.598±0.230）均明显高于正常对照组（0.411±0.309）（Plt;0.05）。SLE患者BCMA mRNA表达水平与SLEDAI评分无相关性（P=0.590）。 结论 SLE患者BCMA mRNA表达水平的增高，可能在SLE的发病机制中具有一定的作用。【Abstract】 Objective To detect the mRNA expression of B-cell maturation antigen (BCMA) in peripheral blood mononuclear cells (PBMC) in patients with systemic lupus erythematosus (SLE), and explore the role of BCMA in the pathogenesis of SLE. Methods From January 2006 to November 2006 the expression of BCMA mRNA in PBMC of 36 patients with SLE and 17 normal controls were measured by half-quantitative RT-PCR. The linear correlation between the expression of BCMA mRNA and SLE disease activity index (SLEDAI) was assessed. Results The level of BCMA mRNA (0.598±0.230) in PBMC significantly increased in SLE patients compared with that in the normal controls (0.411±0.309) (Plt;0.05). The expression of BCMA mRNA in SLE patients showed no correlation with SLEDAI score (P=0.590). Conclusion The results suggest that the expression of BCMA mRNA might play an important role in the pathogenesis of SLE.

白细胞介素-21 对 B 细胞信号调节及与系统性红斑狼疮发病机制的研究

系统性红斑狼疮（systemic lupus erythematosus，SLE）是一种女性多发、累及多器官的慢性难治性自身免疫性疾病。白细胞介素（interleukin，IL）-21 是一种具有多种生物学功能的细胞因子，主要由活化 T 细胞合成与分泌，其受体分布广泛，与 B 细胞表面的 IL-21 受体结合可调控 B 细胞，包含正向促进 B 细胞向浆细胞分化，调节免疫球蛋白产生；反向条件性诱导 B 细胞凋亡及 B10 细胞产生两方面作用。正向作用在于促使 SLE 患者产生自身抗体，而反向作用是促使产生自身抗体的 B 细胞数量减少，而且使 B10 细胞产生更多的具有免疫抑制作用的 IL-10。正反向平衡精准调控有助于对 SLE 病情进行条件性干预，对于疾病的治疗具有重要潜在价值。该文探讨了 IL-21 调控 B 细胞的分化及其与 SLE 之间的关系，对探索 IL-21 对 B 细胞的信号通路在 SLE 发病机制和靶向治疗提供新的思路。

Causal effect of educational attainment on systemic lupus erythematosus: Mendelian randomization study

Objective To analyze the causal relationship between educational attainment and the risk of systemic lupus erythematosus (SLE). Methods Based on the data from publicly available genome-wide association studies, we employed single nucleotide polymorphisms (SNPs) strongly associated with educational attainment as instrumental variables. Two-sample Mendelian randomization analysis was used to investigate the causal relationship between educational attainment and SLE. The primary analysis method used was the inverse variance weighted with multiplicative random effects. Validation methods included inverse variance weighted with fixed effects and MR-Egger methods. Additionally, sensitivity analysis was conducted using the leave-one-out approach. Results Finally, 433 SNPs were included. The inverse variance weighted with multiplicative random effects analysis indicated no causal effect of educational attainment on the risk of SLE [odds ratio =1.111, 95% confidence interval (0.813, 1.518), P=0.509]. Similarly, the other two methods did not find any evidence of a causal relationship (P>0.05); however, significant heterogeneity was observed. The MR-Egger regression analysis provided no evidence of horizontal pleiotropy among the included instrumental variables (P>0.05). The leave-one-out approach did not identify any individual SNP that had a significant impact on the overall effect estimate. ConclusionIn conclusion, this study does not support a causal effect of educational attainment on the risk of SLE.

Detection and Correlation Analysis between Anti-cell Membrane DNA Antibody and Other Autoantibodies

ObjectiveTo analyze the correlation between anti-cell membrane DNA (mDNA) antibodies and other autoantibodies and estimate its diagnosing significance for systemic lupus erythematosus (SLE). MethodsFrom January to August 2015, the sera samples from 254 patients with various autoimmune diseases, including 106 SLE, 80 rheumatoid arthritis (RA), 32 mixed connective tissue disease (MCTD), 29 Sjogren's syndrome (SS), 7 polymyositis/dermatomyositis (PM/DM) and 20 healthy controls, were collected. The anti-mDNA antibody, anti-dsDNA antibody, antinuclear antibody (ANA) and anti-keratin antibody (AKA) were detected by indirect immunofluorescent assay; anti-cyclic citrylinated peptide antibody (CCP) antibody was detected by enzyme-linked immuno sorbent assay; rheumatoid factor (RF) was detected by rat scatter turbidimetry assay; and anti-Sm antibody was detected by Western blotting method. ResultsAnti-mDNA antibody was found in 77 of 106 SLE (72.6%), 4 of 80 RA (5.0%), 6 of 32 MCTD (18.7%), 4 of 29 SS (14.7%), 0 of 7 PM/DM (0.0%) and 0 of 20 healthy controls (0.0%), respectively. It's notable higher in SLE than that in the others (P < 0.001). The sensitivity, specificity and diagnosis efficiency of anti-mDNA antibody for SLE were 72.6%, 91.7% and 84.3%, respectively. Anti-mDNA antibody was significantly correlated with ANA, anti-dsDNA antibody and anti-Sm antibody (P < 0.001), while it had no significant correlation with anti-CCP antibody, AKA and RF (P > 0.05). ConclusionAnti-mDNA antibody is closely related with other SLE associated antibodies and with high sensitivity and specificity for SLE diagnosis.

The Systematic Lupus Erythematosus Liver Harms 30 Examples Clinically To Analyze

摘要:目的:探讨系统性红斑狼疮（SLE）肝损害的临床特点、肝损害发生率与SLE的病情严重程度的关系。方法:对98例SLE的临床资料进行分析,收集所有研究对象的临床资料,对肝损害组(30例)的症状与体征、病情程度、肝功能指标、影像学检查结果进行数据分析,并将其部分生化及免疫学指标与无肝损害组(68对照组)进行比较。结果:SLE患者中SLE肝损害的发生率为30.61%。肝损害组中,病情重度16例(53.33%),17例患者(56.67%)无明显自觉症状,以ALT、AST轻中度升高为主。7例患者肝脏B超异常。肝损害组患者的白细胞值明显低与无肝损害组(Plt;0.05)。而2组的血红蛋白、血小板、CRP、ESR、抗核抗体、抗dsDNA抗体、IgG、补体3等比较差异均无统计学意义(P均gt;0.05)。11例患者接受肾上腺皮质激素(激素)、免疫抑制剂(环磷酰胺、甲氨蝶呤)等药物治疗,9例在出院时复查肝功能正常或好转,另2例肝功能无明显改善;19例患者在接受激素、免疫抑制剂治疗的同时,给予护肝治疗,15例出院复查肝功能正常或好转,另4例无明显改善。而对照组68例中病情重度8例(11.76%)。结论:肝脏是SLE常见累及的靶器官之一,SLE肝损害的临床表现缺乏特异性,以轻至中度肝细胞损害多见,肝损害发生率与SLE病情严重程度成正相关、与SLE的近期预后无关、与长期预后有待进一步研究。Abstract: Objective: To discuss the relationship between the clinical characteristic, The incidence of liver damage and the severity of SLE. Methods: Carries on the mathematical analysis to 98 example SLE clinical material, the collection all object of study clinical material, to liver harm group (30 examples) the symptom and the symptom，Severity the liver function target， the phantom study inspection result carries on the mathematical analysis, and (68 control groups) carries on its part of biochemistry and the immunology target with the nonliver harm group the comparison.Results: In the SLE patient the SLE liver harms the formation rate is 30.61%.In liver harm group, severe illness in 16 cases (53.33%), 17 example patient (56.67%) not obvious subjective symptom, by ALT, AST light moderate ascension primarily.7 example patient liver B ultra exceptionally.The liver harm group patient’s white blood cell value is lower than the nonliver harm group obviously (Plt;0.05), but 2 groups of hemoglobins, the blood platelet, CRP, ESR, the antinuclear immune body, the antidsDNA immune body, IgG, the complement 3 and so on the comparison differences do not have statistics significance (Pgt;0.05). 11 example patients accept the adrenal cortex hormone (hormone), the immunity inhibitor (endoxan, armor ammonia pterin) and so on the medicine treatments, 9 examples when out of hospital reexamines the liver function normal or the change for the better, another 2 example liver function improves not obviously; 19 example patients while accept the hormone, immunity inhibitor treatment, gives protects the liver treatment, 15 example out of hospital reexamination liver function normal or change for the better, another 4 examples improve not obviously. While the control group, 68 cases of severe illness in 8 cases (11.76%).Conclusion: The liver is one of target organs which SLE implicates common, the SLE liver harm. the clinical manifestation lacks the specificity, by sees lightly to the moderate liver cell harm,The incidence of liver damage and SLE severity is positively correlated with shortterm prognosis of SLE has nothing to do with the longterm prognosis remains to be further studied.

Lupus Ischemic Bowel Disease Presenting as Acute Abdominal Pain——Diagnostic Value of Spiral CT

ObjectiveTo evaluate the value of spiral CT in diagnosing ischemic bowel changes in systemic lupus erythematosus (SLE) patients presenting with acute abdominal pain. MethodsThe clinical data and spiral CT imaging files of 23 SLE patients presenting with acute abdominal pain were retrospectively reviewed. Sixteen had contrastenhanced spiral CT scanning of the abdomen, the rest had plain CT study. Observation emphasis was placed on the changes of bowel wall (wall thickness, enhancement pattern, lumen size) and mesentery (mesenteric edema, engorgement of mesenteric vessels and their abnormal arrangement pattern). Other abnormal findings (e.g. fluid accumulation, changes of abdominal solid organs, lymphadenopathy) were also observed. ResultsNineteen patients had intestinal wall thickening (19/23, 82.6%), with the “target sign” in 12 patients (12/16, 75.0%); Bowel lumen dilatation was present in 16 patients (16/23, 69.6%). Mesenteric swelling with increased density of adipose tissue was noticed in 21 patients (21/23, 91.3%); 18 patients had engorgement of mesenteric vessels (18/23, 78.3%), with comb like arrangement in 4 patients (4/16, 25.0%). Other abnormal findings included ascites, hydrothorax, hydropericardium, hepatosplenomegaly and so on. ConclusionThe most common CT findings in SLE patients presenting with acute abdominal pain are the signs associated with ischemic bowel disease. Contrastenhanced spiral CT is a preferable imaging method for both the diagnosis and differential diagnosis of ischemic bowel disease associated with SLE.

Purtscher样视网膜病变为首发症状的系统性红斑狼疮1例

Causal relationship between dietary habits and systemic lupus erythematosus: a Mendelian randomization analysis

Objective This study employs Mendelian randomization analysis to explore the causal relationship between dietary habits and systemic lupus erythematosus (SLE). MethodsWe obtained data from the MRC-IEU database on five dietary habits as instrumental variables for exposure "never eating dairy products" "never eating eggs or foods containing eggs" "never eating sugar or foods/drinks containing sugar" "never eating wheat products" and "I eat all of the above". Summary data related to SLE were retrieved from the MRC-IEU database for the discovery cohort (designated as MSLE) and from a Finnish database for the validation cohort (recorded as FSLE). Two-sample Mendelian randomization analyses were conducted using inverse variance weighting (IVW), MR-Egger, weighted median, Simple Mode, and Weighted Mode methods to investigate the causal relationship between dietary habits and SLE. The MR-Egger intercept test was performed to assess the presence of horizontal pleiotropy, while the leave-one-out method was employed to verify the stability of the results, with Cochran’s Q test and funnel plots used to evaluate heterogeneity. ResultsMendelian randomization analysis indicated that never eating wheat products increases the risk of developing SLE (IVW: P<0.05). In contrast, there was no significant causal relationship between the consumption of dairy products, eggs or foods containing eggs, or the consumption of all of the above with SLE (IVW: P>0.05). Additionally, there was no significant causal relationship between never sugar or foods/drinks containing sugar and MSLE (IVW: P=0.877), although a potential causal association with FSLE was suggested (IVW: P=0.016). The MR-Egger intercept test indicated no evidence of horizontal pleiotropy (P>0.05). ConclusionNever eating wheat products may be an independent risk factor for SLE. However, the causal relationship between never sugar or foods/drinks containing sugar and SLE remains indeterminate.