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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.

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。

系统性红斑狼疮伴肾炎、膀胱炎和肠炎一例

Influence of the Sichuan Wenchuan Earthquake on Disease Activity in Patients with Systemic Lupus Erythematosus

目的:探讨5·12 四川汶川大地震对系统性红斑狼疮(SLE)病情活动性的影响,以及因地震造成的停药、感染、居住环境恶劣(长期紫外线照射)和创伤后应激障碍(posttraumatic stress disease,PTSD)与病情活动性的相关性。方法:分析41例重灾区(北川、平武、江油、安县)SLE患者地震前后实验室指标和临床表现变化情况,采用Logistic回归分析地震造成停药、感染、居住环境恶劣和PTSD四种因素对SLE病情活动性的影响。结果:41例SLE患者地震后有18例病情活动,其中有27%患者出现关节痛,24%出现颜面红斑,19.5%出现发热,22%合并狼疮肾炎，震后C3水平显著低于震前。震后6例患者感染,13例患者停药,16例患者长期居住于恶劣环境中,11例患者患PTSD。通过Logistic回归分析发现PTSD、停药、居住条件恶劣与地震后SLE患者病情活动相关。结论:地震可使一部分重灾区SLE患者病情出现活动,PTSD、停药、居住条件恶劣与SLE病情活动具有相关性。

系统性红斑狼疮继发视网膜中央静脉阻塞1例

系统性红斑狼疮患者行二尖瓣成形术一例

系统性红斑狼疮合并肠穿孔的临床分析

目的探讨系统性红斑狼疮（SLE）引发肠穿孔的临床特点。 方法对2011年1月－2013年1月外科重症监护室收治的3例SLE引发肠穿孔病例，结合相关文献分析该类疾病的临床表现和治疗方法。 结果患者经积极治疗，病情逐渐稳定，均康复出院。 结论SLE合并肠穿孔临床上较少见，迷惑性强，容易误诊误治，未及时发现穿孔可引起严重后果。

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。

Spontaneous pneumothorax secondary to acute lupus pneumonitis: one case report and literature review

Objective To investigate the clinical characteristics of spontaneous pneumothorax secondary to systemic lupus erythematosus (SLE). Methods A case of spontaneous pneumothorax secondary to SLE was reported and related literatures were reviewed. Results A female patient, 26 years old, complaining of repeated fever for 3 months, chest tightness and shortness of breath more than 10 days, was hospitalized in December 2016. After admission, the patient was diagnosed as acute lupus pneumonia and received glucocorticoid, cyclophosphamide and plasma exchange therapy. Sudden right chest pain happened during hospitalization. Bedside chest CT showed right pneumothorax. After closed thoracic drainage, the syndrome of the patient was improved. A total of 14 patients were reviewd but 2 patients were excluded because of incomplete data. The left patients included 9 females and 3 males. The mean age was (28.0±11.9) years. Clinical manifestations of spontaneous pneumothorax secondary to SLE were sudden chest tightness and shortness of breath accompanied by subpleural lung cavity and vesicular changes. The majority of patients could be cured with drainage. Conclusions Pneumothorax secondary to SLE is rare which is associated with the rupture of cavity and cyst in pulmonary secondary to SLE. Majority of patients with pneumothorax could be cured after closed thoracic drainage.

Shrinking Lung Syndrome as Initial Manifestation of Systemic Lupus Erythematosus: A Case Report and Literature Review

ObjectiveTo investigate the clinical features,treatment and pathogenesis of shrinking lung syndrome (SLS). MethodsA patient of systemic lupus erythematosus(SLE)with SLS as initial manifestation was reported.Literatures about SLS were comprehensively reviewed for its clinical characteristics and pathogenesis. ResultsA 27-year-old man was admitted with chief complaint of intermittent dyspnea and chest pain in 2009.Chest X-ray showed small lung volume and elevated diaphragms.Chest CT revealed bilateral pleural thickness and multiple atelectasis,without significant sign of interstitial fibrosis.Lung function showed restrictive pattern.Routine blood test revealed declined white blood cell and platelet count.C3 and C4 were declined.The autoantibody test revealed positive ANA,dsDNA,AHA,and RNP antibody.A diagnosis of SLE with SLS was made.The patient received corticosteroids and immunosuppressive therapy and was followed up for 5 years.His symptoms had relieved,but there was no obvious improvement in chest radiology and lung function.A total of 118 SLS cases were recorded in literature review,among which 100 were females and 18 were males.The average age was 32.98±14.66 years.Common clinical features of SLS included dyspnea,chest pain,orthopnea,breathlessness,and decreased breath sound.Radiology revealed small lung volume,elevated diaphragm,and dysfunction of diaphragm.Lung function showed restrictive pattern.The primary treatment of the disease was corticosteroid and immunosuppressive agents.The use of theophylline,β-agonist and rituximab was also reported. ConclusionWe should be aware of SLS in patients with SLE or other autoimmune diseases when they present with unexplained dyspnea.